Craniofacial Flashcards
(74 cards)
A 2-month-old female infant is brought in by her parents for evaluation of a 4-cm,
enlarging infantile hemangioma on her left cheek that does not obstruct her vision.
Which of the following is the most appropriate management?
A ) Intralesional corticosteroid injection
B ) Laser ablation
C ) Oral propanolol therapy
D ) Surgical excision
E ) Observation
The correct response is Option C.
Systemic propanolol therapy has quickly become a safe and effective treatment for
hemangioma and is well tolerated with few side effects. It is considered the first line
medical treatment of infantile hemangiomas at many medical centers. Surgical treatment is
generally reserved for lesions that are refractory to medical management which present a
functional impairment such as affecting or obstructing vision. Laser ablation and
intralesional corticosteroid injection are less effective in several meta-analyses and some
randomized controlled trials. Observation is reasonable for small, stable lesions in
cosmetically non-sensitive areas.
2019
Cranial bone graft - when does diploic space begin to form in kid?
Approximately 5 years old
An 8-year-old boy is brought to the office with a congenital abnormality of the ear. A
photograph is shown. When the ear is pulled on traction, the upper pole cartilage
becomes visible under the skin. This abnormality is most likely caused by which of the following?
-pic looks like cryptotia-
A ) Anomaly of the intrinsic postauricular muscles
B ) Effacement of the scaphofossa
C ) Formation of a third antihelical crus
D ) Hemifacial microsomia
E ) Intrauterine pressure
Correct answer is A.
This patient has cryptotia of the ear. It is also known as pocket ear. The upper part of the ear
is adherent and the cartilage is buried under the skin in a pocket. Other deformities may be
present, such as a missing upper sulcus, underdeveloped scapha, and antihelical crura.
It is caused by an anomaly of the intrinsic oblique and transverse auricular muscles.
Surgical treatment requires release from the pocket and resurfacing of the post- and
retroauricular defects. In some cases otoplasty may be required for normalization. A number
of techniques are described.
Intrauterine pressure may cause ear deformities, which may spontaneously resolve or are amenable to neonatal molding techniques. It does not cause cryptotia.
Stahl ear is associated with formation of a third antihelical crus.
Hemifacial microsomia is associated with microtia. Microtia is sometimes the only
manifestation of hemifacial microsomia, but subtle clinical findings are often present, such
as mild facial nerve weakness or soft tissue hypoplasia on the involved side.
A 47-year-old Caucasian man comes to the office regarding a painful enlarging mass at
the base of the tongue. He does not smoke cigarettes. The lesion measures 4.5 cm. A
biopsy of the lesion is performed and shows (+) p16 staining, nonkeratinized squamous
cell carcinoma. Further imaging and workup demonstrate an ipsilateral solitary lymph
node measuring 2.3 cm. No distal metastatic disease is found. Which of the following
best describes the stage of his disease?
A ) Stage 1
B ) Stage 2
C ) Stage 3
D )Stage 4
The correct response is Option B.
The correct answer is Stage 2. Previously, this patient would have been Stage 3. The
American Joint Committee on Cancer (AJCC) revised its staging system for squamous cell
cancers that stain p16 positive. These lesions are related to the human papillomavirus (HPV) and have been found to be less virulent tongue base or oropharyngeal cancers. Recent studies have demonstrated that 5 year survival difference for patients with
Stage 4 disease as <50% for HPV-negative patients and >70% for HPV-positive patients,
thus prompting the AJCC to study and revise the staging system for HPV-positive
oropharyngeal cancers. These lesions tend to be more sensitive to radiation therapy and
chemoradiation and a better prognosis overall. Patients with HPV-related squamous cell
cancers tend to be younger, male, and Caucasian. HPV-related squamous cell cancers now represent the majority of newly diagnosed oropharyngeal carcinomas in the United States.
This new staging system for HPV (+) related cancers went into effect 1/1/2017.
