LIMB BODY WALL COMPLEX IN TWO HETEROZYGOTIC TWINS: A CASE REPORT

Abstract

Introdution. Limb-Body Wall Complex (LBWC) is a congenital defect which includes at least two of the following characteristics: abdominal and/or thoracic body wall defects, exencephaly or encephalocoele with or without craniofacial defects (56%) and spinal defects associated with marked vertebral or sacral defects (95%). Case Report. We present a case report of an infant with LBWC, borned by diamniotic twin pregnancy. A prenatal ultrasound reported an healthy fetus and a fetus with multiple malformations. At birth we found a big abdominal wall defect, absence of scrotal sac and testicles, asymmetric chest and no major deformities in craniofacial region. At 2 hours of life, we removed the amniotic sac, we put the stomach and the spleen in the abdominal cavity, which is virtual, and positioned a spring-loaded silo. At 15th day of life we had complete reduction of the intestinal loops and liver and we closed the wall defect with a prosthesis and a cryopreserved skin. The general conditions of the patient, very severe since birth, became progressively worse and he died in the 21st day of life. Discussion. Once the diagnosis is done the physician should offer the parents a therapeutic abortion, and above all, in case they want to carry the pregnancy to term, we need to prepare them to the severity of the malformation and the high probability of death.