www

35 downloads 0 Views 333KB Size Report
Sep 18, 2014 - Congenital constriction ring syndrome also known as amniotic band syndrome, ... treatment of this syndrome are improvement of function and ...
9/18/2014

Congenital Constriction Ring Syndrome

About the Journal | Editorial Board | Instructions to Contributors | Submission & Review | Advertise with Us | Subscribe to E- Alerts

Sitemap | Feedback | Follow Us Search This Site

Search Advanced search

An Open Access Peer-reviewed Scientific Journal catering to Young Medical Researchers HOME

ONLINE FIRST

CURRENT ISSUE

ARCHIVES

ONLINE SUBMISSION

CONTACT US

Article Options

Congenital Constriction Ring Syndrome

FULL TEXT PDF

Avantika Singha, Krishna Adit Agarwalb a-bVardhmanMahavir

PRINTER FRIENDLY VERSION

Medical College &Safdarjung Hospital, New Delhi

Search PubMed for

Corresponding Author: Avantika Singh House Number 285, Devli, New Delhi, Delhi-110062 E-mail: [email protected]

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 :

Like

19

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;

http://www.journalyoungmed.com/articles/2/1/Congenital-Constriction-Ring-Syndrome.htm

1/2

View publication stats

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.

© 2013 Journal of Young Medical Researchers Online ISSN: 2347 – 9418 The contents of this site are intended for health care professionals and scientific researchers.

Last updated on Jul 25, 2014

http://www.journalyoungmed.com/articles/2/1/Congenital-Constriction-Ring-Syndrome.htm

2/2