2010 Craniomaxillofacial

131. A 45-year-old woman comes to the office because she has had a 4-week history of a rapidly enlarging left parotid mass. On physical examination, the patient has ptosis of the left eyebrow and is unable to fully close the left eye or depress the left lower lip. The most likely cause of these findings is tumor involvement at which of the following foramina at the base of the skull? A) Jugular B) Lacerum C) Ovale D) Rotundum E) Stylomastoid

The correct response is Option E. A history of a progressive facial paralysis associated with a parotid mass suggests the diagnosis of a malignant parotid tumor. The temporal, zygomatic/buccal, and ramus mandibularis branches are affected, indicating that the main trunk of the facial nerve is invaded by tumor. The facial nerve exits the skull base from the stylomastoid foramen. The foramen lacerum, foramen ovale, and the foramen rotundum contain the internal carotid artery, mandibular (V3) nerve, and maxillary (V2) nerve, respectively. The glossopharyngeal (IX), vagus (X), and spinal accessory (XI) nerves emerge from the jugular foramen.

129. A 58-year-old man comes to the office for consultation regarding treatment 3 weeks after receiving a diagnosis of squamous cell cancer of the soft palate. He says he has had pain in the left ear for the past 2 months. Examination of the ear shows no abnormalities. The most likely cause of the pain is involvement of which of the following nerves? A) Auricular branch of the vagus (X) B) Auriculotemporal C) Great auricular D) Superficial temporal E) Vestibulocochlear (VIII)

The correct response is Option A. The auricular branch of the vagus nerve (Arnold nerve) carries sensory input from the ipsilateral concha and oropharynx. Chronic external ear pain may alert the astute physician of more serious intraoral pathology. The auriculotemporal nerve carries sensory information from the anterior and superior external auditory canal, and the great auricular nerve relays sensation from the lower half of the ear. The lesser occipital nerve provides sensory input from the superior cranial surface of the ear. Although the superficial temporal nerve is not an anatomically recognized structure, the superficial temporal artery supplies vascular inflow to the anterior external ear.

139. A 73-year-old man undergoes mandible reconstruction for squamous cell cancer. A microvascular thrombosis is most likely to occur within what period of time after the completion of the microvascular anastomosis? A) 0 to 24 Hours B) 25 to 36 Hours C) 37 to 48 Hours D) 49 to 72 Hours E) 73 to 96 Hours

The correct response is Option A. During head and neck reconstruction, a microvascular thrombosis is most likely to occur within 12 hours of completion of the anastomosis. The thrombosis often occurs intraoperatively or upon arrival in the postanesthesia care unit. Nearly 90% of all thromboses occur within 24 hours. Most commonly, thromboses occur as a result of technical issues. Microvascular thrombosis can occur in a delayed manner, even 7 to 14 days postoperatively; however, the rate of these events is much lower than during the initial 24 hours.

150. A 2-year-old boy with a cleft palate and recurrent episodes of serous otitis media caused by impaired eustachian tube function is scheduled to undergo cleft repair. In reconstructing the palate, repair of which of the following muscles is most likely to improve eustachian tube function? A) Levator veli palatini B) Palatopharyngeus C) Salpingopharyngeus D) Superior constrictor E) Tensor veli palatini

The correct response is Option A. In cleft palate reconstruction, repair of the levator veli palatini can improve eustachian tube function. Individuals with unrepaired cleft palate suffer chronic otitis media, which can lead to permanent hearing loss. This is thought to be caused by the dysfunction of the eustachian tube. There are several paratubal muscles that are responsible for the opening and closing of the eustachian tube: the tensor veli palatini, the levator veli palatini, and the salpingopharyngeus. Contraction of the levator veli palatini with superior and posterior displacement of the levator sling opens the eustachian tube. In an unrepaired cleft palate, the ability of the levator veli palatini to open the tube is lost because of its abnormal insertion on the posterior hard palate. Repositioning of this muscle during cleft palate repair restores the levator sling, allowing dilation of the eustachian tube. The tensor veli palatini opens the eustachian tube and may have "pumping action" that milks the contents of the tube. This muscle's function is likely unaffected by clefting. However, its ability to open the eustachian tube may be iatrogenically reduced by complete hamular fracture or division of its tendon during cleft palate repair. The salpingopharyngeus also opens and closes the eustachian tube. However, because of its small size, it is the least important of the paratubal muscles and has minimal functional significance. The palatopharyngeus muscle optimizes velopharyngeal closure. Along with the superior constrictor, it causes medial displacement of the lateral pharyngeal wall. The superior constrictor is the main component of the Passavant ridge and functions to bring about medial displacement of the lateral pharyngeal wall through a sphincteric mechanism

124. A 52-year-old man is evaluated for reconstruction of a nasal defect resulting from right nasal resection for a neglected squamous cell cancer. Physical examination shows a full-thickness defect involving the right lateral nasal wall. The nasal ala and tip subunits are intact. What is the blood supply of the most appropriate lining flap? A) Anterior ethmoid artery B) Facial artery C) Inferior labial artery D) Radial artery E) Supratrochlear artery

The correct response is Option A. Successful reconstruction of full-thickness defects of the nose requires reconstruction of the skin, lining, and support system of the nose. A number of options are available for the lining of nasal defects. Intranasal lining flaps are commonly used because they allow simultaneous placement of cartilage grafts. In addition, cartilage grafts may be harvested from the nasal septum. In the scenario described, the entire lateral nasal wall has been resected, leaving the nasal ala and tip subunits intact. A contralateral mucoperichondrial flap can be harvested based on the anterior ethmoid artery and used for lining of the nasal reconstruction. The septal cartilage is also harvested and removed to provide support. Therefore, the most appropriate answer is the anterior ethmoid artery. The supratrochlear artery together with branches from the supraorbital vessels is the blood supply of the forehead flap and would be used in this case for external skin coverage. The facial artery is the blood supply to the medial cheek and the nasolabial flap. Although the nasolabial flap can be used for lining, it is usually reserved for smaller defects, particularly those involving the nasal ala. The radial artery is the blood supply for the radial forearm flap. Although this flap is occasionally used to provide intranasal lining, it is usually reserved for total or subtotal nasal reconstruction. The inferior labial artery is the blood supply of the lips and is not useful for nasal reconstruction. The septum composite flap can be used for subtotal nasal reconstruction and transfers the residual septum based on the superior labial artery.

101. A 45-year-old man comes to the office for follow-up examination 3 months after undergoing surgical repair of a through-and-through laceration of the left cheek. During the procedure, an injury to the parotid duct was noted and repaired. He says he feels fine, but he now has difficulty playing the trumpet because he is unable to create sufficient air pressure in his mouth. Which of the following muscles was most likely also severed? A) Buccinator B) Levator labii superioris C) Masseter D) Risorius E) Zygomaticus major

The correct response is Option A. The buccinator muscle is the only muscle of facial expression that compresses the cheeks, which is an essential function for playing air-based instruments such as the trumpet. Both the buccinator and the orbicularis oris compress the lips, also necessary for playing trumpets. The buccinator muscle ordinarily contributes to the function of forming a food bolus during mastication. The path of the parotid duct typically leaves the parotid gland from its anterior border and courses superficially to the masseter muscle toward the mid cheek and then pierces the substance of the buccinator muscle, reaching the mucosa of the oral cavity opposite the maxillary second molar. The levator labii superioris, the risorius, and the zygomaticus major muscles all have a function that contributes to separating the lips, which releases the pressure from inside the mouth. These muscles arise from bone and fascia and attach to the lips. The masseter, which originates in the zygomatic arch and inserts on the lateral surface of the ramus, elevates the mandible. The masseter has no role in holding intrabuccal or lip pressure.

136. A 19-year-old man comes to the office because he has a deformity of the bridge of the nose and numbness of the nasal tip 2 weeks after being struck in the nose with a baseball. X-ray studies show a fracture of the nasal bones. The most likely cause of the loss of sensation is injury to which of the following nerves? A) Anterior ethmoidal B) Infraorbital C) Infratrochlear D) Nasopalatine E) Superior alveolar

The correct response is Option A. The external branch of the anterior ethmoidal nerve emerges between the nasal bone and the upper lateral nasal cartilage to supply sensation to the skin, the dorsum of the lower nose, and tip. The innervation of the nose is supplied by the trigeminal nerve. Cranial nerve V1 (ophthalmic division) supplies the infratrochlear nerve, which provides sensation to the skin of the bridge, the upper lateral nasal area, and the anterior ethmoidal nerve. Cranial nerve V2 (maxillary nerve) distributes the infraorbital nerve, which supplies sensation to the skin on the lower lateral half of the nose, and the nasopalatine nerve, which innervates the nasal septum and anterior hard palate. The superior alveolar nerve is also a branch of V2 but does not provide sensation to the nose.

