Group

Associated anomalies

Inheritance

1

Scalp ACC without multiple

anomalies

Cleft lip and palate, tracheoesophageal fistula, patent ductus arteriosus, omphalocele,

mental retardation, polycystic kidneys

Autosomal dominant or sporadic

2

Scalp ACC with limb

abnormalities

Limbs reduced, syndactyly, clubfoot, encephalocele, nail dystrophy or absence, persistent cutis marmorata

Autosomal dominant

3

Scalp ACC with skin/organoid

nevi

Epidermal nevi, corneal opacities, sclera dermoids, eyelid colobomas, mental retardation, seizures

Sporadic

4

ACC overlying embryologic

malformations

Meningomyelocele, spinal dysraphia, cranial stenosis, leptomeningeal angiomatosis,

gastroschisis, congenital midline porencephaly, ectopia of ear, omphalocele

Depends upon

underlying condition

5

ACC with fetus papyraceus or

placental infarcts

Single umbilical artery spastic developmental delay, spastic paralysis, clubbed hands and feet, amniotic bands

Sporadic

6

ACC associated with

epidermolysis bullosa

Blistering of skin and/or mucous membranes, deformed nails, pyloric or duodenal atresia, abnormal ears and nose, ureteral stenosis, renal anomalies, amniotic bands

Depends upon type of

epidermolysis bullosa

7

ACC localized to extremities without blistering

None

Autosomal dominant

or recessive

8

ACC caused by teratogens

Imperforate anus (methimazole), other signs of intrauterine infection with varicella or herpes simplex

Not inherited

9

ACC associated with

congenital syndromes

Trisomy 13, 4p-syndrome, ectodermal dysplasia, focal dermal hypoplasia, amniotic band disruption complex, XY gonadal dysgenesis, Johanson-Blizzard syndrome

Depends upon

Syndrome