Prenatal ultrasonographical features of limb body wall complex: a review of etiopathogenesis and a new classification.
Limb body wall complex is a spectrum of multiple severe anomalies. The etiopathogenesis and clinical classification are still under discussion. In our article, while reviewing previous etiopathogenetical hypothesis, we propose a new clinical classification regarding embryological theories and pheneotypical features. According to the Van Allen diagnostic criteria, the findings of 6 affected fetuses are presented. Prenatal diagnosis was performed in 5 of 6 cases. Craniofacial malformations were present in only 1 case. Thoracic defect and abdominoschisis (either infraumbilical or supraumbilical) associated with visceral eventration, placental-umbilical cord anomalies, and limb defects were detected in the other 5 cases. Aberrant development of each of the 4 embryonic folds (cephalic, 2 lateral abdominal, and caudal) associated with faulty umbilical ring development and placental formation were considered responsible for development of various malformations. In previous clinical classifications, existence or absence of the craniofacial malformation was utilized as an unique discriminating criterion while multiple anomalies exist. In this report, we propose a new clinical classification concerning almost all anomalies caused by defective placental attachment and maldevelopment of the 4 folds.
Sahinoglu
Zeki
Z
Division of Perinatology, Department of Obstetrics and Gynecology, Zeynep Kamil Women and Children Diseases Education and Research Hospital, Uskudar, Istanbul, Turkey. zekisa@superonline.com
Uludogan
Mehmet
M
Arik
Huseyin
H
Aydin
Arzu
A
Kucukbas
Mehmet
M
Bilgic
Remziye
R
Toksoy
Guven
G
D000015:Abnormalities, Multiple / Q000145:classification / Q000175:diagnosis / Q000000981:diagnostic imaging; D005260:Female; D005333:Fetus / Q000033:anatomy & histology / Q000473:pathology; D005865:Gestational Age; D006801:Humans; D017880:Limb Deformities, Congenital / Q000145:classification / Q000175:diagnosis / Q000000981:diagnostic imaging; D010641:Phenotype; D011247:Pregnancy; D016216:Ultrasonography, Prenatal