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Partial Glossectomy in a 2-month-old Infant with Beckwith-Wiedemann Syndrome - A Case Report
Syed Fareed Mohsin1, Azzam Mansour Alrodhan2, Yasin Alavi Aruveeti3
1Department of Oral and Maxillofacial Diagnostic Sciences, College of Dentistry, Qassim University, Buraydah, Saudi Arabia.
Insights
Early glossectomy for Beckwith-Wiedemann syndrome (BWS) in infants significantly improves breathing and feeding. This intervention enhances airway function and overall quality of life for affected children.
Area of Science:
- Genetics
- Pediatric Surgery
- Developmental Biology
Background:
- Beckwith-Wiedemann syndrome (BWS) is a rare genetic overgrowth disorder.
- BWS presents with diverse clinical manifestations, including macroglossia, increasing health concerns.
- Management requires a multidisciplinary approach for metabolic, multi-organ, and structural issues.
Observation:
- A 56-day-old infant with BWS exhibited macroglossia.
- The enlarged tongue caused significant difficulties with sleeping, feeding, and breathing.
- Physical examination confirmed the macroglossia and associated functional impairments.
Findings:
- The infant underwent a successful anteroposterior keyhole glossectomy.
- Surgical intervention improved airway function, eating, and overall quality of life.
- Post-operative follow-up showed normal tongue position, improved respiration, and resolution of feeding issues.
Implications:
- Early surgical intervention for BWS-related macroglossia is crucial.
- Glossectomy can effectively address respiratory compromise and obstructive sleep apnea/hypopnea syndrome.
- Timely treatment promotes normal growth and development in infants with BWS.
Rationale:
Beckwith-Wiedemann syndrome (BWS) is a rare genetic disorder characterised by aberrant growth patterns, tumour risk and a wide range of clinical symptoms. This illness necessitates a multidisciplinary strategy to address the metabolic, multi-organ and structural issues of considerable health concern.
Patient Concern:
A 56-day-old infant presents with macroglossia due to BWS.
Diagnosis:
On examination, the infant revealed an enlarged protruded tongue causing difficulty in sleeping, feeding and breathing.
Treatment:
The infant underwent a successful anteroposterior keyhole glossectomy, which improved his airway function, eating and overall quality of life. Tongue tissue was carefully excised and anatomically reconstructed to enhance the function.
Outcome:
The patient was followed for up to 22 months; the results were acceptable, with the tongue in its normal position, improved breathing and no feeding issues.
Take-Away Lesson:
In this case report, early intervention improved respiratory function, obstructive sleep apnoea/hypopnoea syndrome and encouraged normal growth and development.