148. A 20-year-old man comes to the emergency department because of a deep laceration of the lower lip. Bilateral mental nerve blocks will be used to anesthetize the lip properly prior to repair. Which of the following is the most appropriate landmark for needle placement for the blocks? A) Mandibular second premolar B) Maxillary canine C) Oral commissure D) Retromolar fossa E) Sigmoid notch

The correct response is Option A. The inferior alveolar nerve enters the mandible on the medial side of the ramus about 10 mm below the sigmoid notch. It then courses through the canal closest to the buccal cortical plate in the region of the ramus, angle, and down to the third molar with an average distance of 1.8 mm ± 1 mm. The nerve then swerves away at a position of 4.1 mm + 1 mm from the buccal cortex as it passes the region of the first and second molars. As it traverses the mandibular body, it is lowest and closest to the inferior cortex (7.5 + 1.5 mm) near its exit site at the level of the first molar and second premolar via the mental foramen on the anterior surface of the mandible. The mental nerve supplies the skin of the lower lip and chin right up to the midline. The maxillary canine may be used as a landmark for needle insertion toward the infraorbital foramen during infiltration of the infraorbital nerve. The maxillary second molar is a landmark typically used to locate the opening of the Stensen duct. The oral commissure is used for facial aesthetic measurements and not for nerve blocks. The retromolar fossa, posterior to the mandibular third molar, is the preferred location for needle insertion to anesthetize the buccal nerve, which normally supplies sensibility to the central cheek. The sigmoid notch is used as a landmark to reference the location of the inferior alveolar nerve.

138. A 9-month-old boy is brought to the office because of a midline glabellar mass. The parents report that it has enlarged gradually since they first noticed it 6 months ago; it does not change in size when the patient cries. Physical examination shows a nonmobile, firm, and nontender mass. The nasal root is not broadened, and intercanthal distance is within normal limits. Which of the following is the most likely diagnosis? A) Dermoid cyst B) Encephalocele C) Glioma D) Hemangioma E) Pilomatricoma

The correct response is Option A. The most likely diagnosis in the scenario described is a dermoid cyst. Nasal dermoids are the most common congenital nasal mass. Dermoid cysts most often occur in children in the lateral brow or midline glabellar region. They generally grow slowly, and intracranial communication should be ruled out with either a CT scan or MRI. Intracranial communication is less likely in this scenario because there is no broadening of the nasal root or increased intercanthal distance. An encephalocele is a midline malformation that is present at birth and addressed shortly thereafter. On physical examination, it would be soft and mobile. CT scan or MRI would confirm this diagnosis. A hemangioma or encephalocele may change size with crying. A glioma is heterotopic neural tissue left during the regression of neurologic tissue during embryonic development. Like an encephalocele, broadening of the nasal root and widened intercanthal distance are common. A hemangioma typically presents with sporadic growth during the first 12 months of life, then it reaches a plateau and eventually regresses in most cases. On physical examination, this would be neither fixed nor firm and is typically discolored. A pilomatricoma is a rare, benign, circumscribed epithelial neoplasm that is derived from hair matrix cells. It is classically not fixed and very superficial.

104. A 16-month-old boy who underwent correction of a complete unilateral cleft lip 2 months ago is brought to the office because his mother is concerned about the appearance of the scar on his lip. Physical examination shows a corrected upper lip with a good pout and contour. The scar is flat, slightly widened, and moderately erythematous. Which of the following is the most appropriate management at this time? A) Continued optimal scar management B) Immediate revision of the scar C) Laser resurfacing of the scar D) Revision of the scar in 4 months

The correct response is Option A. The patient described displays a good result after unilateral cleft lip repair. However, the mother is overly concerned about the appearance of the scar, and she needs to be reassured about the result. She should be reeducated concerning good scar care, which includes use of a strong sunblock, and massage of the scar. Even if the scar were a bad one, the best option at this early stage would be optimal scar care. Revision of scars in children earlier than 12 months is generally not advisable, as they typically continue to improve during this time. Laser resurfacing has not been shown to be an effective early modifier of scar outcomes.

144. A 15-year-old girl with a history of an optic glioma, multiple café-au-lait spots, and a large plexiform neurofibroma of the cheek comes to the office for evaluation. Which of the following best represents her lifetime risk of developing a malignant peripheral nerve sheath tumor? A) Less than 15% B) 20% to 35% C) 40% to 60% D) 65% to 80% E) Greater than 85%

The correct response is Option A. The patient described has neurofibromatosis 1 (NF1), an autosomal genetic disorder that leaves affected individuals at risk for developing a variety of benign and malignant tumors. The most common tumors are neurofibromas and optic gliomas. Plexiform neurofibromas are clinically present in approximately 25% of patients. This type of neurofibroma consists of a network of neurofibroma tissue and grows along the length of nerves, often involving multiple nerve fascicles, branches, and plexi. Individuals with NF1 have a 7 to 13% lifetime risk of developing a malignant peripheral nerve sheath tumor (MPNST), which usually arises in a pre-existing plexiform neurofibroma. Diagnosis of a MPNST is problematic within the context of NF1 because the emergence of a lump is not unusual. The clinical symptoms of malignancy are intertwined with the symptoms of benign tumors. Rapid growth and other symptoms, such as pain, are indications for the need for a biopsy.

120. A 4-year-old girl is brought to the office for evaluation of hemifacial microsomia. Physical examination shows maxillary hypoplasia, orbital dystopia, and complete absence of the mandibular condyle. The presence of which of the following additional findings is most suggestive of Goldenhar syndrome? A) Epibulbar dermoids B) Facial nerve VII impairment C) Midfacial port-wine stain D) Multiple pits of the lower lip E) Upper eyelid colobomas

The correct response is Option A. The presence of epibulbar dermoids is a key clinical finding that distinguishes Goldenhar syndrome from hemifacial microsomia. Although Goldenhar syndrome is also frequently associated with defects of cranial nerve VII, this finding is also commonly described in other craniofacial anomalies, including hemifacial microsomia. A port-wine stain present within the V1 or V2 distribution is suggestive of potential Sturge- Weber syndrome. Van der Woude syndrome is commonly associated with lower lip pits as well as cleft lip/palate. Upper lid colobomas are often described in relation to hemifacial microsomia, of which Goldenhar syndrome is a variant.

pic 130. An 18-year-old man is evaluated because of the facial mass shown. The mass was present at birth as a faint red patch of skin. Throughout his life, the mass has continued to enlarge, change color, and has become increasingly painful. Examination of a specimen obtained on biopsy 2 years ago ruled out malignancy. Gadolinium-enhanced MRI shows high vascular flow in and around the lesion. Which of the following is the most likely diagnosis? A) Arteriovenous malformation B) Infantile hemangioma C) Lymphatic malformation D) Sturge-Weber syndrome E) Venous malformation

The correct response is Option A. (Please note that this pictorial appears in color in the online examination) The clinical history and MRI findings strongly suggest that the condition described is an arteriovenous malformation. When there is cutaneous involvement, these anomalies can be observed at birth as a faint area of pink discoloration. Over time, there is a tendency for gradual expansion of the malformation to involve adjacent tissues and to become warm and painful. They are considered "high flow" lesions, implying a significant arterial component to the mass. Most vascular anomalies can be diagnosed clinically. Gadolinium-enhanced MRI can be useful to determine the extent and nature of the vascular components in the lesion. In spite of significant advances in the understanding and classification of vascular anomalies, many malformations are still mislabeled as hemangioma. This type of malapropism is not inconsequential, as the natural history and recommended interventions for these distinct lesions are very different. Infantile hemangioma is a vascular tumor that typically appears shortly after birth, grows aggressively over the first year of life (proliferation), and subsequently regresses (involution). Intervention is rarely indicated, except when there is functional impairment or to facilitate aesthetic improvement after involution. In contrast, vascular malformations are present at birth, tend to enlarge gradually over time, and do not regress. Treatment of these lesions depends on the type of malformation and includes sclerotherapy with or without resection for lymphatic and venous malformations, as well as selective embolization with or without resection in arteriovenous malformation. Infantile hemangioma is incorrect for the reasons described above. Lymphatic and venous malformations are present at birth and enlarge with growth, but they do not have significant arterial components, and they appear as "low flow" lesions on gadolinium-enhanced MRI. Sturge-Weber syndrome is the associated findings of capillary malformation in the V1 distribution of the trigeminal nerve, leptomeningeal vascular malformations, glaucoma, and seizure disorder.

pic 141. A 33-year-old man is brought to the emergency department after sustaining injuries to the face during a snowmobile collision. Axial CT scan is shown. Which of the following is the most appropriate management? A) Ablation of the frontal sinus B) Cranialization and reconstruction of the anterior frontal sinus wall C) Obliteration of the frontal sinus D) Observation with x-ray studies monthly E) Reconstruction of the nasofrontal duct and anterior and posterior frontal sinus walls

