1131 - Complex Custom Stroller for Severe Möbius Syndrome and Swallowing Deficits

Case Diagnosis: Severe Möbius Syndrome

Case Description: 3 y/o non-ambulatory, non-verbal female patient with dysphagia, gastric tube was referred to wheelchair clinic for pediatric stroller evaluation. Patient’s mother reported difficulty keeping the patient's head erect and excessive salivary secretions causing choking. Exam findings included hypotonia, bilateral absence of digits, bilateral talipes equinovarus (TEV), bilateral arm contractures, generalized weakness, significant neck weakness resulting in coughing, poor secretion management. Exam also remarkable for anteverted nostrils, micrognathia, expressionless facies, decreased lateral extraocular movement. Patient was previously diagnosed with Möbius syndrome on the basis of abnormal facies and absent extraocular abduction. Given the exam findings, this could be severe Möbius syndrome or a Möbius-mimic syndrome.

Discussions: Wolf and Koutsandreou (2001) reported all patients with Möbius syndrome exhibited motor delays and 60% had TEV; Verzijil et al (2003) reported 76% had oropharyngeal insufficiency. Our patient exhibits all these findings. The wheelchair seating/mobility evaluation team (PM&R, OT, assistive technology/seating/mobility specialist) recommended pediatric adaptive stroller with manual tilt-in-space/reclining system, including contoured headrest with swivel headband for occipital, suboccipital, mandibular support/active head movements, anterior trunk harness for torso support, elevating leg rests for LE support. The seating/mobility specialist recommended anterior swivel headband for midline head support/control and neck elongation to improve swallowing, reduce aspiration from excessive saliva, and control anterior head migration without restricting lateral rotation. The adaptive seating system accommodates the torso and bilateral LEs allowing patient’s mother to closely observe while pushing the stroller. Three weeks after receiving the stroller, the patient’s mother reported significant improvement in ease of care.

Conclusions: Appropriate adaptive seating and mobility can be highly useful in the care of patients with Möbius and related syndromes. This case demonstrates the benefit of a multidisciplinary team in patients’ complex seating and physical supportive needs.