Amniotic band syndrome with sacral agenesis and umbilical cord entrapment: A case report emphasizing the value of evaluation of umbilical cord

Abstract

Amniotic band syndrome is a rare congenital disorder caused by entrapment of fetal parts by fibrous amniotic bands in utero. The congenital anomalies seen in this syndrome vary widely and defects may be isolated or multiple and do not follow a specific pattern. Asymmetric distribution of defects is the hallmark of this syndrome. The diagnosis is difficult to make on ultrasound and relies on identification of amniotic bands. We report a case of amniotic band syndrome with sacral agenesis diagnosed on routine antenatal ultrasound scan in the second offspring of a recently diagnosed diabetic mother. The associated features were entrapment of umbilical cord, caudal adhesions and lower limb anomalies. Medical termination of pregnancy was done and all the fetal anomalies as well as umbilical cord abnormalities were confirmed. The importance of meticulous scanning to evaluate for amniotic bands and the umbilical cord in addition to the fetal structures is emphasized.

Amniotic Band Syndrome, Perinatal Hospice, and Palliative Care versus Active Management

“…Originally, the intrinsic theory on inherent developmental defects was proposed by Streeter [12, 13]. The most likely explanation, which is supported by the majority of current authors, is the extrinsic theory proposed by Torpin [14–16].…”

“…These findings may be confirmed by fetal MRI, which is often ordered as a complimentary measure upon consideration of fetal surgery [13, 19, 20]. Amniotic bands are now being successfully released fetoscopically through minimally invasive surgery [19].…”

Body wall defects and amniotic band syndrome in pig (Sus scrofa domesticus)

“…Amniotic band syndrome (ABS) is a rare congenital condition caused by entrapment of different embryo parts by fibrous amniotic bands within maternal utero. ABS is characterized by isolated or multiple defects that do not follow a specific pattern and are always asymmetric (Gupta, Venkatesan, Chandra, Rajeswari, & Devi, ). The spectrum of malformations may include body wall defects, cranial anomalies and limb amputation.…”

“…The presence of the characteristic foetal anomalies is enough for amniotic band syndrome diagnosis, even if bands are not present. In human medicine, there is much information on amniotic bands causing congenital diseases (Lockwood et al, ; Gupta et al, ; Martínez‐Frías, , Martínez‐Frías, Bermejo, & Rodríguez‐Pinilla, ; Miller, ; Seeds, Cefalo, & Herbert, ; Torpin, , ). In veterinary medicine, there are some experimental studies about ABS using lambs (Crombleholme et al, ; Devlieger et al, ; Ross, ; Soldado et al, , ; Stelnicki, Hoffman, Foster, Lopoo, & Longaker, ), rabbits (Galvan Montaño et al, ; Rowsell, , ) or rats (López‐Fernández et al, ; Romero‐Valdovinos et al, ).…”

“…ABS is composed of amnion; on the contrary, amniotic sheet membranes are thicker and layered and are composed of two layers of amnion and chorion. Furthermore, amniotic sheet is not related to foetal malformations (Gupta et al, ).…”