Pediatric General Surgery 4 (Exam 1) Flashcards by Kaitlyn McDow

Which of the following sutures lies between the two parietal bones?

A. Coronal suture
B. Sagittal suture
C. Lambdoid suture
D. Squamous suture

B. Sagittal suture

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The anterior fontanelle is located at the junction of which two sutures?

A. Sagittal and lambdoid
B. Coronal and squamous
C. Coronal and sagittal
D. Frontal and metopic

C. Coronal and sagittal

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The _______________ fontanelle is located between the parietal and occipital bones.

A. Anterior
B. Mastoid
C. Sphenoidal
D. Posterior

D. Posterior

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Which of the following are correctly matched sutures or fontanelles visible in the image?
Select 4:

A. Coronal suture – between frontal and parietal bones

B. Lambdoid suture – between parietal and occipital bones

C. Sagittal suture – between temporal and parietal bones

D. Sphenoidal fontanelle – near the greater wing of the sphenoid

E. Mastoid fontanelle – located near the squamous and lambdoid sutures

F. Anterior fontanelle – junction of sagittal and lambdoid sutures

A. Coronal suture – between frontal and parietal bones
B. Lambdoid suture – between parietal and occipital bones
D. Sphenoidal fontanelle – near the greater wing of the sphenoid
E. Mastoid fontanelle – located near the squamous and lambdoid sutures

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During gestation, the _______________ suture connects the developing bones at the back of the fetal skull but usually fuses by early infancy.

A. Coronal
B. Lambdoid
C. Mendosal
D. Squamous

C. Mendosal

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Craniosynostosis refers to:

A. Delayed ossification of cranial bones
B. Infection of the cranial sutures
C. Premature fusion of one or more cranial sutures
D. Absence of fontanelles at birth

C. Premature fusion of one or more cranial sutures

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Which suture is involved in unilateral coronal synostosis, resulting in forehead flattening on one side?

A. Lambdoid
B. Coronal
C. Sagittal
D. Metopic

B. Coronal

Unilateral Lamboid synostosis - lamboid suture

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Which cranial suture closes prematurely in sagittal synostosis?
A. Lambdoid
B. Coronal
C. Sagittal
D. Metopic

C. Sagittal

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What is the hallmark cranial shape associated with metopic synostosis?

A. Trigonocephaly
B. Scaphocephaly
C. Brachycephaly
D. Plagiocephaly

A. Trigonocephaly

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The cranial deformity sagittal synostosis shown in images C & D represents:

A. Trigonocephaly
B. Brachycephaly
C. Plagiocephaly
D. Scaphocephaly

D. Scaphocephaly (Sagittal synstosis)

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The triangular-shaped forehead seen in images E & F is most consistent with:

A. Crouzon syndrome
B. Sagittal synostosis
C. Metopic synostosis
D. Pfeiffer syndrome

C. Metopic synostosis (Trigonocephaly)

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Syndactyly of both hands and feet (as in image K) is a hallmark of which syndrome?

A. Apert syndrome
B. Crouzon syndrome
C. Carpenter syndrome
D. Saethre-Chotzen syndrome

A. Apert syndrome

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Craniosynostosis occurs in approximately:

A. 1 in 1000 live births
B. 1 in 3000 live births
C. 1 in 5000 live births
D. 1 in 10,000 live births

B. 1 in 3000 live births

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True or False

Males are not often affected by craniosynostosis

False

Males are often affected

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Approximately _______________ of craniosynostosis cases involve multiple sutures and are associated with one of over 400 syndromes.

A. 20%
B. 35%
C. 50%
D. 75%

A. 20%

80% of craniosynostosis are isolated

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Apert syndrome is most commonly associated with which type of cranial deformity?

A. Trigonocephaly
B. Cloverleaf skull
C. Scaphocephaly
D. Occipital bossing

B. Cloverleaf skull

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Which of the following genetic abnormalities is associated with Apert syndrome?

A. FGFR10 mutation on chromosome 2
B. FGFR3 mutation on chromosome 4
C. FGFR2 mutation on chromosome 10
D. PAX6 mutation on chromosome 11

C. FGFR2 mutation on chromosome 10

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What is the approximate incidence of Apert syndrome?