A 22-year-old man is evaluated because of a painless, firm, unilateral enlarging mass
of the body of the mandible. He denies trauma to the area and he has excellent oral
hygiene. His dentist performed fine needle biopsy that showed multinucleated giant
cells. CT scan shows a radiolucent bone lesion with an expanded cortex. Which of the
following is the most appropriate next step in management?
A ) Incisional biopsy
B ) Partial mandibulectomy with free margins
C ) Radiation therapy
D ) Resection and curettage
E ) Sclerotherapy
The correct response is Option D.
This patient has an aneurysmal bone cyst (ABC). These lesions may be related to giant cell
granulomas. These lesions are most common in the long bones with 1.9% of them being
reported in the mandible.
The correct answer is resection and curettage. In a study of 120 ABC cases, resection and
curettage was reported to have a 91.8% success rate (recurrence occurred in 11 out of 120
cases). Incomplete resection is hypothesized to be a cause of recurrence. Recurrence was
not related to histopathologic parameters.
Pathologically, these lesions are a pseudocyst comprised of multinucleated giant cells,
woven trabecular bone with caverns, and sinusoids lacking endothelium. Recurrence can be treated with repeat excision curettage, open packing, or block resection. These lesions are
quite vascular and typically bleed until resected, so expeditious removal is recommended
(transfusion with packed red blood cells has been reported in the literature).
Incisional biopsy would be diagnostic but is not recommended for these lesions given their
vascularity and the surgeon’s inability to control the bleeding.
Sclerotherapy is recommended for vascular malformations such as arteriovenous
malformations, venous malformations, or lymphatic malformations. This lesion would not
be responsive to this type of therapy. Partial mandibulectomy with free margins is the preferred treatment for lesions like
ameloblastomas. Given the efficacy of excision and curettage, mandibulectomy is not the
recommended first line treatment for this diagnosis. It can be employed in recurrences
(although as stated above, less aggressive interventions are typically employed first).
In a patient undergoing reconstructive cranioplasty, an increased rate of complications
is most likely if which of the following is present?
A ) Frontal location
B ) Occipital location
C ) Parietal location
D ) Sphenoidal location
E ) Temporal location
The correct response is Option A.
Early decompressive craniectomy is a life-saving maneuver for certain traumatic
brain injuries and can be performed far forward in the theater of war. Patients
treated with decompressive craniectomy for combat injuries are a unique
understudied population. Outcome of treatment of this patient cohort has been
previously reported using a standardized cranial defect treatment protocol using
custom alloplast implants. Two subgroups of patients (large endocranial dead
space and frontal orbital bar injuries) were identified as often having higher rates of
complications than other cranial reconstruction cohorts.
Which of the following structures contributes to the formation of the tragus?
A ) First branchial arch
B ) First branchial cleft
C ) Second branchial arch
D ) Second branchial cleft
The correct response is Option A.
The first branchial arch contributes to the formation of the tragus and anterior helix.
The first branchial cleft is incorrect. It gives rise to the external auditory canal.
The second branchial arch is incorrect. It contributes to the formation of the majority of the
external ear – the antitragus, remainder of the helix, antihelix, and crura all arise from the
second branchial arch.
The second branchial cleft is incorrect. It is typically obliterated during development, but
may persist in the form of a second branchial cleft cyst.
An 18-month-old child is brought to the office after undergoing fronto-orbital
advancement for metopic craniosynostosis. Which of the following is the earliest age
the surgeon can order the x-ray studies and expect to be able to see frontal sinus
development?
A ) 1 year
B ) 2 years
C ) 4 years
D ) 6 years
E ) 10 years
The correct response is Option D.
Frontal sinus development is associated with specific age-related periods of growth of the
skull. The frontal sinus is absent at birth and during the initial phase of growth of the skull.
The sinus is visible only in x-ray studies at the end of the first period of skull growth. This
is the time when the endocranial table of the skull ceases to grow and conforms to the
general shape of the brain. This is not seen on x-ray studies until 6 years of age or 72
months.
