Body stalk anomaly

Sivasamy Manohar, MD, DMRD

India.

Case report

This is a case of a dichorionic diamniotic twin pregnancy. Patient was referred for an ultrasound scan at 23 weeks of gestation. One of the twins was normal, with no detected abnormalities. The second twin was in a fixed position, inseparable from the placenta. The ultrasound examination detected the following findings:

  • Eviscerated organs, liver and heart were attached to the placenta
  • Lateral body wall defect
  • Severe scoliosis of the spine
  • Extremely short umbilical cord with no coiling

The final diagnosis based on the ultrasound findings was Body stalk anomaly.

Images 1,2: Image 1 shows the interamniotic membrane. Image 2 shows the part of the fetus adhering to the placenta.

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Images 3,4: Image 3 shows a visceral organs adhering to the placenta. Image 4 shows distorted feet.

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Images 5,6: Image 5 shows severely distorted fetus adhering to the placenta. Image 6 shows a severe scoliosis.

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Images 7,8: Image 7 shows a transverse view of the thorax, note eviscerated heart. Image 8 shows anterior abdominal wall adhering to the placenta.

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Images 9 - 11: Images show a short umbilical cord without any coiling, umbilical vessels run directly from the placenta to the attached fetus.

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Video 1,2: Videos show herniated visceral organs, liver attached to the placenta, grossly distorted fetus.



Images 12 -14: Images of the fetus, note severely distorted torso and extremitites, anterior abdominal wall defect with herniated visceral organs adhering to the placenta.

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Body stalk anomaly

Synonyms:

Body stalk complex, Limb body wall complex, Cyllosomas.

Differential diagnosis

  • Amniotic band syndrome
  • Gastroschisis
  • Omphalocele
  • OEIS complex
  • Pentalogy of Cantrell
  • Cloacal exstrophy



  Amniotic band syndrome Body stalk anomaly
Incidence Rare, more common among spontaneous abortions
1: 7, 000 - 42, 000, more common in monozygotic twins
Genetic defect Unknown
Unknown
Pathogenesis Rupture of amnion, entrapment of fetal structures by sticky mesodermic bands that originate from the chorionic side of amnion 1) Amnion rupture
2) Vascular disruption
3) Embryonic malformation
4) Primary malformation of the body wall closure with it's fusion with the amnion during the 1st month of development
Clinical features Early entrapment (18-24 days post conception): severe craniofacial and internal malformation

Late entrapment: simple amputation or limb defects

Severe cases difficult to distinguish from body stalk anomalies
2 distinct phenotypes:

Craniofacial defect, amniotic bands (likely due to early vascular disruption)

Abdominal wall defects, urogenital anomalies, anal atresia, spinal and limb defects, abdomino-placental attachment, short or absent umbilical cord (due to embryologic maldevelopment).


Prognosis: lethal, often spontaneous abortion

References:

1. Callen P.W.: Ultrasound Evaluation of the placenta and umbilical cord. In: Ultrasonography in Obstetrics and Gynecology. Philadelphia, USA: Sounders, Elsevier; 2008:751.
2. Woodward P.J., Kennedy A., Sohaey R., Byrne J.: Abdominal wall. In: Diagnostic Imaging - obstetrics. Salt Lake City, USA: Amirsys; 2008: 7-14i.

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