Case report of Larsen syndrome presenting as acute onset quadriparesis

Abstract

Larsen syndrome is one of the rare inherited disorders which affects the development of bones in the body. It is associated with multiple anomalies in different systems. Larsen syndrome involves skeletal system causing features like multiple joint dislocations, hypermobility of the joints, club foot, developmental dysplasia of hip usually teratologic type and supernumerary carpal and tarsal bones. It also involves spinal abnormalities like cervical kyphosis, thoracic scoliosis which can lead to compression on the cord and leading to myelopathy kind of picture which might need surgical treatment. Typical facial features include mid face hypoplasia, nasal bridge prominence, wide set eyes. There are usually of short stature and normal intelligence. In this case report we are presenting a case of Larsen syndrome who presented to us with quadriperesis secondary to subluxation at C3-4 and C4-5 with cervical kyphosis. The patient was a 15 year old boy presented with difficulty in walking for a period of 10 years, weakness of both the upper and lower limbs for a  period of 3 months after a trivial fall. He had typical facial features like prominent forehead, wide set eyes. He underwent three stage cervical decompression and          instrumented fusion. At 9 months follow up he has improved by 2 grades in his upper and lower limbs but he needs support for daily activities. X ray and CT scan shows fusion between C3-C6.