Paraparesis and congenital severe hyperkyphosis in Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: A rare deformity management during the Sars-Cov-2 pandemic

Introduction: Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) syndrome can be classified into two types: type I (isolated) without extragenital abnormalities; type II (associated) with the presence of extragenital dimorphisms. Skeletal abnormalities are the second most frequent extragenital manifestations.

Research question: Association between MRKH and congenital scoliosis has been described; on the contrary, hyperkyphosis is very rare and sparsely described in the medical literature. Here we report our experience in the management of thoracolumbar hyperkyphosis in 16-year-old patient diagnosed with MRKH syndrome with an acute neurological impairment due to T11-T12 disc herniation.

Material and methods: Clinical and radiological images of the case were retrieved from the medical notes, operative records and imaging system.

Results: Posterior surgical correction was proposed to treat the severe spinal deformity; however, surgery was delayed because of SARS-CoV2 pandemic outbreak. During the pandemic, the patient had a major clinical and radiological deterioration with development of paraparesis. Complete clinical resolution of the paraparesis and restoration of balance was achieved with a two stage surgical approach, with a first anterior stage followed by a delayed posterior approach aimed at deformity correction.

Discussion: Congenital kyphosis are rare deformities that can progress rapidly leading to severe neurological deficits and worsening of the deformity. When patient has neurological deficit the surgical strategy to address the neurological problem first and plan the more complex and demanding corrective surgery remains a valid strategy that must be consider.

Conclusion: This is the first reported case of hyperkyphosis in Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) syndrome surgically treated.