Congenital midline scalp and skull defect.

Aplasia cutis congenita of the scalp

“…However, familial cases have been observed, while in other cases the condition is inherited in an autosomal dominant or recessive fashion [6,8,13,23]. In Adams-Oliver syndrome scalp defects are associated with terminal transverse defects in the limbs [2,6,8,10,23].…”

“…The absence of skin can involve any part of the body surface, but the preferential site is the cranial vertex [6,8,12,17]. The majority of cases of ACC are sporadic, but some reports indicate a familial trait [13]. There are also definite instances of autosomal dominant inheritance, although recessive inheritance has also been implicated [6,8,12,17].…”

Familial Occurrence of Atretic Cephaloceles

“…8, 9, 12]. Several authors have reported encephaloceles recur ring in families [6,[8][9][10][11]. This is especially true in the case of cephaloceles associated with known syndromes or chromosomal abnormalities [1,2,4,17].…”

“…These lesions have also been described as being rudimen tary, occult, or abortive cephaloceles meaning sponta neous arrest in the development of a true meningoence phalocele. Although the embryopathogenesis of atretic cephaloceles is not well known at present, many authors include these malformations among the neural tube de fects that develop in the postneurulation period [5], Most cephaloceles usually present as sporadic occurrence [5], There are few references on familial occurrence of cepha loceles [6][7][8][9][10][11][12][13], and in the majority of instances cephalo celes have been noted to be part of a known syndrome [1,2,14],…”

Isolated Aplasia Cutis Congenita on Left Foot in Chinese Neonate

“…Frieden classified the different anomalies into 9 groups based on the number and the presence or absence of other anomalies [5] (Table 1). The lesions in those cases are quite variable, ranging from only local absence of skin to a complete absence of epidermis, subcutaneous tissue, bone, or in some cases the dura [6] [7]. The incidence of ACC is estimated as 1 per 10,000 live births [1].…”