Congenital Hand Amputation From Amniotic Band Constriction
Congenital amputations of the limbs are not uncommon. However, it is very rare to have all the carpals, metacarpals and phalanges absent of only one hand. A 3-week-old boy was brought to the hospital for his first newborn visit. The infant had been born to a 35-year-old mother at 39 weeks of gestation. The pregnancy was uncomplicated. The mother was not exposed to any drugs or toxins during the pregnancy, and there was no family history of congenital malformations. Findings of prenatal ultrasonography were reported as normal.
Physical examination showed that the boy’s left hand had no digits. He had normal extension and flexion of the wrist. The right hand was normal.
Radiographs show a single small bone, perhaps a metacarpal base, at the little finger, but no other structures in the carpus or the fingers consistent with cartilaginous bones. The distal portions of the radius and ulna are unremarkable.
The boy received a diagnosis of congenital amputation of the left hand, most likely due to amniotic band constriction.
The amniotic band consists of fibrous strands that extend from the outer surface of the chorion into the amniotic cavity.2 During intrauterine life, these amniotic fibrous bands can cause amputation and ring constrictions of fetal digits and limbs. A higher incidence of such constrictions is observed in earlier pregnancy, and they occur during the first trimester.4
Causative factors include inflammatory reaction, abnormal development of the amnion, defective development of fetal tissue, and premature rupture of the amnion.3 Deficiency of arterial blood supply is responsible for growth retardation and leads to micromelia or osseous or soft-tissue necrosis, which may be followed by shrinkage and sequestration, causing spontaneous amputation.3-5
Our patient will be treated with a prosthetic hand. Review of the literature recommends fitting of a prosthesis anywhere between the ages of 2 months and 2 years in such cases.1,2 The incidence of rejection is low when the prosthesis is applied before 2 years of age.1 The development of hooks for hands and the use of myoelectric hands have empowered persons with hand amputations to lead a near-normal life.1 Body-powered prostheses generally are used. Early fitting of the prosthesis and early training helps the child psychologically. The prostheses do require adjustments as the child grows.
References
1. Shaperman J, Landsberger SE, Setoguchi Y. Early upper limb prosthesis fitting: when and what do we fit. J Prosthet Orthot. 2003;15(1):11-17.
2. Scotland TR, Galway HR. A long-term review of children with congenital and acquired upper limb deficiency. J Bone Joint Surg Br. 1983;65(3):346-349.
3. Torpin R. Amniochorionic mesoblastic fibrous rings and amniotic bands: associated constricting fetal malformations or fetal death. Am J Obstet Gynecol. 1965;91:65-75.
4. Cignini P, Giorlandino C, Padula F, Dugo N, Cafà EV, Spata A. Epidemiology and risk factors of amniotic band syndrome, or ADAM sequence. J Prenat Med. 2012;6(4):59-63.
5. Jain S, Lakhtakia PK. Profile of congenital transverse deficiencies among cases of congenital orthopaedic anomalies. J Orthop Surg (Hong Kong). 2002;10(1):45-52.
Kallol K. Set, MD, and Mahdi Alsaleem, MD
Children’s Hospital of Michigan, Wayne State University School of Medicine, Detroit