Q.

What is the diagnosis and how would you proceed?

A.

The image demonstrates an example of Amniotic Constriction Band syndrome, also known as Streeter's syndrome.
The annular constriction bands can involve any part of the body and vary from minor digital deformities to reports of nerve palsies and pseudoarthroses of long bones to congenital amputations, including a case report of a congenital decapitation! The band depth varies from a superficial skin constriction to strangulation of the distal limb.
Most cases are sporadic. Streeter proposed a genetic predisposition. Recent reports suggest that interference with embryonic circulation in the first month of gestation can derange the graded expression of organizational genes resulting in local defects in the organization of the embryo. Others suggest that amniotic bands alone cause the constrictions.
Up to 50% have other congenital anomalies including cleft lip and palate and club foot deformity. Hand and finger anomalies occur in up to 80%.
There is a familial variant which is transmitted as an autosomal dominant trait and carries the eponym " Michelin tire baby syndrome" or multiple benign circumferential skin creases.
Band excision and Z or V-Y plasty repair may suffice for soft tissue lesions. Club feet in association with constriction bands are relatively rigid and difficult to treat.
A fetal lamb model of this disorder has been developed by simple banding of extremities in utero and is claimed to replicate the clinical features of the disease.

References:

Bamford JS.
Amniotic band sequence: Streeter's hypothesis reexmined.
Am J Med Genetics. 1992: 44(3); 280-7.

Bass HN, Caldwell S, Brooks BS.
Michelin tire baby syndrome: familial constriction bands during infancy and early childhood in four generations.
Am J Med Genetics.. 1993: 45(3); 370-2.

Crombleholme TM, et al.
Amniotic band syndrome in fetal lambs.
J Ped Surgery. 1995; 307): 974-8.

Foulkes GD, Reinker K.
Congenital constriction band syndrome; a seven year experience.
JPO. 1994. 14(2): 242-8.

Shipp TD, Genest D, Benacerraf BR.
A case of fetal decapitation.
J Ultrasound in Medicine 1996. 15(7): 535-7.