The correct response is Option B. A comminuted fracture of the frontal sinus is shown in the CT scan, with significant displacement of fragments involving both the anterior and posterior frontal sinus walls and the region of the nasofrontal duct. The most appropriate treatment is cranialization and reconstruction of the anterior wall to restore normal forehead contour and protect the brain. Cranialization involves removing the posterior frontal sinus wall to make the sinus part of the intracranial space and blocking the nasofrontal duct, typically with bone or a pericranial flap so that sinus mucosa is excluded from the intracranial space. The anterior frontal sinus wall is also reconstructed as part of this procedure to restore normal forehead contour and to protect the brain. Ablation (or exenteration) involves removing the anterior frontal sinus wall and allowing the skin to collapse in on the posterior wall, if it is intact, or on the dura if the posterior wall requires removal as well (as it would in this scenario). While this may lead to a stable, healed wound in the patient described, it is not the most appropriate management as it would leave the patient with a significant deformity that would be difficult to reconstruct. It would also leave the underlying brain unprotected by bone. Ablation is appropriate only in extreme cases of acute infection that require open drainage and removal of infected bone. Obliteration of the frontal sinus involves removing the sinus mucosa and burring the bony walls to remove mucosal invaginations, plugging the nasofrontal duct, and filling the sinus cavity with fat, muscle, bone, or alloplasts. A variation of this is osteoneogenesis, where the cavity is not filled but allowed to fill spontaneously with bone or scar over time. This would not be feasible in the patient described because of the extreme comminution and displacement of the posterior wall fracture fragments. Observation is appropriate for minimally or nondisplaced fractures of the frontal sinus that do not involve the nasofrontal duct or do not acutely obstruct the nasofrontal duct. Regular plain x- ray studies should be obtained for several months afterward to monitor for development of a frontal sinus mucocele, which requires surgical treatment. Reconstruction involves preserving sinus mucosa and reducing fractures of the nasofrontal duct and sinus walls. There are currently no data to support this technique, and in the patient described it could to lead to mucocele development as the nasofrontal duct became scarred and obstructed postoperatively.

128. An 8-year-old boy is brought to the office because he has been unable to smile, close his mouth, or completely close his eyes since birth. Physical examination shows facial immobility, strabismus, and syndactyly of the ring and little fingers. Which of the following is the most likely diagnosis? A) Klippel-Trénaunay syndrome B) Möbius syndrome C) Pierre Robin sequence D) Poland syndrome E) Treacher Collins syndrome

The correct response is Option B. Although von Graefe described a case of congenital facial diplegia in 1880, the syndrome was reviewed and defined further by Paul Julius Möbius, a German neurologist, in 1888 and 1892. Because of these contributions, Möbius is now the eponym used to describe the syndrome. In most studies, Möbius syndrome is defined as congenital facial weakness combined with abnormal ocular abduction - weakness of cranial nerves VII and VI. The typical phenotypic appearance of an affected individual includes an immobile facial appearance and ocular palsy. A mask-like facial appearance is pathoneumonic. Additional musculoskeletal abnormalities occur in one third of patients with Möbius syndrome. The typical physical features of Treacher Collins syndrome include downward slanting eyes, lower eyelid colobomas, micrognathia, conductive hearing loss, underdeveloped zygoma, and malformed or absent ears. This is also considered the combination of Tessier No. 6, 7, and 8 clefts. Poland syndrome is characterized by underdevelopment or absence of the pectoralis muscle on one side. Syndactyly often occurs on the ipsilateral hand. There are no associated facial anomalies. Pierre Robin is not a syndrome but rather a sequence or a collection of physical findings that appear together. Phenotype includes micrognathia, glossoptosis, and cleft palate. Breathing and eating difficulty often results. Klippel-Trénaunay syndrome is characterized by a triad of port-wine stain, varicose veins, and bony and soft-tissue hypertrophy involving an extremity.

119. A 68-year-old woman undergoes partial glossectomy, resection of the anterior floor of the mouth, and bilateral modified radical neck dissection to treat squamous cell carcinoma in the ventral tongue and anterior floor of the mouth. The resulting defect is reconstructed with a 5 × 6-cm radial forearm free flap. The free flap is anastomosed to the left facial artery and left internal jugular vein. The forearm donor site is reconstructed with a split-thickness skin graft from the thigh. In addition to Current Procedural Terminology (CPT) code 15758 (free fascial flap with microvascular anastomosis), which of the following is most appropriate? A) 13152: Complex repair mouth 2.6-7.5 cm B) 15100: Split thickness skin graft, arm; less than 100 cm2 C) 35761: Exploration of vessels without repair D) 40840: Anterior vestibuloplasty E) 69990: Use of operating microscope

The correct response is Option B. Free flap procedure codes are global and include: 1. 2. 3. 4. 5. 6. 7. 8. Elevation of the flap Isolation of donor flap vessels used for microvascular anastomosis Transfer of the flap to the recipient site Isolation of the recipient vessels used for microvascular anastomosis Microvascular anastomosis of one artery Microvascular anastomosis of one or two veins Inset of the flap in the recipient site Primary closure of the donor site If a free flap procedure involves more than one of the above global components, it is appropriate to report these as added elements, as they are considered over and above the usual free flap procedure. These can include: 1. 2. 3. 4. 5. Vein grafts Neurorrhaphy Nerve grafts Skin grafts - donor site or recipient site Closure of the donor site that is more extensive than primary closure 6. Wound preparation of the recipient site Additionally, CPT 69990, use of the operating microscope, is also included with the free flap codes. It should not be coded separately.

145. A 45-year-old man is brought to the emergency department 2 hours after sustaining a laceration to the face from a circular saw. Physical examination shows a deep, vertically oriented wound that extends from the lateral aspect of the right lower eyelid to the neck. The patient is unable to elevate the right upper lip. Which of the following is the longest interval after the injury during which the distal nerve can be successfully stimulated? A) 3 Hours B) 3 Days C) 3 Weeks D) 3 Months

The correct response is Option B. Injury to the facial nerve should be suspected in any deep laceration in the vicinity of the parotid gland and posterior cheek. Clinical confirmation can be readily observed by signs of complete or partial paralysis of facial musculature. Primary end-to-end repair yields the best results, but interposition nerve grafting may be necessary if there is a segmental defect. The distal end of the transected facial nerve may be stimulated for approximately 72 hours after nerve injury. Beyond this period, the neurotransmitter stores become depleted, and depolarization at the motor end plates of the facial musculature does not occur.

105. An 18-year-old man undergoes Le Fort I advancement for correction of a 10-mm maxillomandibular discrepancy as a result of maxillary hypoplasia. Which of the following diagnoses places this patient at greatest risk for postoperative development of velopharyngeal incompetence? A) Apert syndrome B) Clefting of the lip and palate C) Craniofacial microsomia D) Mandibular prognathism

The correct response is Option B. Substantial advancement of the maxilla in patients with mid face hypoplasia secondary to a repaired cleft of the lip and palate is a risk factor for the development of velopharyngeal incompetence. Key preoperative findings to evaluate the risk include nasal air emission, nasal resonance, borderline velopharyngeal incompetence, or a combination of findings. In patients with an Angle class III malocclusion due to mandibular prognathism, mandibular setback, rather than maxillary advancement, is the procedure of choice. This should not increase a patient's risk for development of velopharyngeal incompetence. Patients undergoing maxillary advancement for other craniofacial anomalies, such as craniofacial microsomia or Apert syndrome, are at lower risk for development of velopharyngeal incompetence.

116. A 26-year-old woman is being evaluated because she has had complete left- sided, flaccid facial paralysis since she awoke 3 hours ago. She also has a metallic taste in her mouth and hypersensitivity to sound. Denervation of which of the following muscles is the most likely cause of the hypersensitivity to sound? A) Levator palatini B) Stapedius C) Tensor tympani D) Tensor veli palatini E) Zygomaticus major

The correct response is Option B. The 26-year-old woman described has the typical history of Bell palsy. In patients with Bell palsy, the entire nerve is inflamed, but the maximum conduction block is either in the meatal or labyrinthine segments. Because the conduction block is proximal to the chorda tympani and stapedial nerve, patients also experience a change in taste and a decreased ability to accommodate (ie, dampen) loud noises. When an acoustic tumor causes facial paralysis, the paralysis is usually slowly progressive. Patients generally present with a hearing loss, not a hypersensitivity to noise. The tensor tympani muscle attaches to the malleus and is innervated by the trigeminal nerve, not the facial nerve. The tensor veli palatini muscle is also innervated by the trigeminal nerve and is responsible for active dilatation of the eustachian tube. Blockage of the eustachian tube would cause a hearing loss. The zygomaticus major muscle is innervated by the facial nerve. Denervation causes a decreased ability to smile, not hearing loss. The levator palatini muscle is innervated by the vagus nerve

125. Which of the following tooth root apexes is at greatest risk for damage during a Le Fort I osteotomy for maxillary advancement? A) Bicuspid B) Canine C) Central incisor D) First molar E) Lateral incisor

