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Multiple pterygium syndrome: Challenge for anesthesiologist.

P Sethi1, P K Bhatia1, N Gupta2

  • 1Department of Anesthesia and Critical Care, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India.

Saudi Journal of Anaesthesia
|July 5, 2016
PubMed
Summary

Multiple pterygium syndrome (MPS) is a rare genetic disorder causing joint contractures and skin webbing. This case highlights MPS presenting with clubfoot and vertical talus in a pediatric patient requiring orthopedic intervention.

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Area of Science:

  • Genetics and rare diseases
  • Pediatric orthopedics
  • Anesthesiology

Background:

  • Multiple pterygium syndrome (MPS) is an extremely rare autosomal recessive disorder.
  • Key features include joint contractures, skin webbing, spinal deformities, and potential airway difficulties due to craniofacial anomalies.
  • Airway management in MPS patients is challenging due to micrognathia, limited neck extension, and restricted mouth opening.

Observation:

  • A 5-year-old female diagnosed with MPS presented with bilateral clubfoot and congenital vertical talus.
  • These foot deformities are significant orthopedic manifestations of the syndrome.
  • The patient required orthopedic intervention for these conditions.

Findings:

  • The case underscores the variability of physical manifestations in MPS.
Keywords:
Anesthesiachallengesmultiple pterygium syndrome

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  • Congenital vertical talus and clubfoot are notable orthopedic findings in this rare syndrome.
  • Successful anesthetic management is crucial for orthopedic procedures in affected children.

    • Implications:

    • Early diagnosis and management of orthopedic deformities in MPS are essential for functional outcomes.
    • This case emphasizes the importance of a multidisciplinary approach involving genetics, orthopedics, and anesthesiology.
    • Further research into the orthopedic spectrum of MPS can improve patient care and treatment strategies.