developmental abnormalities

Question 1

aplasia cutis congenita/hair collar sign

Answer 1

loss of epidermis/dermis and possibly subq
heals with scarring
+hair collar sign is a ring of hair around aplasia site—> indicates cranial dysraphism
if small, no treatment. if large, then surgery. if midline, may have underlying CNS defect

FYI: if midline, there was incomplete closure of neural tube. if lateral, there was incomplete closure of embryonic fusion lines

a/w adams-oliver syndrome, bart syndrome, methimazole use

Question 2

nasal glioma

Answer 2

• firm, non compressible, contender lesion on dorsal nose.
• may be extra nasal or intranasal
• may give appearance of hypertelorism

Tx surgery

Question 3

meningocele/encephalocele

Answer 3

herniation of cranial contents
meningocele - meningeal tissue
encephalocele - meningeal + glial tissue
*** transilluminates on p/e
Tx radiology 1st for surgical planning then surgery

May enlarge with increased intracranial pressure

Question 4

accessory tragus

Answer 4

-faulty development of 1st branchial arch
-extra ear anywhere from preauricular to angle of mouth
-Tx surgery
a/w Goldenhar syndrome

Question 5

congenital rests of the neck

Answer 5

• remnants of branchial arches, occur along fusion lines
• cartilaginous nodules along the cervical neck
• Tx surgery

Question 6

midline cervical cleft

Answer 6

defect of ventral neck
linear atrophic patch with small skin tag and ectopic salivary tissue
may be a/w cleft lip, cleft palate, chin, tongue, mandible
Tx surgery

Question 7

lip pits

Answer 7

little depressions in the lip often lower lip
a/w cleft lip, cleft palate
may also be a/w abnormal salivation
Tx surgery

Question 8

developmental anomalies of the umbilicus

Answer 8

urachal remnant or omphalomesenteric duct fails to regress
+ DRAINAGE
red to pink nodule within umbilicus w/mass underneath
Tx: surgery

Question 9

amniotic band syndrome

Answer 9

premature rupture of amniotic sac and formation of fibrous strands
constriction band of lower extremities, leading to lymphedema, ischemia +/- amputation
Tx surgical correction

Question 10

dermoid cyst

Answer 10

faulty development along embryonic fusion lines
firm, contender, blue nodules on the face
MRI is warranted for lesions in the midline
Tx surgery