TITLE:
Prenatal diagnosis of a particular limb body wall complex
AUTHORS:
B. Fakhir, I. Jadid, A. Aboulfalah, H. Asmouki, A. Soummani
KEYWORDS:
Fetal Malformation; Laparoscisis; Feto-Placental Attachment; Kyphoscolisis
JOURNAL NAME:
Open Journal of Obstetrics and Gynecology,
Vol.4 No.3,
February
21,
2014
ABSTRACT: Limb Body Wall Complex (LBWC) is a rare heterogenic
group of fetal malformations including major defects of the ventral body
(thoracoabdominal) wall associated with other anomalies including those of the
limbs that may range from amelia to mild positional deformations, unusual craniofacial malformations, and a variety
of visceral abnormalities that include the heart, lungs, genitourinary system.
Tow phenotypes are described according to the feto-placental attachment. We
report a particular case and iconography of LBWC associated with a giant sacral
meningocele never described before. The patient was 26 years old, primigravida,
with no illnesses or recent infections. She had no history of prenatal exposure
to teratogenic agents, nor any family history of congenital malformations. She
was referred for polymalformatif syndrome at 30 gestation weeks. Advanced
sonography showed giant omphalocel with eviscerated liver, stomac and kidney,
club feet, kyphoscoliosis and giant sacral fluid cyst. A 2100 g fetus was
delivered, post natal examination confirmed LBWC with abdominoplacental
attachment and the particularity of the presence of giant sacral meningocele,
two bilateral hemi-genital tubercles and anal atresia.