The correct response is Option B. The canine tooth root is the longest root extending onto the maxillary wall near the piriform rim. The length of the canine tooth from the incisal edge to the root apex is approximately 30 mm. The root is at risk during both osteotomy and plating and serves as an important landmark in operative planning. The roots of central and lateral incisors and bicuspids and molars are at less risk because they have shorter roots. Crowns of the central and lateral incisors are at greater risk during disimpaction of the Le Fort I segment if the maxillary disimpaction forceps are not placed properly. With careful osteotomy planning, disimpaction and plating dental injury are infrequent occurrences.

pic 103. A 67-year-old woman comes to the office because of a 2-month history of halitosis and pain and swelling in the jaw. History includes placement of dental implants 20 years ago. The patient is currently undergoing chemotherapy for Stage IV lung cancer with metastases to the spine. Physical examination shows exposure of the mandible and dental implant posts. A photograph is shown. Examination of a specimen obtained on biopsy is consistent with osteonecrosis and is negative for malignancy. Administration of which of the following is the most likely cause of this patient's condition? A) Bevacizumab B) Bisphosphonate C) Cetuximab D) Dexamethasone E) Doxorubicin

The correct response is Option B. The patient described has osteonecrosis of the mandible and an orocutaneous fistula caused by bisphosphonate (Zometa) therapy. Bisphosphonates bind to calcium crystals in the bone and are resistant to degradation by alkaline phosphatase. As such, they inhibit osteoclast-mediated bone resorption. They are used in the treatment of osteoporosis and, in higher doses, for treatment of bone metastases. Although they are an important tool in cancer therapy, bisphosphonates can initiate osteonecrosis of the jaw, particularly in the presence of trauma, infection, foreign body, and radiation injury. Prevention with oral hygiene and avoidance of invasive dental procedures is important. However, once osteonecrosis occurs, it must be treated. Treatment often consists of stopping bisphosphonate therapy and thorough debridement of bone with removal of hardware. More extensive surgical intervention is determined on a case-by-case basis. In the scenario described, the patient was treated with a marginal mandibulectomy, removal of involved dental implant posts, and a submental artery flap to cover the floor-of-mouth defect and seal the orocutaneous fistula. Although bevacizumab (Avastin) and dexamethasone (Hexadrol) have been associated with poor wound healing, and doxorubicin (Adriamycin) and cetuximab (Erbitux) have been associated with mouth sores, none has been associated with osteonecrosis.

110. An 11-year-old boy is brought to the office because of a 1-year history of progressive left-sided hemifacial atrophy. He has the classic coup de sabre appearance. Examination shows facial bony structures that are intact. Which of the following imaging studies is most appropriate to include in a diagnostic workup of this patient? A) MRI of the abdomen B) MRI of the brain C) Plain x-ray study of the chest D) Plain x-ray studies of the hands

The correct response is Option B. The patient described has progressive hemifacial atrophy, or Parry-Romberg syndrome (PRS). This rare disorder is characterized by progressive wasting of the skin and subcutaneous tissues, and, in some cases, the muscle and bone of the face. It is typically unilateral. Age of onset is the first or second decade of life in most cases. Progression of the wasting may continue for several years. The etiology is unknown, but autoimmune, infectious, neurologic, and traumatic causes have all been implicated in various cases and investigations, and it is considered by many to be part of the spectrum of linear scleroderma. Lacking any adequate medical treatment, PRS is typically allowed to run its course, with reconstruction of the resulting defects with fat injection, soft-tissue augmentation with free tissue transfer, bone grafting, and orthognathic surgery as indicated. Neurologic symptoms are commonly associated with PRS, including seizures, migraine, Horner syndrome, and hemiplegia. Various ophthalmologic conditions are also common. Abnormalities are frequently seen on MRI of the brain, even in the absence of neurologic symptoms, and therefore MRI of the brain is indicated such that these abnormalities might be investigated further as needed. Defects in the skin may also occur elsewhere in the body, but bony abnormalities of the extremities or abnormalities of the chest, abdomen, or neck are not typically found. Therefore, the other imaging studies listed would not be expected to yield useful information in most cases of PRS. Other appropriate tests might include serology for viral or autoimmune etiologies of PRS.

pic A 70-year-old man comes to the office because of a 6-month history of a wound in the right supraorbital region that is draining fluid. Photographs are shown. History includes type 1 diabetes mellitus, chronic obstructive pulmonary disease, and basal and squamous cell carcinoma in the supraorbital area, which was treated with Mohs micrographic surgery, cranial burring, split-thickness skin grafting, and radiation. He has smoked one pack of cigarettes daily for 60 years. Physical examination shows a 4 × 2-cm area of exposed bone with no mobility in the immediately adjacent skin. Echocardiography shows an ejection fraction of 25%. Examination of a specimen obtained on biopsy shows recurrence of squamous cell carcinoma. After excising the tumor, a bony deformity of the supraorbital rim and exposed dura are present. Which of the following is most appropriate to correct this patient's defect? A) Alloplastic reconstruction and a local flap B) Rib graft with local soft-tissue coverage C) Scalp flap D) Skin graft

The correct response is Option C. In the scenario described, bony reconstruction will not impact function, and therefore soft-tissue coverage is adequate. A scalp flap is the most appropriate option because it will bring in blood supply and soft-tissue coverage without the risks associated with extended general anesthesia. Skin grafting would likely not heal in a radiated bed. The long history of a draining wound is a contraindication to the use of alloplastic material. Although a rib graft would provide bony support, it would also increase risk because of the donor site morbidity in the patient described, who has chronic obstructive pulmonary disease and is at high risk for postoperative pneumonia

142. A 53-year-old woman comes to the office for consultation about lip reconstruction 1 week after resection of an upper lip adnexal tumor with negative margins. Examination shows a central full-thickness defect of 75% of the upper lip. Which of the following is the most appropriate method of functional reconstruction? A) Abbe flap only B) Bilateral Estlander flaps with an Abbe flap C) Bilateral Karapandzic flaps with an Abbe flap D) Radial forearm flap only E) Radial forearm flap with a palmaris longus sling

The correct response is Option C. Lip defects are commonly encountered as a result of skin cancers, such as squamous cell cancer and basal cell cancers; however, tumors of the minor salivary glands are also seen. In the relatively young patient described, who has a large resection of the central upper lip, the most appropriate choice for reconstruction is bilateral Karapandzic flaps with a central Abbe flap for philtral reconstruction. Karapandzic flaps enable transfer of the remaining upper lip while maintaining the innervations of the musculature (unlike the Gilles flap). Although bilateral Karapandzic flaps may be useful for defects up to 80% of the width of the upper lip, in the patient described, this is not an ideal choice because the philtrum would be lost, thereby resulting in a significant cosmetic deformity. The Estlander flap is useful for reconstruction of commissure defects and not central defects. The Abbe flap alone would be insufficient to close a 75% defect. The radial forearm flap, either with or without a palmaris sling, would be suboptimal because of differences in color match, innervation, and lack of vermillion reconstruction.

137. Which of the following muscles is used to construct the sphincter during a sphincter pharyngoplasty for the treatment of velopharyngeal insufficiency? A) Levator veli palatini B) Musculus uvulae C) Palatopharyngeus D) Superior constrictor E) Tensor veli palatini

The correct response is Option C. Routinely, postoperative velopharyngeal insufficiency is treated with either a posterior pharyngeal flap or a sphincter pharyngoplasty. A sphincter pharyngoplasty is performed by taking the posterior tonsillar pillar, containing the palatopharyngeus muscle, and elevating it inferiorly to superiorly. The elevated posterior tonsillar pillar, pedicled superiorly, is rotated 90 degrees medially, positioned side by side, and sewn into an incision made horizontally on the posterior pharyngeal wall at the level of the adenoid pad. The levator veli palatini muscle, in the normal palate, rests horizontally (coronally) within the middle third of the velum (soft palate) and functions as the motor of the velum. This muscle pulls the soft palate posteriorly and superiorly, allowing for apposition of the free edge of the velum against the posterior pharyngeal wall (velopharyngeal competence). The musculi uvulae arise as paired slips from the palatine aponeurosis and course sagittally in the velar midline, ending in the uvula. The musculi uvulae act as a flexible beam, providing a stiffness-modifying mechanism for the velum. The superior pharyngeal constrictor arises from the hamulus and course sagittally along the lateral pharyngeal walls to decussate in the midline of the posterior pharyngeal wall. The superior pharyngeal constrictor is the muscle from the posterior pharyngeal wall that is utilized in the posterior pharyngeal flap. The tensor veli palatini muscle arises from the skull base and courses inferiorly and medially around the hook of the hamulus and into the palate as the tensor aponeurosis. It joins the velum (soft palate) to the hard palate.

