Amniotic band syndrome associated with sacral meningocele and anal atresia

Amniotic band syndrome (ABS) is characterized by congenital annular bands associated with different malformations, and the amniotic bands may disrupt the skin, extremities (amputation, ring constrictions, syndactyly), face (clefts), or trunk (abdominal or thoracic wall defects). Encephaloceles and craniofacial defects are frequently reported, but spinal defects are very rare in association with ABS.According to our literature review, this association has not been reported in the literature to date. A female neonate was born at 29 weeks estimated gestational age to a 28-year-old gravida 2, para 1 woman. Her parents were not consanguineous. The mother reported oligohydroamniosis but no recent infections or other illness, no prenatal exposure to teratogenic agents, and no family history of congenital malformations. On physical examination the neonate’s weight was 1310 g (50–75th percentile), and her length was 30 cm (3–10th percentile). Her head circumference was 27.8 cm (75–90th percentile). Ocular, auricular, and oral structures were normal. On the left hand the index and middle fingers were amputated from the distal interphalangeal joint, and the fourth and fifth fingers were amputated from the proximal interphalangeal joint. Syndactyly between the fourth and fifth fingers was noted. On the right hand, the first four digits were amputated at the level of the proximal interphalangeal joint, and syndactyly between these fingers was also noted (Fig. 1). The left lower extremity showed complete constriction in the cruris and thigh. Furthermore, pes varus deformity and rudimentary accessory finger were noted. The right lower extremity showed semi-constriction between femur and tibia. Ring constriction around the right distal cruris was noted. The plantar face of the right foot had skin pedicle. In addition, anal atresia, rectovestibular fistula, sacral pedicle, and sacral meningocele were noted (Fig. 1). The infant had normal tone and strength in all extremities. There were no neurological deficits. Magnetic resonance imaging of the spine demonstrated a defect in the posterior arch at the level of the S2–3 and saccula had no neural tissue (meningocele). Renal ultrasonography was normal. Anal atresia, rectovestibular fistula, and sacral meningocele were corrected at 2 months of age. The band on the right thigh was corrected by Z plasty incision procedure in the 10 th month. Syndactyly was corrected at the age of 3 years. The patient is in follow up and is due for other operations. A high incidence of prematurity and associated low birthweight have been reported. The present patient also was a very low-birthweight neonate. Involvement of the craniofacial region can occur in up to one-third of cases. Cranial defects associated with ABS include hydrocephalus, microcephaly, asymmetric encephalocele, meningocele, exencephaly, acrania, acalvaria, and anencephaly.Although ABS with spinal defects has been reported rarely, this is the first case reported of an association of ABS with sacral meningocele and anal atresia.In conclusion, we emphasize that it is crucial to look for other anomalies in patients with ABS.