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When A Child Presents With Amniotic Band Syndrome
Noting the uncommon presentation of amniotic band syndrome, these authors offer insights on the diagnosis and treatment of a 3-month-old baby with constrictive bands on both legs.
A 3-month-old patient presented to our institution through the emergency department. Her mother was concerned about right foot swelling and skin discoloration.
According to her mother, the patient was a healthy female without any other medical conditions. The mother noted a normal, non-complicated vaginal birth. She stated that her baby had bands on her legs that were present since birth. The mother noticed they had gotten worse as the child has grown. She noted that the baby’s foot and digits would intermittently become swollen and have purple discoloration.
Upon examining the child, we noted a circumferential band approximately five cm to the right ankle, which was causing constriction and indentation of skin. Another band was present at the left foot, involving the first through third digits with acrosyndactyly to the first and second digits. There was a small wound visible at the left hallux with surrounding dried blood. The fibrous band around the hallux appeared to involve the nail bed, causing the nail to be small and hypertrophic with incurvation.
The extremities displayed no acute evidence of necrosis or ischemia. All digits on the left foot displayed brisk capillary refill. Given the findings, there was no need for any emergent surgical intervention.
What You Should Know About Amniotic Band Syndrome
We diagnosed the patient with amniotic band syndrome. This syndrome, also known as constriction band syndrome, has a reported incidence that varies from 1/1,200 to 1/15,000 live births with no sex predilection.1
Authors have proposed two theories as to the etiology for amniotic band syndrome. The first is the intrinsic theory, proposed by Streeter in 1930, which suggests constriction band syndrome represents an inherent development defect in embryogenesis.2 The bands arise from an endogenous defect in germ plasm differentiation. This causes the limb to become necrotic and form fibrous bands.2,3
In 1965, Torpin proposed the extrinsic theory, which is the most widely accepted etiology today.4 This hypothesis suggests that early amnion rupture forms adherent bands that constrict, entangle and can lead to amputation. As the amnion ruptures, it slips off the chorion to form the detaching mesoblastic fibrous strands that become entangled around the digits or limbs.3,4
Clinically, patients with amniotic band syndrome predominantly present with constrictions of limbs, fingers, syndactyly, acrosyndactyly, phalangeal hypoplasia, and amputation of limbs and fingers. Amniotic band syndrome has also shown an association with clubfoot, limb length discrepancies and other bone anomalies (i.e., cleft lip and palate, visceral and body wall defects, and anencephaly).1
How To Diagnose And Treat Amniotic Band Syndrome
Diagnosis of this condition can occur prenatally with the use of ultrasonography analysis. This allows the visualization of amniotic bands attached to the fetus. The bands are easiest to visualize during the second or third trimesters.5
Treatment ranges from cosmetic repair to emergency, limb-sparing band release. Shallow bands usually do not require operative treatment unless they interfere with circulation or lymphatic drainage. Deep bands typically require release by circumferential Z-plasty or W-plasty. Deep bands typically present with swelling distal to the band, extreme pain and diminished vascularity. Removal of constriction bands usually occurs in a two-stage release, beginning at the age of three months. One releases 50 percent of the band at a time. After re-establishing the cutaneous circulation across the scar, one releases the remaining band. An interval of six to 12 weeks between releases is advised.3
The patient who presented to our institution displayed acrosyndactyly. This type of syndactyly involves adjacent digits with binding in a “lassoed” appearance. The surgical goals for this condition are to create a web space to provide functional results. One should use standard syndactyly techniques when possible. Generally, separate the phalanges with carefully planned zigzag incisions and create a broad commissural space with a dorsal skin flap.3
In Conclusion
Constriction band syndrome is an uncommon congenital abnormality with multiple manifestations. Early amnion rupture with entanglement of fetal parts by amniotic strands is the primary etiology. The management is early recognition and release to improve function and development.
Dr. Megorden is a third-year resident at Maricopa County Medical Center in Phoenix.
Dr. Fishco is board-certified in foot surgery and reconstructive rearfoot and ankle surgery by the American Board of Podiatric Surgery. He is a Fellow of the American College of Foot and Ankle Surgeons, and a faculty member of the Podiatry Institute. Dr. Fishco is in private practice in Phoenix.
References
1. Walter JH Jr., Goss L, Lazzara A. Amniotic band syndrome. J Foot Ankle Surg. 1998; 37(4):325–33.
2. Streeter GL. Focal deficiencies in fetal tissue and their relation to intrauterin amputations. Contrib Embryol Carnegie Inst. 1930; 22(1):1.
3. Kawamura K, Chung KC. Constriction band syndrome. Hand Clin. 2009; 25(2):257–64.
4. Torpin R. Amniochorionic mesoblastic fibrous strings and amniotic bands: associated constricting fetal malformations or fetal deaths. Am J Obstet Gynecol. 1965; 91(1):65–75.
5. Shetty P, Menezes LT, Tauro LF, Diddigi KA. Amniotic Band Syndrome. Indian J Surg. 2013; 75: 401.
