Amniotic Band Syndrome

Abstract

Amniotic band syndrome is an uncommon congenital disorder without any genetic or hereditary disposition. It involves fetal entrapment in strands of amniotic tissue and causes an array of deletions and deformations. Primary treatment is plastic and reconstructive surgery after birth with in utero fetal surgery also coming in vogue.

Case Report and Discussion

A 9-month-old baby boy, of consanguineous parents, presented to us with constricting band around both the arms and legs (Fig. 1).There was either partial or total autoamputation of the finger digits and toes (Fig. 2). There was right clubfoot with syndactyly. This condition is known as amniotic band syndrome. He underwent Z-plasty in staged phase and is doing well on follow-up.

Fig. 1.

Ring constriction bands over the upper and lower limb with club foot and syndactyly

Fig. 2.

Autoamputation of the digits

The condition is also known as ADAM complex, amniotic band sequence, Streeter’s dysplasia, congenital constriction bands, and pseudoainhum.

It is found approximately 1 in 1200 live births. It is a sporadic condition and does not appear to be genetic or hereditary. It presents with constriction bands, autoamputations, and other deformities of the fetal limbs, digits, and body.They are associated mostly with other congenital anomalies such as syndactyly, clubfoot, cleft palate, and cleft lip and can also present with severe craniofacial and visceral deformities [1].

Two pathogenesis theories have been put forward: the exogenous theory, which proposes the early partial rupture of the amniotic sac leading to fibrous bands; these fibrous bands float in the amniotic fluid and can encircle and entrap a part of the fetus.These act as constricting bands as the fetus grows, causing reduced blood circulation, wich can lead to autoamputation of a digit or limb in utero [2]. In some cases, it leads to necrosis requiring surgical amputation following birth. The other endogenous theory seconds vascular compromise [3].

The diagnosis is difficult; a prenatal ultrasound scan can help indirectly showing swelling of digits or limbs distal to the constriction [2]. Three dimensional ultrasound and magnetic resonance imaging can be used for more detailed and accurate diagnosis when suspicious about amniotic band syndrome [4].

Treatment mostly occurs after birth, but with advancement of prenatal radiodiagnosis, fetal surgery in utero has been tried [5]. Plastic and reconstructive surgery planning with timing of repair are important to provide improved function and development while providing a more acceptable aesthetic appearance [6]. Surgery can range from simple to complex depending on the deformity and may require long-term physical and occupational therapy.