Amniotic Band Syndrome (Streeter Dysplasia) Treatment & Management

Updated: Sep 10, 2020
  • Author: Twee T Do, MD; Chief Editor: Jeffrey D Thomson, MD  more...
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Treatment

Approach Considerations

Indications for intervention to treat amniotic band syndrome (ABS; also referred to as amniotic band sequence or Streeter dysplasia) depend on the medical stability of the child and on the neurovascular status of the limb. Anencephaly usually is incompatible with life, but the other deformities can be triaged for correction and reconstruction. Of all the deformities, only the tight constriction bands with gross lymphedema, vascular compromise, or both necessitate immediate surgical release.

Clubfeet should be manipulated and put in casts early, as is the case for all idiopathic clubfeet. The paralytic feet in ABS, however, usually are severe and do not respond well to conventional treatment. Surgical correction is also associated with a high incidence of recurrence and complications due to neurovascular injury from proximal bands over the peroneal nerve.

Cleft lip and palate require reconstruction, but this is best done when the child is aged approximately 3-6 months, depending on the severity of the cleft and the infant's ability to feed.

Mild bands that only cosmetically affect the superficial skin, similar to the creases found in the Michelin tire baby syndrome (circumferential-ringed creases with specific histologic findings and/or karyotype abnormalities), do not require any intervention. As growth occurs, progressive constriction and edema may necessitate band excision and Z-plasty, but in general, excision is not indicated for superficial bands, because of the potential complications of wound infections and neurovascular compromise.

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Medical Therapy

Because ABS is an intrauterine phenomenon probably caused by the rupture of amniotic membranes and constriction of the developing tissue, no medical treatment exists for the condition. Avoiding certain drugs that can lead to spontaneous rupture of membranes, such as cocaine and mifepristone, may help decrease the potential risk.

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Surgical Therapy

Tight constrictions on the digits or extremities often render urgent surgical treatment necessary for patients with vascular compromise. Surgery also is indicated for patients with syndactyly or acrosyndactyly that compromises hand function. Thumb amputation (which is rare), clubfeet, cleft lip, and cleft palate also warrant reconstruction, but these procedures can be performed electively at a later time and at the discretion of the physician and family. [35]

In bands identified by three-dimensional (3D) ultrasonography (US) as causing neurovascular compromise, early in-utero fetoscopic surgery for release can be performed. Early studies in animal models yielded promising results. [36] Multiple series of case reports also documented the effectiveness of fetoscopic releases on the outcomes for the constrictions affecting the fetus and the relative safety of the procedure for the mothers. [37, 38, 39, 40, 41]

Early intervention for severe constriction bands after birth includes band excision with 1-2 mm of normal skin to avoid recurrence. The entire band must be excised. This is performed on a maximum of 65% of the ring (most authors recommend 50%) by Z-plasty for lesser constrictions and by V-Y plasty or W-plasty for tighter bands. A staged correction ensures adequacy of vascularity to the residual limb or digit. Additionally, debulking of the fibrofatty soft tissues followed by subcutaneous tissue advancement as described by Upton [42] may further improve the cosmetic appearance of the digits after band release.

For a study of the formation of phalanges and small joints through tissue engineering, see the work of Isogai et al. [43]

For severely constricted bands with wide tissue excision, W-plasty is preferred to provide additional skin for closure. In band excision, intraoperative consideration should be given to possible attenuation of the neurovascular bundle and its proximity to the surface of the band. Careful dissection under magnifying loupes prevents possible damage to these vital structures.

The first part of acrosyndactyly release consists of separation of the digits, especially if the constricting rings are not compromising circulation. Digits are separated when infants are aged 6 months to 1 year. Dobyns reported that the number of fingers is not as important as their length, bulk, stability, spacing, and control. [44]

The groin is prepared for skin graft harvest, which will be necessary for closure. Reconstruction typically is performed in a proximal-to-distal direction and requires complete removal of the sinus tracts, which tend to be located more distally than the normal web spaces are. If the thumb is severely shortened or its function is compromised, the on-top plasty or toe-to-thumb transfer may be considered. Despite cosmetic improvements, the digits will continue to be stiff.

Intrauterine amputations do not warrant intervention unless they involve the thumb at the metacarpophalangeal (MCP) joint. In these cases, the on-top plasty or toe-to-thumb transfer may improve function.

In the lower extremities, teratologic clubfeet associated with deep congenital constriction bands usually require constriction band excision, Z-plasty, and posteromedial release and casting. This is typically described as a staged procedure. The band excision and Z-plasty may be performed in parts (three-stage clubfoot release) or in full (two-stage clubfoot release). [45]

Performing the procedure in staged parts was previously considered necessary to avoid any vascular compromise with the clubfoot surgery, skin sloughing, and infection. Greene, [46] however, published good results in three patients with four affected clubfeet who were treated with band excision, Z-plasty, and posteromedial release in a single full procedure with no significant complications. The benefits of a single complete procedure include ease of postoperative patient care and reduced patient exposure to general anesthetics.

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Complications

Complications from ABS include severe lymphatic or venous congestion at the time of birth due to tight bands. This congestion may lead to necrosis and gangrene [18] if not urgently treated with excision and release. Other potential complications include neurovascular compromise caused by release of the entire band at one sitting or lack of attention to the superficial level of the attenuated nerves and vessels.

Clubfeet are teratologic in 50% of cases and do not respond well to surgical posteromedial releases alone. The constriction bands often require excision, and tendon transfers may be required later because of peroneal nerve compromise.

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Long-Term Monitoring

All patients with ABS should be monitored regularly until skeletal maturity because of the potential for recurrence of the rings and for secondary contractures that may develop and must be addressed on an individual basis.

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