congenital contriction band

Congenital constriction bands are anomalous bands that encircle a digit or an extremity. Congenital constriction band syndrome is  rare condition and is mostly associated with other musculoskeletal disorders

Congenital bands, also called ring constrictions,rather than lower extremity

Streeter dysplasia, amniotic bands, annular defects are

anomalous bands that encircle, either partially or

completely a digit or an extremity(1). Incidence (2) of

this condition in new born is 01%. These may involve

just the skin but they can be as deep as the underlying

skeletal structures and some have caused subtotal or total

autoamputation (3). The part distal to the band may appear

to be entirely normal, or it may be edematous or even

gangrenous. Additional musculoskeletal disorders that may

be present include club foot,synacdactly or

acrosynacdactly, hypoplastic nails, hypoplastic fingers,

pseudoarthrosis of underlying bones,absence of

bones,peripheral nervedefects,distal lymphodema,

intrauterine amputations, cleft lip and cleft palate and

umblical hernia (4). Etiology of this syndrome is unknown.

This syndrome may be caused by prenatal environmental

factors and it appears to be result of excessive contraction

of the uterine muscles and hemorrhages from marginal

blood sinus.Being a rare condition and mostly associated

with other musculoskelton disorders, it is worth reporting.

Case

A boy aged six years  old , borned with full term normaldelivery, presented to bangkinang hospital

                                     

Occasionally, constriction ring syndrome can be diagnosed before birth by

prenatal ultrasound. However, the majority of cases are diagnosed at or

shortly after birth. The diagnosis is typically made by the treating physician

after a thorough medical history and physical examination. X-rays of

the affected limb are also used to help assess the degree of involvement.

How is constriction ring syndrome treated?

Treatment of constriction ring syndrome is individualized according to your

child’s specific condition. In general, if your child has shallow, incomplete

constriction rings, he may be treated with a simple day surgical procedure.

If your child has deep constriction rings, one or more surgeries may be

required to improve the appearance and function of the affected hand.

Surgeries are usually performed after your child and his hand have had

time to grow (6 months -1 year). Deep constriction rings that impede

blood flow must be addressed immediately, however. It is important to

note that despite the differences in appearance, affected hands/fingers

generally have excellent long-term function.

How is constriction ring syndrome treated?

Treatment of constriction ring syndrome is individualized according to yourchild’s specific condition. In general, if your child has shallow, incompleteconstriction rings, he may be treated with a simple day surgical procedure.If your child has deep constriction rings, one or more surgeries may be required to improve the appearance and function of the affected hand.Surgeries are usually performed after your child and his hand have hadtime to grow (6 months -1 year). Deep constriction rings that impede blood flow must be addressed immediately,  however. It is important to note that despite the differences in appearance, affected hands/fingers generally have excellent long-term function.

Constriction ring syndrome occurs in about 1 in every 10,000-15,000

births. It is believed to occur more frequently in the upper extremity

.