Limb-body wall complex : a compound anomaly pattern in body-wall defects

Our presentation of four cases demonstrates the essential features of limb-body wall complex (LBWC), representing a compound anomaly pattern in body-wall defects. The diagnosis of this entity is based on two of the three following characteristics: (1) exencephaly/encephalocele and facial clefts; (2)...

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Bibliographic Details
Published in:Pediatric surgery international 2001-07, Vol.17 (5-6), p.486-490
Main Authors: PUMBERGER, W, SCHALLER, A, BERNASCHEK, G
Format: Article
Language:English
Subjects:
Abdominal Muscles - abnormalities
Abdominal Muscles - diagnostic imaging
Abdominal Muscles - embryology
Abnormalities, Multiple - embryology
Biological and medical sciences
Diseases of mother, fetus and pregnancy
Female
Gynecology. Andrology. Obstetrics
Humans
Infant, Newborn
Limb Deformities, Congenital - diagnostic imaging
Limb Deformities, Congenital - embryology
Medical sciences
Pregnancy
Pregnancy. Fetus. Placenta
Ultrasonography, Prenatal
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Summary:Our presentation of four cases demonstrates the essential features of limb-body wall complex (LBWC), representing a compound anomaly pattern in body-wall defects. The diagnosis of this entity is based on two of the three following characteristics: (1) exencephaly/encephalocele and facial clefts; (2) thoraco- and/or abdominoschisis; and (3) limb defects. A definite association with internal anomalies and severe kyphoscoliosis makes a more distinct concept of the pathogenesis reasonable. Limb-body wall malformations result from a malfunction of the ectodermal placodes involving the early embryonic folding process. The poor prognosis of LBWC calls for early antenatal diagnosis.
ISSN:0179-0358
1437-9813
DOI:10.1007/s003830000497