Amniotic band sequence: prenatal diagnosis, phenotype descriptions, and a proposal of a new classification based on morphologic findings.

OBJECTIVE: The aim of this study is to describe the phenotype of fetuses affected by amniotic band sequence (ABS) that were diagnosed at the Instituto Nacional de Perinatología Isidro Espinosa de los Reyes and to propose a new classification based on morphologic findings. MATERIAL AND METHODS: Cases with a final diagnosis of amniotic band sequence, diagnosed between January 1993 and July 2010 in the Department of Maternal Fetal Medicine, were reviewed. Demographic, clinical, and periconceptional data were collected, and the defects were described and classified. The association frequencies of the defects were also determined. RESULTS: We included 50 cases with prenatal diagnosis of amniotic band sequence. The mean maternal age was 25.7 ± 6.9 years. Of these patients, 54% (27/50) were primiparous compared to 22% (11/50) who had three or more previous pregnancies. Craniofacial defects were seen in 78% (39/50) of the cases, followed by defects of the extremities 70% (35/50), abdominal wall, spine, and/or thorax 52% (26/50). The most frequent defects were the following: a) Encephalocele and facial clefts in the craniofacial group. b) Shortening at any level in the limb defects group, and c) Alterations of the spinal column curvature in the group of "other" defects. CONCLUSIONS: The amniotic band sequence shows a tendency to affect women who are in their earlier years of reproduction. We observed an inverse relationship between the number of pregnancies and the frequency of presentation of this pathology. The majority of affected fetuses showed a phenotype that fit into one of many groups. Therefore, we propose classifying the amniotic band sequence phenotypes into the following groups: I. Craniofacial defect + limb defect. II. Craniofacial defect + limb defect + abdominal wall, spinal column, and/or thoracic defects. III. Limb defect + abdominal wall, spinal column, and/or thoracic defects; and IV. Isolated defect (craniofacial, limb, or thoraco-abdominal wall). This classification system will be helpful in diagnosing amniotic band sequence. Based on future research studies, we hope that we can use this classification system as a prognosis fetal factor to establish a more accurate fetal prognosis and recurrence probability. Finally, we created a flowchart describing all of the steps that were followed by our Department from the moment an amniotic band was found by ultrasound until the definitive diagnosis was made and the follow up according to the fetal findings.

Síndrome de limb-body wall complex en embarazo gemelar a propósito de un caso y revisión de la literatura / Syndrome of Limb-body wall complex in twin Dizygotic pregnancy topurpose a case and review of the literature

Presentamos un caso de Limb-Body Wall Complex en embarazo gemelar dicigótico. El gemelar acompañante no tenía malformaciones y solo era pequeño para la edad gestacional. El primer feto se obtiene por vía vaginal normal. Al intentar extraer en poládico al dismórfico no fue posible. Se extrajo por cesárea y muere a los pocos minutos de nacido. El feto malformado presentaba amelia de brazo y pierna derechos, escoliosis severa, toraco-abdominosquisis y cordón con tres vasos. Se apreciaba magma en cara anterior de tronco donde había órganos torácicos externos, rodeados de membrana amniótica. Pensamos que al haber ruptura del amnios en etapa temprana embrionaria, se producen adherencias toraco-parietales que mantienen el corazón fuera del tórax e impiden el cierre esternal para siempre junto a un fenómeno de disrupción vascular que ocasionaria las malformaciones de los miembros y cabeza