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Metopic and Sagittal Craniosynostosis in Williams Syndrome

Sarut Chaisrisawadisuk1, Inthira Khampalikit2, Mark H Moore3

  • 1Division of Plastic Surgery, Department of Surgery, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.

The Journal of Craniofacial Surgery
|May 29, 2023
PubMed

Insights

Surgical intervention for craniosynostosis in infants with Williams syndrome (WS) is feasible. This case report shows successful calvarial remodeling improved global development in a WS patient with metopic and sagittal craniosynostosis.

Area of Science:

  • Pediatric Surgery
  • Genetics
  • Developmental Biology

Background:

  • Craniosynostosis, the premature fusion of skull sutures, is a known complication in Williams syndrome (WS).
  • WS patients often present with complex cardiovascular anomalies, increasing surgical risks, particularly under anesthesia, leading to conservative management preferences.
  • This approach highlights a deviation from conservative management, addressing craniosynostosis surgically in a WS patient.

Purpose of the Study:

  • To report a successful multidisciplinary surgical approach for craniosynostosis in an infant with Williams syndrome.
  • To evaluate the impact of surgical intervention on global development in this specific patient population.

Main Methods:

  • A 12-month-old female infant diagnosed with Williams syndrome and concurrent metopic and sagittal craniosynostosis was managed.
  • A multidisciplinary team approach was employed, involving surgical planning and execution of calvarial remodeling procedures.

Main Results:

  • The infant successfully underwent calvarial remodeling surgery.
  • Post-operative clinical outcomes demonstrated a dramatic improvement in the patient's global development.

Conclusions:

  • Surgical correction of craniosynostosis is a viable option for select patients with Williams syndrome.
  • Calvarial remodeling can lead to significant improvements in neurodevelopmental outcomes in infants with WS and craniosynostosis.

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