Abstract

BackgroundBody stalk anomaly is a generally lethal malformation of the thorax and/or abdomen. It is often associated with limb defects. The intrathoracic and abdominal organs lie outside the abdominal cavity. These are contained within a sac composed of amnioperitoneal membrane attached directly to the placenta. The umbilical cord may be totally absent or extremely shortened. Severe kyphoscoliosis is often present. This case is reported to highlight this rare malformation and its clinical presentation.Case presentationWe present a case of a 27-year-old primigravida Oromo woman who had been amenorrheic for 9 months. She presented with an urge to bear down of 10 hours and passage of liquor of the same duration. The patient was referred from a district primary hospital with a diagnosis of preterm labor and hand prolapse. A lower uterine segment cesarean section was performed at Jimma University Medical Center for an indication of active first stage of labor with nonreassuring fetal heart rate pattern (fetal bradycardia) and hand prolapse to effect an anomalous fetus that had only a rudimentary right lower extremity and liver and intestine found outside the abdominal cavity contained within a sac composed of transparent membrane attached directly to the placenta. The umbilical cord was very short, measuring about 7 cm. The fetus had severe scoliosis. It also had a heartbeat upon extraction, which stopped after 5 minutes of delivery. The placenta and fetal body parts together weighed 2400 g.ConclusionTermination of pregnancy is usually offered because this abnormality is generally considered lethal. If the pregnancy is continued undetected as in our patient’s case, vaginal delivery is recommended, given the highly lethal nature of this anomaly. Good prenatal screening and counseling are recommended for early detection and management.

Highlights

  • Body stalk anomaly is a generally lethal malformation of the thorax and/or abdomen

  • The disease known as body stalk anomaly (BSA), limb body wall complex (LBWC), or body stalk complex is a very rare anomaly (1 in 14,000 to 42,000 pregnancies; 1 in 7500 fetuses from 10 to 14 weeks of gestation)

  • This medical condition is characterized by a complex anomaly of the anterior abdominal wall, severe kyphoscoliosis, rudimentary umbilical cord, and anatomical defects of the pelvis and lower limbs [1–3]

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Summary

Conclusion

Termination of pregnancy is usually offered because the abnormality is generally lethal. If the pregnancy is continued undetected, as in our patient’s case, vaginal delivery is recommended only if there is no contraindication for vaginal delivery. Because of the lethal nature of the anomaly, the patient should be extensively counseled on the prognosis of the fetus. The family must be reassured that there is no risk for recurrence. Good prenatal screening and counseling are recommended for early detection and management

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