Limb Body Wall Complex: A Rare Anomaly
A 42-year-old, G3P2 L0 presented to the obstetrics outpatient department in the second trimester of her third pregnancy for a routine antenatal check up. She had two still births in the past, the details of which are not known. She is not a hypertensive or diabetic. There was no history of any drug intake except for iron and folic acid supplementation. Her routine hematological and biochemical investigations were within normal limits. A routine anomalous scan done at 2nd trimester showed a fetus with features of intrauterine growth retardation along with a large fluid-filled sac in the abdominal region which was compressing the thorax. Both upper and lower extremities were visualized. However, due to oligohydraminos no further comment could be made upon the fetal anatomy. Color flow showed two umbilical arteries along the wall of the cyst. The intraabdominal unilocular cystic mass was assumed to be the urinary bladder and in conjunction with the presence of oligohydraminos and Intrauterine growth retardation a proposed diagnosis of congenital megacystitis or Prune Belly Syndrome was made [Figures and ]. The parents were informed about the diagnosis and after counseling, they opted for termination of pregnancy. The pregnancy was terminated after obtaining the consent, and the abortus was sent for pathological examination.\nAt autopsy, the fetus weighed 75 g and on external examination a number of anomalies were noted. The genitalia were ambiguous. Encephalocoele was seen over the head of the fetus in the occipital region. The most striking abnormality was a left sided anterior abdominal wall defect, from which gastrointestinal organs were protruding. The organs identified included the liver and the intestine. Although these organs were not encased by a membrane, a membrane like structure was present near the anterior abdominal wall opening [Figures and ]. A defect in the posterior abdominal wall was also noted. A number of skeletal abnormalities were noticed. There were scoliosis and amelia of the digits of the right upper limb. The lower limbs exhibited malrotation and were pushed to the right lateral side by the protruding abdominal organs []. The umbilical cord was short and was found in a membrane like structure that was partially covering some of the protruding organs. A radiological examination was also done, which confirmed the gross skeletal anomalies and the encephalocele []. On internal examination, there was agenesis of anal canal along with agenesis of genitourinary tract, and the lungs were hypoplastic. The remaining organs appeared normal on gross examination and congested on microscopy. In view of the above combination of malformations, a diagnosis of LBWC was offered.