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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
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.
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.
- 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.
