Aplasia cutis congenita is a heterogeneous group of conditions usually involving the scalp as well as any other part of the body and is associated with a number of other congenital anomalies. We report on a newborn male with almost complete absence of skin and subcutaneous tissue in association with choanal atresia, syndactyly, imperforate anus, pulmonary hypoplasia, and other anomalies. To our...

A 1-day-old boy with the characteristics of Adams-Oliver syndrome was presented. Adams-Oliver syndrome has a wide spectrum of anomalies ranging from aplasia cutis congenita, cutis marmorata telangiectatica congenita and transverse limb defects to lethal anomalies. Our patient had aplasia cutis congenita with scalp, skull and dura defect. He had also a large dura defect with herniation of brain ...

Aplasia cutis congenita is the congenital absence of skin. The majority of these defects involve the vertex of the scalp in the area overlying the sagittal sinus. The larger defects are predisposed to sudden lethal hemorrhage and require urgent closure. Local rotational scalp flaps are recommended for closure of the larger defects because they provide the most reliable coverage, eliminate the r...

The patient was a female neonate bornprematurely at 36 weeks of gestation by a Cesareansection. During pregnancy, the mother had nohistory of fever, drug use, or X-ray exposure.The mother was gravida 2 and had no history ofabortion. Her first child was healthy. There wasno record of birth trauma. The perinatal historywas negative for intrauterine trauma, the use ofantithyroid medication, or mis...

Aplasia cutis congenita (ACC) is a rare disorder, which is defined by the localized, or less commonly widespread absence of skin involving the epidermis, dermis and subcutaneous tissue with an incidence of 1/10.000 newborns. The underlying bone and dura mater can be also affected, and muscle and bone involvement occur in approximately 20 to 30% of the cases. Aplasia cutis congenita most often o...

Background: Aplasia cutis congenita (ACC) is a rare condition regarded as congenital absence of the epidermis, dermis, and in some cases, subcutaneous tissues newborn. The pathogenic mechanism unclear, although has been described result disrupted development or degeneration skin utero. ACC may be observed with fetus papyraceous (FP). Case Presentation: We report case an 8-hour-old newborn femal...

how to cite this article: saeed m, haq a, qadir kh.bart’s syndrome associated corpus callosum agenesis and choanal atresia. iran j child neurol. 2014 autumn;8(4): 76-79. abstract objective bart’s syndrome is defined as congenital localized absence of skin, and associated with epidermolysis bullosa. a newborn with bart’s syndrome is reported because it is a very rare condition, especially when a...

Epidermal nevus syndrome (ENS) is a rare condition characterized by congenital epidermal nevi (EN) associated abnormalities of other organs including central nervous system (CNS), skeletal system, eyes, and oral cavity (1). ENS is divided into a group of distinct disorders, based on the associated epidermal nevus and genetic inheritance pattern. Organoid nevus could be seen in some ENS, includi...