9/18/2014
Congenital Constriction Ring Syndrome
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Congenital Constriction Ring Syndrome
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Avantika Singha, Krishna Adit Agarwalb a-bVardhmanMahavir
PRINTER FRIENDLY VERSION
Medical College &Safdarjung Hospital, New Delhi
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Corresponding Author: Avantika Singh House Number 285, Devli, New Delhi, Delhi-110062 E-mail:
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Singh A Agarwal KA Search Google Scholar for
Received: 14-APR-2014 Accepted: 31-MAY-2014 DOI: http://dx.doi.org/10.7869/jymr.25
Singh A
Published Online: 06-JUN-2014
Agarwal KA Article Statistics
Abstract A 15-year old male presented with congenital constrictionring syndrome affecting multiple limbs with right-lower limb being most severely affected. He had a deep circumferential constriction band present immediately distal to the knee joint on his right-lower limb. The limb distal to the band had severe lymphedema and hyperpigmentation. It had sensory compromise and cyanosis with a nonpalpabledorsalispedis and feeble popliteal pulse.Left-lower limb showed congenitally amputated toes. Right-upper limb showed acrosyndactyly(fusion of ring and index fingers) with a fenestration (sinus) presentat the base of the web fingers (not visible in the Figures 1, 2). Keywords :
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Congenital Constriction Ring Syndrome, Amniotic Band Syndrome, Amniotic Bands.
Introduction Congenital constriction ring syndrome also known as amniotic band syndrome, Streeter dysplasia, and constriction band syndrome is categorized as a class VI congenital limb malformation.[1,2]It has an incidence ranging from 1/1200 to 1/15,000 live births and a 1:1 ratio of male: female prevalence.[3] The exact cause has not been elucidated and multiple theories have been postulated including intrinsic, extrinsic and intrauterine trauma theory.[4] It is usually associated with multiple and highly variable congenital anomalies (40%-80% cases) and it is very rare to find a single ring with no other manifestations.[4,5]Club foot is seen in up to one-third cases.[6] It manifests with asymmetric anomalies and no two patients have similar manifestations.[5,7]Some anomalies associated with this disorder are craniofacial abnormalities (eg,encephalocele, clefts in unusual locations, anencephaly), body wall defects (abdominal or thoracic contents may herniate through this defect into the amniotic cavity), visceral defects (eg, lung hypoplasia). Scoliosis and other spinal defects may be present. Ambiguous genitalia may be seen. Often a short umbilical cord is associated due to the restricted motion of the fetus. Multiple limb defects can be seen including but not limited to constriction rings, amputation, syndactyly, clubfoot and hand deformities.[8] The location of the bands, resulting amputations or lymphedema, presence of syndactyly or acrosyndactyly vary from patient to patient.[2,5]The main objectives for the treatment of this syndrome are improvement of function and improvement of cosmetic appearance of the limb.[9]. Different surgical techniques are usedin a one or two stage procedure such as Z-plasty, W-plasty or direct closure after the excision of the constricting band.[2,3]
However, in this case when the patient presented the limb was not salvageable. Patient was offered amputation. References 1. Swanson AB. A classification for congenital limb malformations.J Hand Surg. 1976; 1(1): 8-22. 2. Light TR, Ogden JA. Congenital constriction band syndromepathophysiology and treatment.Yale J Biol Med. 1993;
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9/18/2014
Congenital Constriction Ring Syndrome
66: 143-155. 3. Choulakian MY, Williams HB. Surgical correction of congenital constriction band syndrome in children: replacing Zplasty with direct closure. Can J Plast Surg. 2008 Winter; 16(4): 221-223. 4. Adu EJ, Annan C. Congenital constriction ring syndrome of the limbs: a prospective study of 16 cases. Afr J PaediatrSurg(serial online) 2008; 5: 79-83.Available athttp://www.afrjpaedsurg.org/text.asp? 2008/5/2/79/44182.Accessed Apr 17, 2014 5. Patterson T. Congenital ring constrictions. Br J Plast Surg. 1961; (14): 1-31. 6. Foulkes GD, Reinker K. Congenital constriction band syndrome: a seventy-year experience. J PediatrOrthop. MarApr, 1994; 14(2): 242-248. 7. Chandran S, Lim MK, Yu VY. Fetal acalvaria with amniotic band syndrome.Arch Dis Child Fetal Neonatal Ed. Jan 2000; 82(1): F11-F13. 8. tenDonkelaar HJ, Hamel BC, Hartman E, et al. Intestinal mucosa on top of a rudimentary occipital meningocele in amniotic rupture sequence: disorganization-like syndrome, homeotic transformation, abnormal surface encounter or endoectodermal adhesion? ClinDysmorphol. 2002; 11:9. 9. Kawamura K, Chung KC. Constriction band syndrome. Hand Clin. May 2009; 25(2): 257-264.
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