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Trigger finger in children with hurler syndrome - distribution pattern and treatment options
Andreas Jokuszies1, Lorenz Grigull2, Tobias Mett1
1Department of Plastic, Aesthetic, Hand and Reconstructive Surgery, Hannover Medical School, Hannover, Germany.
Insights
Mucopolysaccharidosis patients with trigger fingers and carpal tunnel syndrome experience pain relief and improved hand function after surgical intervention. This study highlights effective treatment for this rare condition.
Area of Science:
- Pediatric Orthopedics
- Rare Diseases
- Metabolic Disorders
Background:
- Mucopolysaccharidosis is a rare genetic disorder causing glycosaminoglycan buildup.
- This buildup can lead to carpal tunnel syndrome and trigger finger due to tendon sheath deposition.
- Early diagnosis and intervention are crucial for managing symptoms.
Purpose of the Study:
- To evaluate the treatment outcomes for trigger fingers in pediatric patients with mucopolysaccharidosis.
- To assess the effectiveness of surgical interventions for associated hand conditions.
- To improve pain relief and hand function in affected children.
Main Methods:
- Interdisciplinary evaluation of 6 pediatric patients with mucopolysaccharidosis-associated trigger fingers.
- Clinical examination including palpation and range of motion assessment.
- Surgical release of affected A2 and A3 pulleys, carpal tunnel, and/or Loge de Guyon.
Main Results:
- 43 fingers across 6 patients were affected, with A2 and A3 pulleys most commonly involved.
- Trigger symptoms were present in 13 fingers, leading to surgical indications for 5 children.
- Surgical procedures resulted in significant pain relief and functional improvement in all treated cases.
Conclusions:
- Treating trigger fingers in mucopolysaccharidosis patients presents diagnostic and therapeutic challenges.
- Surgical intervention, including pulley release and carpal tunnel decompression, is effective for pain relief and functional recovery.
- A multidisciplinary approach is essential for optimal management of these rare cases.
Abstract:
Introduction: Mucopolysaccharidosis is a rare and congenital autosomal recessive lysosomal storage disorder of glycosaminoglycans. An enzyme defect leads to cell, tissue and organ dysfunction. Carpal tunnel syndrome and trigger finger are the results of mucopolysaccharid deposition. Material and methods: We are treating 6 patients with mucopolysaccharide associated trigger fingers in an interdisciplinary setting with the department of pediatric hematology and oncology at Hannover Medical School, where each patient is examined inter alia for symptoms of trigger finger annually. Besides an interview of the parents about abnormalities with regard to hand function, pain and/or neurologic symptoms the children are examined by palpation and by assessment of the active and passive range of finger motion. In the case of finger locking due to an impaired excursion of the flexor tendons in the A2 and A3 pulley region, we performed a trap-door incision technique for A2 pulley widening and a simple release of the A3 pulley. Results: In 6 patients 43 fingers were affected. The average age was 10 years. Pulley thickening was palpated in 19 fingers of to the left hand and 24 fingers of the right hand. In 7 fingers the A1 pulley was affected, in 28 fingers the A2 pulley and in 25 fingers the A3 pulley. The A4 and A5 pulley were not affected in any case. Trigger symptoms were seen in 13 fingers. Five of the 6 children were given an operation indication. In these cases we performed carpal tunnel release, release of Loge de Guyon, and trigger finger release, either in combination or alone. In all cases the procedure led to pain relief and functional improvement. Conclusion: The treatment of trigger fingers in children with mucopolysaccharidosis as a rare disease is challenging with regard to diagnostics and indication. The main treatment goal is pain relief and improvement of hand function.