A female infant is born with severe Treacher Collins syndrome and bilateral Pruzansky
III mandible (absence of condyle). Tracheostomy is performed for respiratory distress.
Which of the following surgeries is most likely to allow decannulation?
A ) Alloplastic condylar reconstruction
B ) Bilateral sagittal split osteotomy
C ) Costochondral rib grafts
D ) Mandibular distraction
E ) Tongue-lip adhesion
The correct response is Option C.
Patients with Treacher Collins syndrome may have a varied presentation. The mandible
hypoplasia may be mild or severe. In this case the patient has no temporomandibular fossa
or condyles. Tongue-lip adhesion and mandibular distraction are used in severe Pierre
Robin sequence. Because of the absence of condyles and temporal mandibular joints,
distraction, and sagittal split osteotomy are not the best options. A costochondral graft will
provide better airway support and can also be distracted in the future.
Which of the following statements is correct about Tessier clefts No. 3, No. 4, and No. 5?
A ) Tessier No. 3 involves the alveolar ridge, while Tessier No. 5 does not
B ) Tessier No. 3 is medial to the infraorbital nerve, while Tessier No. 4 is lateral
C ) Tessier No. 3 only affects the oral region, while Tessier No. 4 only affects the orbital
region
D ) Tessier No. 4 involves the piriform aperture, while Tessier No. 5 does not
E ) Tessier No. 4 is medial to the infraorbital nerve, while Tessier No. 5 is lateral
The correct response is Option E.
Tessier No. 3 and No. 4 are medial to the infraorbital nerve, but Tessier No. 5 is lateral.
Tessier No. 3 involves clefts of the nose, orbit, and lip (naso-oral-ocular cleft), whereas
Tessier No. 4 involves the lip and orbit (oral-ocular cleft), and the nose is uninvolved.
Tessier No. 5 involves oral, cheek (maxillary sinus), and orbital cleft and is the rarest.
A newborn male is brought to the tertiary multidisciplinary referral center for evaluation
of anorectal malformation, tracheoesophageal fistula and absent right thumb. Which of
the following associated VACTERL diagnoses is the best predictor of inpatient
mortality?
A ) Aniridia with brain stem hypoplasia
B ) Anomalies of spine or vertebrae
C ) Cardiac disease
D ) Renal or urinary anomaly
E ) Tracheal stenosis with strido
The correct response is Option C.
Anomalies of the spine or vertebrae (V), anorectal malformations (A), congenital cardiac
anomalies (C), esophageal atresia/tracheoesophageal fistula (TE), renal and urinary abnormalities (R), and limb lesions (L) frequently co-occur and are recognized as
VACTERL anomalies. VACTERL association is typically diagnosed in the presence of at
least three characteristic features in the absence of evidence for an overlapping condition, and is estimated to occur in approximately 1 in every 10,000 to 40,000 live births. The presence of either anorectal malformation or esophageal atresia alone generally triggers a
workup for associated VACTERL diagnoses because of their significant impact on
morbidity and mortality. For example, in a large cohort of children undergoing surgical
repair of anorectal malformations, Lautz et al. found associated VACTERL diagnoses
including congenital heart disease in 40.4%, renal or internal urinary disease in 34.7%,
spinal or vertebral anomalies in 31.4%, esophageal atresia/tracheoesophageal fistula in 7%, and limb defects in 5.6%. The most common limb defects in VACTERL association include
poorly developed or missing thumbs, or underdeveloped forearms and hands, polydactyly,
syndactyly, and reduction deformities of the lower limb.
Independent predictors of mortality in any patient with VACTERL association include
congenital heart disease (greatest for those who require cardiac surgery than those with a
diagnosis but no operation), birthweight < 2 kg, and black race. Of note, the association
between cardiac disease and higher mortality has been reproduced in several studies.
Aniridia, brain stem hypoplasia, and tracheal stenosis with stridor are not primary
characteristics of VACTERL association.
