PMC4673583_01
(A) Posterior view of Patient 1 showing fetus with sacrolumbar myelomeningocele, and contracted lower extremities. (B) Lateral fetal view depicts the attached amnion covering omphalocele with a short umbilical cord wrapped around the amnion covering the abdominal wall defect. The fetus also has bilateral flexion contracture deformity of lower extremities. (C) Post-mortem antero-posterior x-ray showing segmentation anomalies of the thoracic vertebrae (thick arrow), omphalocele (thin arrow) and meconium peritonitis (small thin arrows). (D) Post-mortem lateral x-ray showing lumbosacral myelomeningocele (arrow).
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F0001
External examination revealed a nonmacerated fetus whose sex could not be determined initially due to absence of external genitalia, weighing 245 grams, and with a crown-heel length of 19.0 cm and foot length of 2.2 cm (Figure 1). Postmortem X-rays of the body - antero-posterior (AP) (Figure 1C) and lateral views (1D) demonstrate a small, completely opacified chest. The omphalocele demonstrated were multiple underlying calcifications, which were likely due to prior bowel perforation and subsequent meconium peritonitis (Figure 1C, small thin arrows). Multiple segmentation anomalies of the vertebrae are noted in the thoracic spine (Figure 1C, larger arrow). There was lumbosacral spinal dysraphism with soft tissue noted to project dorsally, consistent with a lumbosacral myelomeningocele (Figure 1D, arrow).
(A) Gross picture of Patient 1 showing fetus with attached cord and placenta. (B) Closer view of short umbilical cord covered with amnion. Notice a large interface of abdominal skin with omphalocele sac.
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F0002
The fetus had a large defect in the anterior abdomen covered by the amnion, measuring 6.0 x 6.0 x 4.5 cm containing part of the liver and gastrointestinal tract and a very short umbilical cord partially wrapped with the amnion and the placenta still attached to the fetus ( Figure 2A,B). The fetus was delivered with the placenta still attached to a short umbilical cord wrapped around the amniotic sac attached to abdominal skin (Figure 2A&B).
PMC4673583_02
Gross photograph of Patient 2 showing fetus with a right antero-lateral body wall thoracoabdominoschisis covered by amnion. The right upper extremity is missing, the umbilical cord is short and the lower extremities are slightly contracted.
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F0003
Due to increasing vaginal bleeding and maternal abdominal pain, labor was induced at 18 weeks of gestation and resulted in a nonmacerated fetus of 191 grams (Figure 3), crown-heel length of 16.8 cm, cephalic circumference of 14.6 cm and foot length of 2.6 cm.
PMC4673583_03
(A-B). Gross picture of macerated fetus after formalin fixation showing club-feet deformity and an abdominal wall defect. The lower extremities are contracted. (C-D) Post-mortem antero-posterior and lateral x-rays showing severe levoscoliosis, widely separated ischial ossification centers (larger arrows), and external protrusion of abdominal contents (smaller arrow), and club foot deformity.
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F0004
Labor was induced at 18 weeks gestational age and resulted in a severely macerated female fetus weighing 200 grams (Figure 4 ), crown-heel length of 21.5 cm, and foot length of 2.0 cm. Post mortem X-rays of the body:AP (Figure 4C) and lateral (Figure 4D) views demonstrated marked levoscoliosis of the thoracolumbar spine without any congenital vertebral body anomalies. There was an abdominal body wall defect with protrusion of bowel contents externally (Figure 4C, smaller arrows). The ischial ossification centers were widely separated (Figure 4C, larger arrows).
PMC4673583_04
(A) Gross photograph of fetus with no external genitalia and omphalocele sac involving abdomen and pelvis. Notice flexion deformity of both lower extremities. (B) Post-mortem antero-posterior x-ray showing a large lucency occupuing the chest and abdomen consistent with post-mortem air, there are vertebral body segmentation anomalies and hypoplastic sacrum (arrow). (C) Photograph depicts a closer view of extracorporeal organs. (D) Gross picture of formalin fixed organ block of Patient 4. The lower portion demonstrates the omphalocele sac still attached to abdomino-pelvic organs.
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F0005
Labor was induced at 19 weeks gestational age and resulted in a nonmacerated fetus weighing 238 g (Figure 5), crown-heel length of 20.9 cm, cephalic circumference of 15 cm, and foot length of 3.0 cm. AP X-ray of the body (Figure 5B) demonstrated a large lucency occupying the entire chest and abdominal cavities consistent with post mortem air. Abdominal contents were not seen which was consistent with the clinical history.