A. 1 in 100 live births
B. 1 in 1,000 live births
C. 1 in 10,000 live births
D. 1 in 100,000 live births

D. 1 in 100,000 live births

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Which of the following features are consistent with Apert syndrome (acrocephalosyndactyly)?
Select 4:

A. Midface hyperplasia
B. Syndactyly of hands and feet
C. FGFR3 mutation
D. Midface hypoplasia
E. Macroglossia
F. Proptosis
G. Increased risk of OSA

B. Syndactyly of hands and feet
D. Midface hypoplasia - increased ICP
F. Proptosis - protrusion of eyeballs
G. Increased risk of OSA

Hyperterlorism - unusual distance between two body parts

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Apert Syndrome primarily affects derivatives of which embryologic structure?

A. Second branchial arch
B. Third pharyngeal pouch
C. First branchial arch
D. Fourth neural crest

C. First branchial arch

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Which of the following structures are derived from the first branchial arch, and are therefore affected in Apert Syndrome?

A. Mandible and maxilla
B. Larynx and pharynx
C. Thyroid and parathyroid glands
D. Trapezius and sternocleidomastoid muscles

A. Mandible and maxilla

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Which suture is most commonly involved in Apert Syndrome leading to the cloverleaf skull?

A. Sagittal suture
B. Coronal sutures
C. Lambdoid suture
D. Metopic suture

B. Coronal sutures (bilateral)

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Which of the following cranial features are commonly associated with Apert Syndrome?
Select 4:

A. Tall skull shape
B. Narrow skull AP diameter
C. Oakleaf skull shape
D. Large ears
E. Skull bitemporal widening
F. Flat forehead

Study These Flashcards

A. Tall skull shape
B. Narrow skull AP diameter (front to back)
E. Skull bitemporal widening (side to side)
F. Flat forehead

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True or False

Syndactyly can range from partial webbing to complete fusion of digits

Study These Flashcards

True

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Crouzon syndrome is classified as a type of: A. Neural crest malformation B. Somite-derived disorder C. Branchial arch syndrome D. Neuroendocrine dysplasia

C. Branchial arch syndrome "Similar to Apert with differing ophthalmic defects" ## Footnote 131

A hallmark feature of Crouzon syndrome is _______________, which puts the eyes at risk for injury. A. Ptosis B. Anisocoria C. Proptosis D. Mydriasis

C. Proptosis ## Footnote 131

What percentage of patients with Crouzon syndrome are affected by optic atrophy? A. 20% B. 25% C. 30% D. 35%

A. 20% ## Footnote 131

Unlike Apert syndrome, patients with Crouzon syndrome **do not** exhibit _______________ or ___________abnormalities. Select 2 A. Eye B. Foot C. Cranial D. Hand

B. Foot D. Hand ## Footnote 131

The gene mutation for Crouzon syndrome is **inherited** in 50% of cases and involves the _______________ gene on chromosome 10. A. FGFR1 B. FGF8 C. FGFR2 D. COL1A1

C. FGFR2 (Same as Apert) The other 50% is sporadic mutations ## Footnote 131

Which of the following are valid indications for craniofacial reconstructive surgery? Select 2 A. Seasonal allergies B. Obstructive sleep apnea C. Unilateral inguinal hernia D. Increased ICP E. Central sleep apnea

B. Obstructive sleep apnea (OSA) D. Increased ICP ## Footnote 136

Which of the following are valid indications for craniofacial reconstructive surgery? Select 3 A. Severe exophthalmos B. Decreased ICP C. Psychosocial concerns D. Craniofacial deformity E. Severe panocularis

A. Severe exophthalmos C. Psychosocial concerns D. Craniofacial deformity ## Footnote 136

Which of the following are key intraoperative strategies for craniofacial reconstruction? Select 3 A. Type & cross B. Ketamine C. Foley catheter placement D. Use of petroleum-based lubricants E. Arterial line placement

A. Type & cross for blood products C. Foley catheter placement E. Arterial line placement ## Footnote 137

Which interventions are standard for blood management and monitoring in long craniofacial surgeries? Select 3 A. Arterial line B. Two large-bore IVs C. Avoid blood products D. Prevent hypothermia E. Foley catheter optional F. Frequent ABG monitoring

B. Two large-bore IVs D. Prevent hypothermia F. Frequent ABG monitoring ## Footnote 137

# True or False In patients with elevated intracranial pressure, hyperventilation may be used intraoperatively to blunt sympathetic response.