2019
Patients with unilateral cleft lip and associated nasal deformities have each of the following findings EXCEPT
(A) attenuation and inferior positioning of the lower lateral cartilage on the side of the cleft
(B) elongation of the philtrum
(C) insertion of the orbicularis oris muscle into the cleft margin and alar wing
(D) outward rotation and projection of the premaxilla
(E) unilateral shortening of the columella
The correct response is Option B.
In patients who have unilateral cleft lip and associated nasal deformities, the premaxilla is rotated and projected outward, and the lateral maxillary element is collapsed and retropositioned. The inferior edge of the septum lies outside of the vomer groove, while the nasal spine is located in the floor of the normal nostril. The affected columella is 25% to 50% shorter than the unaffected side. The lower lateral cartilage is attenuated, and the nasal dome lies separate, below the opposite cartilage. The alar base is flared and rotated outward, and the vestibular lining is deficient on the side of the cleft.
With regard to the lip deformities, the philtrum termination of the orbicularis oris muscle in the lateral lip is shortened at the margin of the cleft; at this point, the muscle inserts into the alar wing. The muscles between the philtral midline and the cleft are hypoplastic. Two thirds of the cupid’s bow is preserved, as well as one philtral column and a dimple hollow.
A 15-year-old patient with a left unilateral cleft lip and palate is evaluated because of a congenitally absent left lateral incisor. The patient’s orthodontist recommended orthodontic canine substitution in the lateral incisor space rather than space maintenance. Relative to patients who do not undergo canine substitution, which of the following best describes this patient’s post-orthodontic risk for malocclusion?
A) Decreased risk for Angle class II malocclusion
C) Decreased risk for Angle class III malocclusion
C) Increased risk for Angle class II malocclusion
D) Increased risk for Angle class III malocclusion
E) No change in risk for Angle class malocclusion
The correct response is Option D.
Patients with complete cleft lip that includes the alveolus have a higher risk for lateral incisor tooth agenesis. Both congenital absence and orthodontic canine substitution are independently associated with an increased risk for maxillary hypoplasia, Angle class III malocclusion, and the subsequent need for orthognathic surgery.
2024
A 17-year-old girl with a history of bilateral cleft lip and palate comes for evaluation. Physical examination shows severe mid face deficiency and Angle class III malocclusion with 10 mm of negative overjet. Le Fort I distraction using an internal distractor is recommended. Compared with conventional Le Fort I maxillary advancement, Le Fort I maxillary distraction is most likely to result in which of the following for this patient?
A) Decreased complication rate
B) Decreased risk for relapse
C) Improved psychosocial outcomes
D) Increased predictability of final occlusion
E) Increased risk for postoperative speech dysfunction
The correct response is Option B.
Le Fort I distraction is a powerful tool to address Angle class III malocclusion in patients with cleft lip and palate. Numerous studies have shown that distraction yields improved speech outcomes when controlling for the degree of advancement, while also decreasing the rate of relapse, as compared with conventional distraction. However, final occlusion with internal maxillary distraction is less predictable than external distraction or conventional maxillary advancement surgery. Complication rates and psychosocial outcomes have not been shown to differ between these techniques.
2024
An otherwise healthy 8-year-old girl is referred by her pediatrician because of poor speech intelligibility that is affecting her school performance. Physical examination shows no abnormalities in the patient’s facial features. Intraoral examination shows a bluish discoloration in the midline palate. On speech analysis, the patient is hypernasal and unable to produce the consonants p, b, and g. Which of the following is the most likely diagnosis?
A) Arterial venous malformation
B) Chronic tonsillitis
C) Palatal neoplasm
D) Submucous cleft palate
E) Torus palatini
The correct response is Option D.
Patients with submucous cleft palate may have delayed presentation to cleft centers due to undiagnosed disease. They might be considered developmentally behind and have behavioral issues given their inability to express themselves and be understood. It is not uncommon to miss their abnormal soft palate anatomy, which can be subtly V-shaped and have the midline bluish line (pellucid zone).
