Updated: 11/23/2019

Congenital Popliteal Webbing

0%
Topic
Review Topic
0
0
N/A
N/A
Questions
1 1
0
0
https://upload.orthobullets.com/topic/422805/images/image..jpg
https://upload.orthobullets.com/topic/422805/images/imag..jpg
Introduction
  • Overview
    • congenital popliteal webbing is an inherited condition that affects the face, limbs, and genitalia
      • also known as popliteal pterygium syndrome
      • treatment is usually surgical resection
  • Epidemiology
    • incidence
      • rare
        • affects 1:300,000
    • demographics
      • affects males and females equally
  • Genetics
    • inheritance pattern
      • autosomal dominant
      • can also be sporadic
        • associated with advanced paternal age
    • mutations
      • results from a mutation in the IRF6 gene
      • encodes a transcription factor that is active in cells that give rise to tissues in the head and face
        • also involved in the development of the skin and genitalia
  • Associated conditions
    • cleft lip/palate
    • syndactyly
    • criptorchidism
    • bifid scrotum
    • hypoplasia of the labia majora
    • congnitive delays
    • spina bifida
Anatomy
  • Popliteal pterygium 
    • subcutaneous fibrous band
      • lies superficially along the free edge of the pterygium
    • sciatic/peroneal nerve
      • lies deep to the fibrous band, usually halfway between the free edge and the apex
    • popliteal artery/vein
      • usually lies deep in the popliteal space
Presentation
  • Physical exam
    • extensive web running behind the knee from ischial tuberosity down to the heel
      • usually bilateral
      • limits lower extremity extension, abduction, and rotation
    • syndactyly
    • triangular folds of skin over the nails of the large toes
    • tissue connecting the upper and lower eyelids (ankyloblepharon) or jaws (syngnathia)
    • abnormal genitalia
      • hypoplastic labia majora in females
      • cryptorchidism or bifid scrotum in males
    • cleft lip/palate
Imaging
  • Radiographs
    • recommended views
      • AP
      • lateral
    • findings
      • knee flexion contracture
  • MRI
    • indications
      • operative planning
        • used to assess location of neurovascular structures given wide variation and risk for injury
Treatment
  • Nonoperative
    • observation
      • indications
        • rare
  • Operative
    • surgical resection 
      • indications
        • most cases
Techniques
  • Surgical resection
    • technique
      • resection or tenotomy of fibrous band
      • z-plasty of overlying skin
      • can perform sciatic nerve grafting or tendon lengthening if needed
 

Please rate topic.

Average 4.0 of 1 Ratings

Questions (1)

(SAE07PE.94) Figures 43a and 43b show the clinical photographs of a 4-month-old child with bilateral popliteal pterygium. The fixed knee contractures measure 100 degrees bilaterally. What future treatment is most likely to successfully correct this deformity? Review Topic | Tested Concept

QID: 6154
FIGURES:
1

Serial casting of both knees weekly

12%

(23/189)

2

Physiotherapy and dynamic splinting

7%

(13/189)

3

Soft-tissue releases of the knees, including Z-plasties of skin, excision of fibrotic bands, hamstring lengthenings, and posterior knee capsulotomies

50%

(95/189)

4

Femoral shortening osteotomies combined with soft-tissue releases of the knees (Z-plasties of skin, excision of fibrotic bands, hamstring lengthenings, and posterior knee capsulotomies)

26%

(50/189)

5

Gradual correction with a circular external fixator without soft-tissue release

2%

(4/189)

N/A E

Select Answer to see Preferred Response

Topic COMMENTS (0)
Private Note