True ## Footnote 137

# True or False Post op pain is often extreme withcraniosynostosis and craniofacial repairs and is managed with only opioids.

False Post op pain. Pretty surprisingly is relatively mild and these craniosynostosis and craniofacial repairs it's usually managed with NSAIDs and Tylenol. ## Footnote 137

What is the typical positioning for a craniosynostosis repair in an infant? A. Lithotomy position with yellowfins B. Supine with head elevated C. Swan dive position using a bean bag D. Lateral decubitus with flexed neck

C. Swan dive position using a bean bag ## Footnote 138

What is one reason for placing duoderm patches on pressure points during craniosynostosis surgery? A. Prevent IV infiltration B. Promote blood clotting C. Minimize skin breakdown D. Prevent ET tube dislodgement

C. Minimize skin breakdown on bony prominences ## Footnote 138

In the swan dive position, what should be verified after final positioning? Select 2 A. Total blood loss B. Time out in the chart C. Bilateral breath sounds D. Eye opening E. IV patency

C. Bilateral breath sounds E. IV patency (especially the Arterial line, have a NIBP on just to double check accuracy) ## Footnote 138

Which facial anomaly is the second most common after cleft lip and palate? A. Craniosynostosis B. Hemifacial microsomia C. Crouzon syndrome D. Treacher Collins syndrome

B. Hemifacial microsomia ## Footnote 140

Hemifacial Microsomia is the result of malformation of which embryologic structures? A. 1st and 2nd branchial arches B. Neural crest cells C. Nasal placodes D. Forebrain and midbrain junction

A. 1st and 2nd branchial arches ## Footnote 140

Which of the following structures is derived from the first branchial arch? A. Hyoid bone B. Stapes C. Styloid process D. Mandible

D. Mandible ## Footnote 140

The second branchial arch gives rise to which of the following structures? A. Masseter B. Muscles of mastication C. Hyoid bone D. Tongue muscles

C. Hyoid bone and arch ## Footnote 140

In patients with hemifacial microsomia, approximately __________ of cases are easy intubations, but __________ are very difficult. A. 100%; 11% B. 70%; 9% C. 50%; 8% D. 30%; 6%

B. 70%; 9% ## Footnote 140

What does the “O” in the OMENS classification stand for in evaluating hemifacial microsomia? A. Oral cavity malformation B. Otic cartilage abnormality C. Orbital distortion D. Occipital deformity

C. Orbital distortion ## Footnote 140

The “M” in the OMENS classification of hemifacial microsomia stands for __________. A. Muscle atrophy B. Mental nerve impingement C. Maxillary rotation D. Mandibular hypoplasia

D. Mandibular hypoplasia ## Footnote 140

The "E" in OMENS classification stands for __________. A. Eye protrusion B. External auditory canal atresia C. Ear anomalies D. Endocrine dysfunction

C. Ear anomalies ## Footnote 140

# True or False The “N” in OMENS classification refers to neural involvement in hemifacial microsomia.

True ## Footnote 140

What does the "S" in the OMENS classification system for hemifacial microsomia represent? A. Soft tissue deficiency B. Suture closure C. Skeletal fusion D. Sensory impairment

A. Soft tissue deficiency ## Footnote 140

Which of the following features may be found in a patient with hemifacial microsomia? (Select 3) A. Mandibular hypoplasia B. Cleft palate C. Frontal bone bossing D. E. Facial nerve defects

A. Mandibular hypoplasia B. Cleft palate E. Facial nerve defects ## Footnote 141

Risk factors for difficult intubation in hemifacial microsomia include: (Select all that apply) A. Midfacial hypoplasia B. Retrognathia C. Tracheomalacia D. Asymmetric mouth opening E. Macrostomia → Correct Answers: A, B, D

Pediatric General Surgery 4 (Exam 1) Flashcards

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