Patients with arterial venous malformation of this area do not necessarily produce hypernasal speech. Chronic tonsillitis can be associated with hyponasality, enlarged tonsils, and bad breath. Palatal neoplasm is usually slow-growing and not associated with a pellucid zone or long-term speech issues. Torus palatini does not produce hypernasal speech.
2024
In a patient with a cleft lip and palate, the congenital absence of which of the following permanent maxillary teeth is most common?
A) Canine
B) Central incisor
C) First premolar
D) Lateral incisor
E) Second premolar
The correct response is Option D.
Dental anomalies are frequent in patients with cleft lip and palate. The maxillary lateral incisor is the most frequently congenitally absent secondary tooth, missing in around 60% of patients.
The other teeth listed are missing at a much lower incidence.
2024
A 15-year-old boy with repaired left unilateral cleft lip and palate undergoes alveolar bone grafting. The patient did not undergo preoperative orthodontic preparation. He was unable to tolerate oral intake for 15 hours after surgery, and no postoperative antibiotics were administered. Which of the following is the strongest predictor of graft failure in this patient?
A) Age greater than 12 years
B) Lack of orthodontic preparation with maxillary arch expansion
C) No postoperative antibiotic regimen
D) Time to postoperative oral intake greater than 12 hour
The correct response is Option A.
Secondary bone grafting is widely accepted in cleft care; however, there is limited consensus on the technique, timing, and perioperative management of the cases. In general, the age of the patient at secondary bone grafting is typically at the time of mixed dentition and ranges from 8 to 12 years of age. Several studies have demonstrated that later age at time of bone grafting is associated with higher rates of graft failure, especially when the canine has already erupted.
Lack of orthodontic preparation and postoperative antibiotic regimen were not predictors of graft failure. Time to postoperative oral intake was also not predictive of graft failure.
2024
A 1-month-old female infant born with a cleft palate and micrognathia is brought for evaluation of consistent desaturations. The patient undergoes placement of mandibular distractors, and postoperative examination shows asymmetry of the lower lip when she cries. Which of the following is the most likely cause of this patient’s current symptoms?
A) Buccal nerve palsy
B) Hardware failure
C) Marginal mandibular nerve injury
D) Orbicularis oris muscle injury
E) Zygomatic nerve palsy
The correct response is Option C.
The marginal mandibular nerve is a branch of the facial nerve that innervates the depressor labii inferioris muscle, depressor anguli oris muscle, and mentalis muscle. A patient with injury to the marginal mandibular branch of the facial nerve presents with a deformity opening the mouth, smiling or grimacing. Injury to the marginal mandibular nerve causes paralysis of muscles of the lower lip of that side.
The other options presented would not produce weakness of the depressor labii inferioris muscle.
2024
A 10-month-old female infant is evaluated for cleft palate reconstruction. A traditional Furlow palatoplasty is planned with the flap labeled “A” in the preoperative photograph shown. Which of the following best describes the tissue of this flap and the direction it will transpose?
“A” is the left side of patient’s designed Z
Tissue type Transposition direction
A) Mucosa and muscle anteriorly
B) Mucosa and muscle posteriorly
C) Mucosa only anteriorly
D) Mucosa only posteriorly
The correct response is Option B.
In a traditional Furlow palatoplasty, the flap labeled “A” includes the levator veli palatini muscle as a musculomucosal flap, and it is transposed posteriorly in the final Z-plasty.
2024
A 2-year-old boy who was born with bilateral cleft lip and palate and recently adopted internationally is brought by his parents for evaluation. The cleft lip was repaired before adoption, but the palate was not repaired. A photograph is shown. The parents would like to discuss management options for the best functional and aesthetic outcome for their child and ease of social integration. Which of the following is the most appropriate next step in management?