112. A 65-year-old man undergoes hemiglossectomy and modified radical neck dissection for tongue cancer. Which of the following flaps is most appropriate for reconstruction? A) Jejunum B) Latissimus C) Radial forearm D) Scapula E) Vertical rectus abdominus myocutaneous

The correct response is Option C. The radial forearm flap is based on the radial artery and its vena comitans. In addition, the cephalic vein can usually be harvested together with the flap to provide venous outflow. In the patient described, the radial forearm flap is the best choice because it provides a thin, pliable skin paddle suitable for repair of a hemiglossectomy defect. In addition, the long pedicle vessels enable anastomosis to the contralateral neck. The scapula flap can be used in this defect; however, the flap is more bulky. The latissimus flap and the rectus flap are similarly too bulky for a hemiglossectomy and are better choices for a total glossectomy. Finally, the jejunum flap is not useful for a hemiglossectomy defect and is used most commonly for circumferential pharyngeal defects.

146. A 4-year-old child has a congenital sinus tract opening at the anterior border of the lower third of the sternocleidomastoid muscle. Which of the following nerves is most likely to be injured during surgical excision of the fistulous tract? A) Accessory (XI) B) Facial (VII) C) Hypoglossal (XII) D) Lingual E) Vagus (X)

The correct response is Option C. The child described has a branchial cleft sinus or fistula, which is an embryologic remnant of the cleft between the second and third branchial arches. The fistulas can be bilateral in up to 30% of cases. Histologically, they are lined with stratified squamous epithelium but may also have some pseudostratified columnar ciliated epithelium in their walls. They also contain keratin, hair follicles, sweat glands, and sebaceous glands. They must be completely excised to prevent recurrence. The second branchial arch descends over the third, resulting in an external opening in the lower neck. The internal opening lies at the anterior aspect of the posterior pillar of the fauces, just behind the tonsil (which is the junction between the second and third branchial arches). Usually, the fistula will follow the carotid sheath upwards before crossing the hypoglossal (XII) nerve and passing between the internal and external carotid arteries to reach the tonsillar fossa. As a result, the hypoglossal nerve is at risk during surgery. The facial (VII) nerve is not in the vicinity of the fistula. The facial nerve is intimately related to first branchial arch sinuses and cysts. The spinal accessory (XI) nerve is in the posterior triangle of the neck and is not in the vicinity of the surgical field to remove this type of fistula. The lingual nerve lies at a higher level and is safe. The left recurrent laryngeal nerve arises from the vagus (X) nerve low in the neck before hooking around the subclavian artery and passing medially behind the common carotid artery to reach the groove between the trachea and the esophagus. As a result of this anatomy, it too is out of harm's way. The vagus (X) nerve lies in the carotid sheath behind, and somewhat between, the internal jugular vein and the common carotid artery. The fistula lies on the other side of the great vessels. At the carotid bifurcation, the vagus nerve is lateral to the fistulous tract and stands less chance of injury than does the hypoglossal nerve.

132. A 2-day-old male newborn is evaluated in the neonatal intensive care unit because of a "jaw deformity" and difficulty breathing. The patient was born at term following an uncomplicated pregnancy and delivery. He responds appropriately to stimulation. Examination shows micrognathia, glossoptosis, and a cleft palate. The infant demonstrates retractions while breathing. Pulse oximetry shows an oxygen saturation of 92%. Which of the following is the most appropriate initial management? A) CT scan of the head with three-dimensional reconstruction B) Endoscopic evaluation of the airway C) Prone positioning of the newborn D) Tongue-lip adhesion E) Tracheostomy

The correct response is Option C. The patient described has Pierre Robin sequence. These patients have micrognathia and glossoptosis; sometimes they will have a cleft palate in addition to the other findings. Most patients do not require operative intervention to correct their mandibular hypoplasia. The most important first step is airway control. These patients have airway obstruction secondary to the large size of their tongue relative to their diminutive mandible. Placing them in prone position allows for the tongue and jaw to fall forward, frequently alleviating their airway problems. Should this be insufficient, tongue-lip adhesion is a possibility. Many surgeons would consider a more thorough evaluation of the airway endoscopically and by CT scan to decide on subsequent management. Should the airway obstruction be tongue-based as expected, bilateral mandibular distraction could be considered. If lower airway anomalies are also present, then tracheostomy is more appropriate.

126. An otherwise healthy 50-year-old man is evaluated prior to excision of squamous cell carcinoma of the anterior aspect of the floor of the mouth. CT scan shows invasion into the lingual mandibular cortex. Lymph nodes are suspicious for metastases bilaterally. No distant metastases are noted. In addition to bilateral neck dissection, which of the following is the most appropriate treatment? A) Marginal mandibulectomy, reconstruction with radial forearm fasciocutaneous free flap, and postoperative radiation therapy B) Marginal mandibulectomy, reconstruction with split-thickness skin graft, and postoperative radiation therapy C) Segmental mandibulectomy, reconstruction with fibula osteocutaneous free flap, and postoperative radiation therapy D) Segmental mandibulectomy, reconstruction with radial forearm fasciocutaneous free flap and titanium plate, and postoperative radiation therapy

The correct response is Option C. The patient described has an advanced (stage IV) cancer of the floor of the mouth. The most appropriate treatment for the primary cancer is segmental mandibulectomy, reconstruction with a fibula osteocutaneous free flap, and postoperative radiation therapy. A marginal mandibulectomy is defined as excision of the alveolar process, sparing a portion of the mandible. It is performed when the cancer abuts or is adherent to the mandibular periosteum but does not invade the bone. Reconstruction after marginal mandibulectomy is performed by primary closure, secondary intention, skin grafting, or free flap, depending on the size and nature of the defect. For segmental mandibular defects of the anterior mandible, reconstruction with vascularized osseous or osteocutaneous flaps, such as the fibula osteocutaneous free flap, is preferred, particularly when postoperative radiation therapy is planned. Reconstruction of the anterior mandible using a titanium reconstruction plate and soft-tissue free flap or pedicled flap is associated with a high rate of complications. Postoperative radiation therapy results in good local control in patients with floor-of-mouth cancers, but it is associated with poor survival rates in advanced-stage cancers when used as a single treatment modality.

pic 147. A 66-year-old man comes for a follow-up examination 7 months after resection of a T4 N1 M0 squamous cell carcinoma in the region of the retromolar trigone, including alveolectomy, followed by soft-tissue reconstruction with a platysma flap. Postoperatively, he received radiation therapy to the primary tumor site (6 Gy) and to the neck bilaterally (64 Gy). He completed radiation therapy 5 months ago. Examination today shows a malodorous, tender area of exposed, soft bone at the operative site. A panoramic x-ray study (Panorex) is shown. Multiple biopsies are negative for recurrent carcinoma. Which of the following is the most appropriate management? A) Long-term intravenous antibiotic therapy B) Open reduction and internal fixation C) Segmental resection and vascularized tissue transfer D) Sequestrectomy

The correct response is Option C. The patient described has osteoradionecrosis of the mandible, a complication that occurs in up to 40% of patients receiving adjuvant radiation therapy for head and neck malignancies caused by hypoxia, hypovascularity, hypocellularity, and impaired collagen synthesis. The traditional definition is an area of exposed, irradiated bone that is nonhealing over 3 months. Treatment depends on the severity of the disease. Debridement and antibiotic therapy, plus or minus hyperbaric oxygen therapy, with soft-tissue reconstruction as needed, may be curative in up to 90% of cases of osteoradionecrosis limited to the alveolar ridge or mandible superior to the alveolar canal. When more extensive destruction of the mandible is present, or when there is a pathologic fracture, as seen in the scenario described, resection of all the necrotic bone and soft tissue is indicated, followed by reconstruction with vascularized bone and soft tissue. Successful healing occurs in up to 80 to 90% of patients with more extensive disease when treated in this way. Local flaps are of limited use for soft-tissue coverage because of the radiation.

140. An 18-year-old man is evaluated because of an overbite. Cephalometric analysis shows an SNA angle of 83 degrees (N 82 ± 3) and an SNB angle of 74 degrees (N 80 ± 3). Which of the following is the most likely underlying cause of this condition? A) Prognathic maxilla B) Retrognathic maxilla C) Prognathic mandible D) Retrognathic mandible

The correct response is Option D. An "overbite" (Angle class II malocclusion) may be caused by several different etiologies: a prognathic maxilla, a retrognathic mandible, or both; even a prognathic mandible with a more severely prognathic maxilla, or a retrognathic maxilla with a more severely retrognathic mandible, is possible. The patient described is exhibiting isolated mandibular deficiency, or retrognathia, which is characterized by a decreased sella-nasion-point B (SNB) angle combined with a normal sella-nasion-point A (SNA) angle. The SNA and SNB angles determine the position of the maxilla and mandible relative to the cranial base. The SNA angle measures the position of point A (subspinale) relative to the anterior cranial base with the normal value being 82 degrees plus or minus 3 degrees. The SNA angle is increased in maxillary prognathism and decreased in maxillary retrognathism. The SNB angle measures the position of point B (supramentale) relative to the anterior cranial base with the normal value being 80 degrees plus or minus 3 degrees. The SNB angle is increased in mandibular prognathism and decreased in maxillary retrognathism.

