Poland Syndrome (Pediatric)

Synonyms
Poland's syndrome, Poland anomaly, Poland sequence, and Poland syndactyly

INTRODUCTION

Definition

A congenital condition with the characteristic absence of chest wall muscles and short, webbed fingers.

Epidemiology

  • Incidence is 1 in 32,000 live births (USA).
  • Occurs in 10 % of syndactyly cases.
  • Males are more often affected than females.
  • The right side is more involved than the left.
  • It may be associated with Klippel-Feil syndrome and Möbius syndrome.

Etiology

  • The exact cause of Poland syndrome is not known. It might be linked to subclavian artery hypoplasia.
  • Most cases arise sporadically.

CLINICAL FEATURES

Signs

Inspection:
  • Chest and shoulder deformities:
    • Unilateral chest wall hypoplasia due to absence of sternocostal head of pectoralis major.
    • Hypoplasia or absence of the deltoid, pectoralis minor, serratus anterior, external oblique, and latissimus dorsi muscles.
    • Scoliosis.
    • Dextrocardia.
    • Sprengel deformity.
    • Absence or underdevelopment of the breast with absent nipple and areola and minimal subcutaneous fat. 
  • Hand deformities:
    • Hypoplasia of hand and forearm: Absence or hypoplasia of metacarpals and phalanges.
    • Syndactyly :
      • Symbrachydactyly and shortening of middle fingers. There is simple syndactyly of ulnar digits and absence of shortening of middle digits.
    • Carpal bone abnormalities: Carpal coalition or hypoplasia.
    • Absence of flexor and extensor tendons.
    • Can be associated with radioulnar synostosis.
    • Nail agenesis.

DIAGNOSIS

Computer Tomography (CT)

CT scan can show an absent perctoralis major. 

Magnetic Resonance Imaging (MRI)

MRI scan can show an absent perctoralis major. 

TREATMENT

Overview

Treatment usually include syndactyly release.

Operative Treatment

Syndactyly release: Caution with lack of soft tissue coverage requiring full thickness skin graft.
  • Indication: Most patients.
  • Technique: Complete syndactyly release produces skin deficiency that requires skin grafting.

COMPLICATIONS

Complications

Complications related to surgery:
  • Excessive tension.
  • Skin graft failure.
  • Improper flap planning.
  • Digital artery injury.
Other complications:
  • Web creep.
  • Nail deformity.
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References