A) Cleft rhinoplasty
B) Complete cleft lip revision
C) Repair of the cleft palate
D) Speech therapy
E) Video fluoroscopy
The correct response is Option C.
The timing of cleft palate repair affects speech outcome. This child is already older than optimal timing, so repair of the cleft palate to allow for generation of normal speech is the correct next step. There is no reason for speech therapy or video fluoroscopy since the palate is not repaired yet. Primary nasal repair is done at the time of original lip repair, and revisions depend on the degree of deformity, but the definitive rhinoplasty is deferred until skeletal maturity. A complete lip revision for this patient’s hypertrophic scar can improve the appearance of this child but should wait until after palate repair.
2024
A 7-year-old boy with 22q11.2 deletion syndrome (DiGeorge syndrome) and a previously repaired submucous cleft palate with persistent velopharyngeal insufficiency is scheduled to undergo posterior pharyngeal flap surgery for speech improvement. Awareness of which of the following anatomical differences in this patient will ensure the safest repair possible?
A) Abnormal course of glossopharyngeal nerve
B) Extra-anatomic tonsillar tissues
C) Inferiorly positioned maxillary artery
D) Medial positioning of internal carotid arteries
E) Superior pharyngeal constrictor hypertrophy
The correct response is Option D.
In patients with 22q11.2 deletion syndrome (DiGeorge syndrome) with velopharyngeal insufficiency that persists after palatoplasty, posterior pharyngeal flap surgery is an option, but it must be performed carefully because these patients tend to have medialized internal carotid arteries, and these can be injured during dissection of the posterior pharyngeal flap.
The other options are incorrect, since these are not features of the anatomy in patients who have 22q11.2 deletions.
2024
A 25-year-old man is brought to the emergency department 1 hour after a motorcycle collision. The patient reports poor alignment of his teeth. Physical examination shows maxillary mobility and an anterior open bite. Which of the following muscles is most likely responsible for these findings?
A) Buccinator
B) Masseter
C) Mentalis
D) Pterygoid
E) Zygomaticus major and minor
The correct response is Option D.
This patient has a Le Fort I fracture. Malocclusion with an anterior open bite deformity is caused by the vector of pull of the medial and lateral pterygoid muscles. These muscles pull the fractured maxilla inferiorly and posteriorly, leading to premature posterior occlusal contact during bite. The mentalis muscle elevates the base of the lower lip. The masseter muscles elevate the mandible and pull it anteriorly. The zygomaticus muscles are muscles of facial expression and elevate the upper lip. The buccinator muscles are also muscles of facial expression and assist with chewing.
2024
Which of the following bones does NOT form part of the orbit?
A) Maxilla
B) Nasal
C) Palatine
D) Sphenoid
E) Zygoma
The correct response is Option B.
There are seven bones that make up the orbit: 1) sphenoid, 2) maxilla, 3) palatine, 4) zygoma, 5) ethmoid, 6) lacrimal, and 7) frontal. The nasal bones are not included.
A 13-year-old girl is brought to the clinic because of a 3-day history of headache, fever, vomiting, and swelling of the forehead and scalp. The patient reports that she sustained a head injury during a sports game 3 weeks ago. Physical examination shows no additional abnormalities. Which of the following is the most likely diagnosis?
A) Benign skin neoplasm
B) Frontal bone osteomyelitis
C) Nasal bone fracture
D) Orbital floor fracture
The correct response is Option B.
Frontal bone osteomyelitis with subperiosteal abscess (Pott puffy tumor) is most commonly seen in adolescents with history of head trauma, sinus infection, dental infection, and after neurosurgical procedures.
Patients who sustained orbital floor fractures may present with headache, nausea and vomiting, extraocular muscle restriction, and enophthalmos, but no forehead and scalp swelling or fever. The symptoms associated with a benign neoplasm will likely be more gradual and insidious, without fever or headache. A patient 3 weeks out from sustaining a nasal bone fracture could have deviation or headache, but would likely not have fever or swelling.
2024