118. A 28-year-old man comes to the office because of fever and increasing pain 1 week after partial amputation of the right ear when he was bitten during a fight. The wound was irrigated and repaired in the emergency department. Temperature is 100.4°F (38.0°C). Examination shows erythema and swelling of the entire ear and a small area of dehiscence containing pus along the posterior suture line. Which of the following is the most important step in management? A) Application of wet-to-dry dressings B) Completion of the amputation with sparing of cartilage in a remote subdermal pocket C) Intravenous antibiotic therapy D) Irrigation, debridement, and packing the wound open E) Oral antibiotic therapy

The correct response is Option D. Auricular chondritis and perichondritis is a serious surgical infection requiring immediate surgical intervention as the primary course of treatment in traumatic cases. Culture swabs should also be obtained and will guide antibiotic therapy for associated cellulitis; however, broad spectrum coverage initially is appropriate. Chondritis complicating elective otoplasty is sometimes handled in a more stepwise approach, sometimes initiating intravenous antibiotics while removing a few sutures to allow drainage and/or insertion of a small irrigating catheter. Without a reasonable response in these cases, then standard open irrigation and debridement are done with removal of all sutures, and repeat otoplasty correction is deferred until several months after resolution of the infection. Antibiotics alone, orally or intravenously, for localized suppurative chondritis are likely to be ineffective without surgical treatment. Likewise, topical dressings without formal opening of the suture line for wide exposure and drainage of the infected cartilage would also be ineffective. Completing the amputation when the tissues still appear viable is overaggressive at this stage. If and when the majority of the tissue appears unable to sustain sufficient circulation to support its viability, then discarding precious tissue is justified. Salvaging the cartilage framework component in a remote subdermal pocket may be useful; however, the cartilage is infected in this scenario and would require caution, including thorough debridement and lavage with antibiotic solution and close monitoring of the bank.

109. A 59-year-old man comes to the emergency department because of erythema of a neck incision and salivary drainage from the wound 10 days after undergoing pharyngolaryngectomy with immediate hypopharyngeal reconstruction with a jejunal free flap to treat recurrent carcinoma of the larynx. He underwent radiation therapy for laryngeal cancer 3 years ago. Endoscopic evaluation shows a viable jejunal free flap. Which of the following is the most appropriate initial management? A) Debridement and anterolateral thigh free flap B) Debridement and ipsilateral pectoralis major myocutaneous flap C) Debridement of the wound edges and local flap advancement closure D) Local wound care E) T-tube decompression of the jejunal segment

The correct response is Option D. Complications of free jejunal transfer include thrombosis and flap loss, salivary fistula, and stricture. Thrombosis and flap loss typically occur in the first few days after surgery. Because of the poor ischemic tolerance of the jejunum flap, thrombosed flaps are rarely salvageable. In these instances, early debridement and repeat free flap reconstruction is the best approach. The instance of salivary fistula is approximately 10%, with the majority of patients having received prior radiation therapy. Postoperative salivary fistula after free jejunal transfer can usually be treated conservatively with maintenance of nothing by mouth (NPO) status, dressing changes, and wound care. Larger and more persistent leaks may respond to advancement of wound edges and local flap closure with T-tube decompression of the bowel segments, or pectoralis myocutaneous flap reinforcement of the wound closure. In the setting of a viable free tissue transfer, conservative measures are the most appropriate initial management of this complication.

pic 117. A 30-year-old man comes to the office for consultation regarding the facial abnormality shown. He reports that he first noticed a change 15 years ago and that the deformity has worsened since then. Medical history includes atrophy of the soft tissue and bone of the face. The facial atrophy in this syndrome progresses according to the specific distribution of which of the following nerves? A) Facial (VII) B) Glossopharyngeal (IX) C) Hypoglossal (XII) D) Trigeminal (V) E) Vagus (X)

The correct response is Option D. Parry-Romberg syndrome is a progressive hemifacial atrophy that follows a specific distribution of one or more branches of the trigeminal nerve (cranial nerve V). It involves the skin, soft tissue, cartilage, and bone and was first described in 1825 by Parry and then in 1846 by Romberg. The epidemiology shows a female-to-male ratio of 1.5:1. It can appear in early infancy or adolescence. The etiology is unclear, and theories include a genetic alteration in the embryogenesis of the central nervous system, loss of the cervical sympathetic nerve after neuritis, and viral or bacterial infection.

113. A 48-year-old woman is evaluated because of a 2.5-cm defect on the dorsum of the nose after undergoing Mohs micrographic surgery for morphea-type basal cell carcinoma. Examination shows a defect extending from the dorsum of the nose to the nasal sidewall on the right and to the upper borders of the nasal ala. The defect includes the full thickness of skin, subcutaneous tissue, and nasal muscle. The perichondrium of the lower lateral and upper lateral cartilages is missing. Which of the following is the most appropriate reconstructive technique? A) Acellular dermis covered by a thin split-thickness skin graft B) Bilobed flap C) Full-thickness skin graft D) Paramedian forehead flap E) Superiorly based nasolabial flap

The correct response is Option D. There are a multitude of techniques for reconstructing nasal defects. The defect in the scenario described is 2.5 cm and full thickness in nature. A paramedian forehead flap would be the most appropriate means of reconstruction for this defect. Superiorly and inferiorly based nasal labial flaps are a favorite technique for repair of alar defects up to 2 cm in diameter. They can also be used for reconstruction of the columella and nasal lining. In general, small defects that are less than 5 mm in diameter can be closed primarily. Defects ranging from 5 to 10 mm, particularly on the concave portions of the nose and upper lateral sidewall, can be treated with skin grafts or left to heal by secondary intention. The larger the defect, the less aesthetic the skin graft will appear. Lesions ranging from 1 to 1.5 cm and confined to the nose are best treated by local flaps, such as bilobed flaps, dorsal nasal flaps, or banner flaps. Defects larger than 1.5 cm are often too large for reconstruction with a local flap and are best reconstructed with a paramedian forehead flap. The skin color match of the forehead flap is excellent. Although this is a staged reconstruction requiring division of the flap pedicle as well as potential step for thinning of the flap, the forehead flap provides an excellent option for aesthetic reconstruction of the nose for larger defects.

114. When reattaching the medial canthal ligaments during a transnasal canthal wiring procedure, which of the following is the most appropriate placement of the drill holes with respect to the lacrimal fossa? A) Anterior and inferior B) Anterior and posterior C) Posterior and inferior D) Posterior and superior

The correct response is Option D. When treating congenital and traumatic deformities of the naso-orbital-ethmoid region, reconstruction and reattachment of the medial canthal tendons are often necessary. Overcorrection with this procedure is essentially impossible, and every effort should be made to prevent relapse and recurrent telecanthus. To this end, the transnasal wires containing the medial canthus should be placed through drill holes positioned posterior and superior to the posterior crest of the bony lacrimal fossa.

pic 127. An 8-year-old girl (shown) is brought to the office because of bilateral microtia. She has severe conductive hearing loss bilaterally. Her middle ear ossicles are fused, and she is not a good candidate for middle ear reconstruction. Reconstruction of the ears using autologous rib cartilage and placement of bone-anchored hearing aids (BAHAs) are planned. Which of the following is the most appropriate time for placement of the BAHAs? A) During elevation of the carved-rib framework B) During placement of the carved-rib framework C) During rotation of the earlobe D) Following completion of the ear reconstruction E) Prior to the ear reconstruction

The correct response is Option D. (Please note that this pictorial appears in color in the online examination) Patients with severe microtia almost invariably have conductive hearing loss resulting from abnormalities of the middle ear. In some patients, the auditory ossicles are sufficiently formed to allow attempted reconstruction of the auditory canal and the middle ear ossicles. Eligibility for this procedure depends upon the development and shape of the auditory ossicles (Jahrsdoerfer grade), as determined by a specialized CT scan of the temporal region. In patients with unilateral microtia and normal contralateral hearing, assistive devices or middle ear reconstruction are rarely indicated and would serve only to improve sound localization. In these patients, protection of hearing in the normal ear is paramount. When bilateral conductive hearing loss is present, methods to improve hearing competence include external hearing aids, bone-anchored hearing aids (BAHAs), and middle ear reconstruction in selected patients. The BAHA is affixed in the mastoid region using an osseointegrated implant. Since this can compromise the integrity and mobility of the skin envelope that will cover the autologous rib cartilage framework, it is often recommended that placement of the BAHA device be deferred until after the ear construction is complete.

pic 135. A 2-month-old female infant is brought to the office because her parents are concerned about the flat appearance of her forehead that they first noticed 2 weeks ago. Physical examination shows flattening of the right side of the forehead and left side of the occiput, and the left ear is positioned farther forward than the right. A photograph is shown. Which of the following is the most appropriate initial management? A) Calvarial vault remodeling B) CT scan C) MRI D) Placement of a molding helmet E) Repositioning exercises

The correct response is Option E. (Please note that this pictorial appears in color in the online examination) The physical findings described are consistent with deformational (positional) plagiocephaly. In contradistinction to craniosynostosis, deformational plagiocephaly will demonstrate deviation of the nasal root away from the side of the forehead with flattening, and the supraorbital rim will be depressed or lowered. Physical findings are sufficient in the scenario described to diagnose the child without CT scan. Occupational and physical therapy should be instituted to encourage behaviors such as head-turning to the right and tipping the top of the head to the left shoulder. In cases that are either severe or are diagnosed late, molding helmets may be advisable.

