Home | About Us | Current Issue | Ahead of print | Archives | Search | Instructions | Subscription | Feedback | Editorial Board | e-Alerts | Login 
Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
 Users Online:1387 
  Print this page Email this page   Small font sizeDefault font sizeIncrease font size


 
CLINICAL IMAGE
Year : 2010  |  Volume : 15  |  Issue : 3  |  Page : 110-111
 

Popliteal pterygium syndrome


Department of Pediatric Surgery, Advanced Pediatrics Center, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012, India

Date of Web Publication18-Oct-2010

Correspondence Address:
Prema Menon
Department of Pediatric Surgery, Advanced Pediatrics Center, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9261.71747

Rights and Permissions

 



How to cite this article:
Mahalik SK, Menon P. Popliteal pterygium syndrome. J Indian Assoc Pediatr Surg 2010;15:110-1

How to cite this URL:
Mahalik SK, Menon P. Popliteal pterygium syndrome. J Indian Assoc Pediatr Surg [serial online] 2010 [cited 2019 Sep 22];15:110-1. Available from: http://www.jiaps.com/text.asp?2010/15/3/110/71747


A term male baby weighing 2490 gms was seen with a rare anomaly. The left knee was flexed secondary to a tight popliteal web. The left foot had talipes equinovarus deformity with absence of 4 th and 5 th toes. Another toe was present behind the heel giving the appearance of a partial duplication of the foot. Penoscrotal transposition, bifid scrotum with bilateral descended testis, and proximal penile hypospadias were noted. A large mucosal patch was seen in the perineum on the left side [Figure 1]. The anal opening was normally located. The left kidney was not visualized on ultrasonography. Radiographs of the left lower limb showed normal long bones and a rotated calcaneum.
Figure 1: Left popliteal web with bifid scrotum, penoscrotal transposition, abnormal perineal mucosal patch, and foot anomaly

Click here to view


The popliteal pterygium syndrome is a rare autosomal dominant disorder seen in 1 in 300,000 live births. The characteristic feature is a web which usually extends from the heel to the ischial tuberosity. This contains a palpable cord of connective tissue and occasionally the popliteal artery and peroneal nerve. Absence of muscles or abnormal muscle and tendon insertion may be associated. Other anomalies show a wide range of expressivity and affect the face, limbs, and genitalia. Orofacial anomalies include cleft palate, cleft lip, micrognathia, ankyloblepharon, and choanal atresia. Anomalies of nail and digits, talipes equinovarus, spina bifida occulta, bifid ribs, and short sternum are described. Genital anomalies include hypoplastic labia majora, vagina and uterus, clitoral hypertrophy, cryptorchidism, bifid or absent scrotum and ambiguous genitalia. There is no growth disturbance and intelligence is usually normal. [1],[2]

Prenatal sonography may detect an associated cleft lip/ palate along with inability of the fetus to stretch the knee. Magnetic resonance imaging is the test of choice before resection of fibrous bands and Z-plasty of the web. [3],[4] Nerve grafting may be required for a short sciatic nerve. The overall prognosis is good. The index patient is awaiting staged reconstruction of the various anomalies.

 
   References Top

1.Froster-Iskenius UG. Popliteal pterygium syndrome. J Med Genet 1990;27:320-6.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]  
2.Sasidharan CK, Ravi KV. Popliteal pterygium syndrome with unusual features. Indian J Pediatr 2004;74:269-70.  Back to cited text no. 2      
3.Donnelly LF, Emery KH, Do TT. MR Imaging of popliteal pterygium syndrome in pediatric patients. AJR 2002;178:1281-4.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]  
4.Gardetto A, Piza-Katzer H. A case of familial popliteal pterygium syndrome: Early surgical intervention for successful treatment. Pediatr Surg Int 2003;19:612-4.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]  


    Figures

  [Figure 1]



 

Top
Print this article  Email this article
Previous article Next article

    

 
  Search
 
   Next article
   Previous article 
   Table of Contents
  
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Article in PDF (665 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    References
    Article Figures

 Article Access Statistics
    Viewed7093    
    Printed87    
    Emailed0    
    PDF Downloaded121    
    Comments [Add]    

Recommend this journal


Contact us | Sitemap | Advertise | What's New | Copyright and Disclaimer 

  2005 - Journal of Indian Association of Pediatric Surgeons | Published by Wolters Kluwer - Medknow 

Online since 1st May '05