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Limb Threatening Constriction Ring Syndrome of Right Leg

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Authors:  Farrukh Mahmood,* Shehzadi Tasneem

Institute: Department of Pediatric Plastic Surgery, The Children’s Hospital and the Institute of Child Health Lahore, Pakistan

Address for Correspondence: Dr. Farrukh Mahmood, Department of Pediatric Plastic Surgery, The Children’s Hospital and the Institute of Child Health Lahore, Pakistan

*Email: drfarrukhmahmood@gmail.com

* Corresponding Author



Journal of Neonatal Surgery

Volume 1(3), Jul-Sep 2012

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Submitted On: 11-03-2012

Accepted On: 15-04-2012

 Published on: 01-07-2012

Local ID: jns-1-35

J Neonat Surg 2012; 1(3): 47

© Mahmood et al, 2012

Conflict of Interest: None declared

Source of Support: Nil


How to cite:

Mahmood F, Tasneem S. Limb threatening constriction ring syndrome of right leg. J Neonat Surg 2012; 1: 47.


Figure 1: Various steps of multiple Z-plasties. (A) Marking, (B) Release (C) Z-plasties.


Figure 2: At two weeks follow up- lymphovenous edema had reduced by 40%.


A 25-day-old male neonate was received in our outpatient department with constriction ring of right leg with bluish discoloration and swelling of foot and acro-syndactly of both hands. The constriction ring was threatening to the limb that already had lost digits as an in-utero event. Multiple Z-Plasties of half circumference of leg was done with release of constriction band. Wound was stitched with 5/0 vicryl rapide (Fig.1). Patient had uneventful recovery and swelling quickly subsided by 40% during two weeks of the operation (Fig.2). The patient is under close surveillance for need of multiple Z-plasties of the remaining half of the constriction ring.


Constriction ring syndrome (CRS) is a rare congenital anomaly characterized by partial or complete constriction anywhere around the limb. It can lead to limb threatening complications. CRS was first described by Van Helmont in 1689 as intrauterine amputation and Montgomery in 1832 as free strands of tissue. Estimated incidence is approximately 1 in 10,000 live births with equal sex distribution. Malformation can be classified into five groups which aids in determining the timing and type of surgical management. Club foot, facial clefts, cleft lip & palate, body wall defects, and cutis aplasia are common associations. Tight constriction ring leads to distal lymphovenous obstruction and threat to the distal extremity [1,2]. Our case of CRS with distal obstruction was presented with limb threatening situation where extremity was salvaged by immediate surgical intervention.



  1. Samra S, Samra AH. Threatened lower extremity in a neonate from a severly constricting amniotic band. Ann Plast Surg. 2006;57:569–72.

  2. Sentilhes L, Verspyck E, Patrier S, Eurin D, Lechevallier J, Marpeau L. Amniotic band syndrome: pathogenesis, prenatal diagnosis and neonatal management. J Gynecol Obstet Biol Reprod. 2003;32:693–704.

This is an Open Access article distributed under the terms of the Creative Commons Attribution unported License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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