Brand-Ad-30-6
 IADVL
Indexed with PubMed and Science Citation Index (E) 
 
Users online: 6216 
     Home | Feedback | Login 
About Current Issue Archive Ahead of print Search Instructions Online Submission Subscribe What's New Contact  
  Navigate here 
  Search
 
   Next article
   Previous article 
   Table of Contents
  
 Resource links
   Similar in PUBMED
    Search Pubmed for
    Search in Google Scholar for
  Related articles
   Article in PDF (105 KB)
   Citation Manager
   Access Statistics
   Reader Comments
   Email Alert *
   Add to My List *
* Registration required (free)  

 
  In this article
   Abstract
   Introduction
   Case report
   Discussion
   References
   Article Figures

 Article Access Statistics
    Viewed7705    
    Printed121    
    Emailed2    
    PDF Downloaded188    
    Comments [Add]    
    Cited by others 6    

Recommend this journal

 


 
CASE REPORT
Year : 2006  |  Volume : 72  |  Issue : 3  |  Page : 222-223

Poland syndrome (anomaly) with congenital hemangioma: A new association


1 Departments of Dermatology, Medical college, Calicut, India
2 Departments of Pediatrics, Medical college, Calicut, India

Correspondence Address:
Najeeba Riyaz
Arakkal, Chalappuram, Calicut 673 002
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0378-6323.25785

Rights and Permissions

  Abstract 

Unilateral defect of pectoral muscle and ipsilateral syndactyly constitute Poland syndrome. Absence or hypoplasia of the breast and nipple, axillary hair loss and dermatoglyphic abnormalities have also been reported in this syndrome. The primary defect could be in the development of the proximal subclavian artery with early deficit of blood flow to the distal limb and the pectoral region, resulting in partial loss of tissue in those regions. However, the association of congenital hemangioma with Poland sequence has not been observed so far. Such an association is being reported here in a 1-year-old infant, second-born of nonconsanguineous parents, who also had polydactyly instead of the documented syndactyly.


Keywords: Congenital hemangioma, Poland syndrome, Polydactyly


How to cite this article:
Riyaz N, Riyaz A. Poland syndrome (anomaly) with congenital hemangioma: A new association. Indian J Dermatol Venereol Leprol 2006;72:222-3

How to cite this URL:
Riyaz N, Riyaz A. Poland syndrome (anomaly) with congenital hemangioma: A new association. Indian J Dermatol Venereol Leprol [serial online] 2006 [cited 2020 Oct 24];72:222-3. Available from: https://www.ijdvl.com/text.asp?2006/72/3/222/25785



  Introduction Top


In 1841 Poland reported unilateral absence of pectoralis minor and the sternal portion of pectoralis major muscle in an individual who also had syndactyly of the hand on the same side.[1] Simple, complete or incomplete syndactyly can occur. This unique pattern has been noted in several cases and the incidence is about 1/30,000. Other associations observed are hypoplasia of the upper extremity (brachysymphalangism); absence or hypoplasia of the breast, nipple; loss of axillary hair and dermatoglyphic abnormalities. It has been estimated that 10% of the patients with syndactyly of the hand have Poland sequence. We report a case of Poland sequence in a girl with congenital hemangioma and polydactyly of the affected side.


  Case report Top


A 1-year-old baby girl, second-born of nonconsanguineous parents, was evaluated for lower respiratory infection. She had a depression on the right side of chest at birth itself and after 5 days, she developed a small compressible red spot, which later extended up to the midline and right upper limb. There was no family history of similar complaints. There was no history of convulsions and her development was normal. Her only sibling, a 5-year-old boy, was normal.

Examination revealed an active child whose weight was 8 kg, length 76 cm and head circumference 45 cm. She had a high arched palate but no facial dysmorphism. The sternocostal part of pectoralis major was absent on the right side. The right nipple was placed at a lower level. There was an extensive well-defined erythematous compressible lesion with surface smooth in most of the areas and lobulated in other areas involving the right side of chest, axilla, flexor surface of the right upper limb and thenar eminence (C 4-7 and T 1 dermatomes) [Figure - 1], suggestive of strawberry angioma. The baby also had preaxial polydactyly on the right side but no syndactyly, brachydactyly or oligodactyly. There was no wasting of muscles, nail changes or Sprengel's deformity (i.e., elevation of scapula on the affected side). Other systems were clinically normal.