122. A 4-year-old girl is undergoing mandibular reconstruction involving the temporomandibular joint. Use of which of the following types of bone graft is most likely to result in overgrowth on the reconstructed side? A) Calvaria B) Fibula C) Iliac crest D) Radius E) Rib

The correct response is Option E. A variety of bone sources may be used in reconstructing the deficient mandible in pediatric patients. The majority of reconstructions are done for congenital anomalies involving the mandible, such as hemifacial microsomia and Treacher Collins syndrome. However, bone grafting may also be needed following tumor resection and traumatic loss. Cortical bone may be harvested from the iliac crest, calvaria, rib, radius, and fibula. Typically, when rib bone is used to reconstruct the mandible, including the temporomandibular joint, a cartilaginous cap is left on the end of the rib when it is harvested. This allows for growth of the rib as the child grows, but it can also result in overgrowth. This overgrowth can result in further asymmetry and malocclusions.

111. A 25-year-old woman who is pregnant with her second child comes to the office for consultation regarding the risk that the child will have a cleft lip and palate. She has a history of a cleft lip and palate, and her first child also has a cleft lip and palate. The father has no history of cleft lip or palate. Which of the following best represents the likelihood that the patient's second child will be born with a cleft lip and palate? A) 2 % B) 4 % C ) 5% D ) 10% E) 1 4 %

The correct response is Option E. An affected parent with one affected child has a 14% risk for future offspring to have a cleft lip and palate. If both parents are not affected, and their first child has a unilateral defect, the risk would be 2.7% for the next child and 5.4% if the first child had a bilateral defect. If both parents were unaffected and had two affected children, then the risk for the subsequent pregnancy to result in a cleft lip and palate would be 10%.

pic 149. A 1-year-old boy has had the anomaly shown since birth. Which of the following syndromes is the most likely diagnosis? A) Apert B) Crouzon C) Goldenhar D) Saethre-Chotzen E) Treacher Collins

The correct response is Option E. Bilateral lower eyelid colobomas are commonly found in patients with Treacher Collins syndrome (TCS). TCS is also known as mandibulofacial dysostosis, first and second branchial arch syndrome, and Franceschetti-Zwahlen-Klein syndrome. Edward Treacher Collins described the syndrome in 1900. It is autosomal dominant with variable penetrance and has an incidence of 1 in 7000 live births. TCS has significant dysmorphology, which includes lower eyelid colobomas, cleft and hypoplastic zygomas, cleft lateral orbit, hypoplastic mandible, lateral canthal vertical dystopia, antimongolian palpebral fissure, ear deformities, long anterior sideburns, anterior open bite, cleft palate, and macrostomia. Crouzon, Apert, and Saethre-Chotzen syndromes involve craniosynostosis, typically bicoronal. Patients with these syndromes also have underdevelopment of the mid face. They do not have eyelid abnormalities. Patients with Apert syndrome also may have a cleft palate and syndactyly of the hands and feet. Goldenhar syndrome involves epibulbar dermoids of one or both eyes and underdevelopment of one or both sides of the face. It is also known as hemifacial and bifacial microsomia. The soft tissue and the bone are hypoplastic.

pic 102. A 12-month-old boy is referred by his pediatrician for possible craniosynostosis. He is healthy and has achieved developmental milestones appropriately. His parents report that he has a ridge on his forehead that they first noticed when he was 3 months of age. Physical examination shows a palpable midline ridge with normal facial contour. CT scans obtained by his pediatrician are shown. Which of the following is the most appropriate management? A) Diagnostic plain x-ray studies B) Endoscopic strip craniectomy and postoperative helmet therapy C) Fronto-orbital advancement D) Serologic testing for mutations of fibroblast growth factor receptors 1, 2, and 3 E) Observation

The correct response is Option E. Craniosynostosis results in characteristic changes to the cranium. The metopic suture is different because unlike other calvarial sutures that ultimately fuse in the third decade of life, it normally closes before 12 months of age. Accordingly, the finding of a closed suture at this age is not abnormal. The phenotype associated with premature (or pathological) metopic fusion is trigonocephaly ("triangle head"). Patients present with a midline forehead prominence, a variable degree of frontal and lateral orbital narrowing, bilateral parietal widening, and hypotelorism. In contrast, the presence of an isolated metopic ridge is a normal variant. It is the shape of the fronto-orbital region that determines the need for operative intervention. The CT scans clearly demonstrate metopic fusion; plain x-ray studies are not necessary to confirm the diagnosis. Because the patient described has only an isolated metopic ridge (not trigonocephaly), operative treatment is not warranted. Additionally, endoscopic strip craniectomy is generally not effective after 3 months of age. Mutations in fibroblast growth factor receptors 1, 2, and 3 have been identified in unilateral and bilateral coronal synostosis but not in isolated metopic synostosis

143. The vector of commissure movement in a free gracilis muscle flap for facial reanimation should simulate the pull of which of the following facial muscles? A) Buccinator B) Levator labii superioris C) Risorius D) Temporalis E) Zygomaticus major

The correct response is Option E. Free gracilis muscle transfer is a common method to produce a smile in patients who have complete facial nerve paralysis. It has several properties that make it ideal for this purpose: it is thin, has good contractility, leaves no functional deficit after muscle harvest, and has a relatively long motor nerve. The inset of the muscle, including appropriate tensioning and orientation, are critical for success. The muscle is attached proximally to the body of the zygoma or the temporalis fascia and distally to the orbicularis oris muscle near the modiolus just lateral to the oral commissure. Although there can be some variation in flap positioning, the desired vector of pull most closely simulates the normal pull of the zygomaticus major muscle. The temporalis and buccinator muscles are not involved in smiling. The levator labii superioris originates from the anterior zygoma and inserts near the orbicularis oris muscle in the upper lip. The vertical direction of this makes it a powerful vertical elevator of the upper lip. The risorius does not elevate the oral commissure but instead pulls the corner of the mouth in a nearly horizontal direction.

108. A 20-year-old man comes to the office because he has had paraesthesia of the anterior lateral aspect of the tongue since undergoing removal of the mandibular third molars 3 weeks ago. The most likely cause is injury to which of the following nervous structures? A) Chorda tympani B) Facial C) Glossopharyngeal D) Hypoglossal E) Lingual

The correct response is Option E. General sensation of the anterior two thirds of the tongue is supplied by the lingual nerve, which is a branch of the mandibular division of the trigeminal. Taste in the anterior two thirds of the tongue is supplied by the chorda tympani from the facial nerve. The chorda tympani joins the lingual nerve and runs anteriorly in its sheath. The glossopharyngeal nerve supplies the mucosa of the posterior one third of the tongue. The hypoglossal nerve is the motor nerve to the tongue, and the facial nerve is the motor nerve to the face.

133. A 30-year-old woman comes to the office because of a 1-year history of a clicking sensation when she opens her mouth. She was involved in a motor vehicle collision in which her face struck the steering wheel 1 year ago. Physical examination shows midline dental structures without deviation. Which of the following is the most likely cause of this patient's condition? A) Disruption of the lateral pterygoid muscle B) Foreign body within the joint space C) Malunion of a coronoid fracture D) Nonunion of a condylar fracture E) Subluxation of the articular disk

The correct response is Option E. Motion at the temporomandibular joint (TMJ) is best appreciated by placing one's fingers either inside the external auditory canal or just anterior to it. The sensation of clicking when the jaw is repeatedly opened and closed is usually caused by subluxation of the articular disk. The disk normally lies centrally between the two joint spaces. Conservative treatment involves adjustment of the patient's bite with a splint, anti-inflammatory drugs, and physical therapy. Surgical treatment is reserved for patients who fail conservative therapy. Air within the joint space may occur following open fractures of the mandibular condyle. The presence of a foreign body within the joint space produces pain and decreased range of motion rather than clicking. Similar symptoms are also noted in patients with degenerative disease affecting the TMJ.

123. A 14-year-old boy is referred by his pediatrician for evaluation because of a 2- year progressive enlargement of the right side of the face. History includes capillary malformation since birth and seizures. Physical examination shows enlargement of the soft tissues and the maxilla on the right and macular capillary staining of the forehead, eyelid, and cheek. Which of the following syndromes is the most likely diagnosis? A) Cobb B) Fibrous dysplasia C) Klippel-Trénaunay D) Nevus flammeus neonatorum E) Sturge-Weber

The correct response is Option E. Sturge-Weber syndrome is characterized by capillary malformations in the distribution of the ophthalmic or the maxillary division of the trigeminal nerve. It may be unilateral or bilateral. Often there is also gradual enlargement and hypertrophy of the cheek, lip, and maxilla, and occasionally there is gradual enlargement and hypertrophy of the mandible. MRI may show additional vascular anomalies of the leptomeninges and choroid plexus. Cobb syndrome consists of a capillary malformation in the midline scalp region overlying an encephalocele or in the skin posterior to an area of dysraphism in the cervical or lumbosacral spine. Fibrous dysplasia is an overgrowth syndrome of bones that is caused by abnormal proliferation of bone-forming mesenchyme. It does not involve soft-tissue hypertrophy nor is it associated with vascular malformations. Albright syndrome is a specific variety of polyostotic fibrous dysplasia and includes endocrine abnormalities and café-au-lait spots. Klippel-Trénaunay syndrome is a capillary-lymphatic-venous malformation typically involving hypertrophy of the extremities and sometimes the thorax of one side of the body. It does not involve the head and neck. The skin surface shows deep red staining with hemolymphatic vesicles. A pathognomonic feature of this condition is the presence of the embryonal lateral vein of Servelle in the lower extremity. Parkes-Weber syndrome is similar to Klippel-Trénaunay but is confined to an upper or lower extremity. Overgrowth of the extremity is characteristic along with microscopic arteriovenous fistulas, and unlike Klippel-Trénaunay, lymphatic anomalies are rare. It does not involve the head or neck. Nevus flammeus neonatorum is an entity that behaves more like a hemangioma and not a vascular malformation in that it is usually gone or faded by 1 year of age. It is typically located on the upper face and posterior neck but is not associated with soft-tissue or bony hypertrophy.

121. A 77-year-old man comes to the office because of a 5-month history of a bruise- like patch of skin on the forehead that has enlarged gradually in a centrifugal pattern. Examination shows a 3 × 4-cm irregular ovoid lesion confined to the superior forehead and anterior scalp with small nodular areas of ulceration. A specimen obtained on biopsy shows angiosarcoma. Which of the following is the most appropriate initial management? A) Chemotherapy B) Cryotherapy C) External beam radiation therapy D) Intralesional injection of interferon-alfa E) Wide excision

The correct response is Option E. The lesion described is a classic angiosarcoma. The most common form of this disease is cutaneous angiosarcoma without lymphedema in elderly patients. The highest incidence occurs in patients over 70 years of age, with a 2:1 male-to-female predilection. At least half of these tumors involve the head and neck. Lesions typically present as deceptively benign-appearing bruise-like patches on the central face, forehead, or scalp. Facial swelling and edema may be present. More advanced lesions are violaceous and contain elevated nodules that bleed easily. Ulceration may be present. The lesion gradually spreads and eventually covers large portions of the head and neck. Prognosis is invariably poor with a less-than-15% survival rate over a 5-year period. Surgical excision with wide margins and immediate reconstruction is the most appropriate initial management. In spite of aggressive initial surgical management, the goal of histologically negative margins is extremely difficult to achieve in this disease process. Given the poor results obtained with surgery alone, radiation therapy has been offered as the best possible adjuvant therapy. However, there is no proof that radiation therapy provides benefit. Adjuvant chemotherapy offers no statistically significant benefit for survival, although one agent that appears to have some activity against this disease is paclitaxel. Immunomodulators, such as interferon or interleukin, may be successful alternatives to chemotherapy, but data are lacking. There is no role for cryotherapy in the management of angiosarcoma.

134. A 5-year-old boy is brought to the office because of a 10-day history of inflammation of a midline neck mass that his parents first noticed 1 year ago. Physical examination shows a 35-mm mass just inferior to the hyoid bone. Which of the following is the most likely diagnosis? A) Branchial cleft remnant B) Lingual thyroid gland C) Mucoepidermoid carcinoma D) Reactive lymph node E) Thyroglossal duct cyst

The correct response is Option E. The patient described has a thyroglossal duct cyst. Thyroglossal duct cysts can form anywhere along the thyroglossal duct, which extends from the foramen cecum of the tongue to the final position of the thyroid gland in the neck, below the laryngeal cartilage. Normally, the thyroglossal duct atrophies and disappears. However, a remnant of it may persist and form a cyst in the tongue or anterior midline of the neck, most commonly inferior to the hyoid bone. Thyroglossal duct cysts are often asymptomatic unless they become infected, as this one has. Reactive lymph nodes are the most common neck mass in children. They are usually found laterally in the submandibular and jugulodigastric areas. Branchial cleft remnants (sinuses and cysts) arise from the branchial apparatus. They are also located laterally, along the anterior border of the sternocleidomastoid muscle, usually just inferior to the angle of the mandible. Lingual thyroid glands are a type of ectopic thyroid located within the tongue. Ectopic thyroid glands can be located along the course of the thyroglossal duct and are a result of failure of the thyroid to descend. Unlike thyroglossal duct cysts, they represent the only thyroid tissue present in the patient. Mucoepidermoid carcinomas are salivary gland malignancies found in children, and they most commonly appear within the parotid gland.

pic (Please note that this pictorial appears in color in the online examination) 115. A 17-year-old girl is brought to the emergency department after she was hit in the left eye with a batted softball. A photograph is shown. Physical examination shows increased intraorbital pressure and decreasing visual acuity. Review of the CT scan confirms fracture of the orbital floor. Which of the following is the most appropriate immediate management? A) Administration of mannitol followed by exploration of the orbital floor B) Anticoagulation C) Exploration of the orbital floor and repair with a bone graft D) Exploration of the orbital floor and repair with synthetic material E) Lateral canthotomy and cantholysis

The correct response is Option E. Immediate lateral canthotomy and cantholysis are the most appropriate management of the condition described. Retrobulbar hematoma or orbital hemorrhage can follow either a direct injury to the orbital contents or a fracture that involves surrounding bones. Symptoms and signs include pain, reducing visual acuity, history of trauma, periorbital/lid hematoma, chemosis, proptosis, raised intraocular pressure, and ophthalmoplegia. There is a narrow therapeutic interval of 90 minutes before permanent damage to vision may occur. Immediate exploration of the orbital floor is contraindicated when a significant retrobulbar hematoma resulting in increased intraorbital pressure is present. A delayed repair of the orbital fracture, generally within one week of the trauma, is performed if necessary. Medical treatment of raised intraorbital pressure with mannitol or dexamethasone should be regarded as an adjunct to surgery. The first line of treatment is surgical, and lateral canthotomy at the bedside is the most effective immediate treatment for increased intraorbital pressure. Anticoagulation is contraindicated because of suspected bleeding.

106. A 1-year-old girl is brought to the office because of a 1-cm hemangioma over the right brow and upper eyelid. The parents report that they first noticed the lesion shortly after birth and that it has enlarged since then. Results of ophthalmologic examination are normal bilaterally. Which of the following is the most appropriate management at this time? A) Patching of the contralateral eye B) Pulsed-dye laser therapy of the lesion C) Surgical resection D) Systemic and intralesional injection of a corticosteroid E) Observation

The most appropriate action at this time is close observation with frequent vision and refractive examination by an ophthalmologist. Hemangiomas are the most common tumor of infancy, with a predilection for the head and neck and the upper eyelid. They are more common in females, usually noted in the weeks following birth, and are characterized by a rapid proliferative phase of 6 to 12 months. This phase is followed by a period of quiescence, then slow involution beginning the second year of life. Most hemangiomas are initially managed by observation. When a hemangioma interferes with the visual axis or airway, medical or surgical intervention may be indicated. Periocular hemangiomas may permanently affect vision by causing ptosis of the eyelids, resulting in visual field obstruction, strabismus, or anisometropia (a condition in which the two eyes have unequal refractive power). Each of these may, in turn, result in astigmatism, amblyopia (impairment of vision without an organic cause within the eye, in this case, caused by deprivation of visual stimuli centrally), or blindness. As this particular lesion is not interfering with the child's vision and, based on her age, is likely to enter a quiescent phase, observation is the most reasonable course of action. Cosmetic and functional results of spontaneous involution, particularly for smaller lesions, are often superior to those obtained by invasive treatment. Systemic corticosteroids can have multiple side effects, and intralesional corticosteroids carry the risk of blindness secondary to central retinal artery occlusion. Patching of the contralateral eye is usually performed after debulking of a hemangioma to treat amblyopia.