Her hemogram and urine analysis were normal. Histopathology of the skin lesion was consistent with strawberry hemangioma. Computerized tomography and MRI scan of the chest confirmed the absence of sternocostal part of pectoralis major muscle on the right side. Radiograph of thoracic spine and ultrasound scan of abdomen did not show any abnormality.


  Discussion Top


The exact etiology of Poland's sequence is unknown. The male-female ratio is 3:1. In 75% of cases, right side is affected. Bouvet[2] et al . and Bavinck[3] et al . have suggested that the primary defect may be in the development of the proximal subclavian artery with diminished blood flow to the affected side, leading to partial loss of tissue in that region. Vascularization of the fetal skin begins during the third month of intrauterine life, but these vessels do not anastomose with the deeper vasculature until late in gestation.[4] It has been suggested that infantile hemangioma arises where islands of embryonic cutaneous angioblastic tissue fail to establish normal contact with the rest of the developing vascular system.[5]

Since Poland's sequence and congenital hemangiomas are of vascular etiology, their association may be due to an intrauterine vascular anomaly rather than an accidental coincidence. Parent-to-child transmission is possible with marked variability in expression. In some patients, absence of the pectoralis muscles alone or in others syndactyly alone can occur.

 
  References Top

1.David TJ. Nature and etiology of the Poland anomaly. N Eng J Med 1972;287:487-9.  Back to cited text no. 1  [PUBMED]  
2.Bouvet J, Maroteaux P, Guillemot M. Poland's syndrome: Clinical and genetic studies. Physiopathol Nouv-Presse Med 1976;5:185.  Back to cited text no. 2    
3.Bavinck J, Weaver DD. Subclavian artery supply disruption sequence: hypothesis of a vascular etiology for Poland, Klippel-Feil and Moebius anomalies. Am J Med Genet 1986;23:903.  Back to cited text no. 3    
4.Folkman J. Towards a new understanding of vascular proliferative disease in children. Pediatrics 1984;74:850-6.  Back to cited text no. 4    
5.Pack GT, Miller TR. Hemangiomas: Classification, diagnosis and treatment. Angiology 1958;1:405-26.  Back to cited text no. 5    


    Figures

[Figure - 1]

This article has been cited by
1 Polandís Syndrome: A Concise Review of the Clinical Features Highlighting Associated Dermatologic Manifestations
Aria Vazirnia,Philip R. Cohen
American Journal of Clinical Dermatology. 2015; 16(4): 295
[Pubmed] | [DOI]
2 Neurofibroma and Pectoralis Muscle Hypoplasia: A Mild Degree of Poland?s Syndrome
Caiping Chen,Jianju Lu,Xiang Lu,Wanxin Wu,Wenlan Han
Breast Care. 2012; 7(6): 490
[Pubmed] | [DOI]
3 Poland Syndrome: Evaluation and Treatment of the Chest Wall in 63 Patients :
Alan E. Seyfer, Justin P. Fox, Conrad G. Hamilton
Plastic & Reconstructive Surgery. 2010; 126(3): 902
[VIEW] | [DOI]
4 Autologous rib transplantation and terylene patch for repair of chest wall defect in a girl with Poland syndrome: a case report
Fang Zhou,Wenying Liu,Yunman Tang
Journal of Pediatric Surgery. 2008; 43(10): 1902
[Pubmed] | [DOI]
5 Autologous rib transplantation and terylene patch for repair of chest wall defect in a girl with Poland syndrome: a case report
Zhou, F., Liu, W., Tang, Y.
Journal of Pediatric Surgery. 2008; 43(10): 1902-1905
[Pubmed]
6 Persistent, arteriopathic ulcers in metameric hemangioma of the lower limb.
Mazzotta, F., Colonna, V., Troccoli, T., Bonifazi, E.
European Journal of Pediatric Dermatology. 2008; 18(1): 13-17
[Pubmed]



 

Top
Print this article  Email this article
Previous article Next article

    

Online since 15th March '04
Published by Wolters Kluwer - Medknow