Neurological deterioration years after closure of myelomeningocoele – `the second lesion'

Abstract

Case presentationMP is a 36-year-old man who had an upper lumbar myelomeningocoele closed on the ®rst day of life. He had a degree of hydrocephalus but never required any treatment for this. As a child there was a marked disparity in lower limb function ± with the right leg near normal but with the left leg spastic, very weak and shortened. He had undergone left adductor tenotomy and psoas transplant aged 10 years and had a left femoral fracture at 16 years but remained well motivated and was mobile with elbow crutches and calipers. He had a neurogenic bowel, a neuropathic bladder with a suprapubic catheter, and preserved sexual function. Mobility and function otherwise were generally static over the next 20 or so years.At the age of 35 years he presented to his GP with a year's history of worsening back pain, especially severe at night, disturbing sleep. This was associated with loss of sensation of the previously normal right lower limb. He was referred to a Spinal Orthopaedic Clinic and the referral was prioritised as`routine' with an expected waiting time of 53 weeks. Twelve months later his pain and sensory loss had worsened and he was having di culty weightbearing on the right leg: his outpatient appointment was expedited. In the interim he had a fall fracturing his left tibia. By chance, he was seen for this at the Fracture Clinic by an Orthopaedic Surgeon with a Paediatric interest; he reported an abrupt marked deterioration in power in the right leg some 3 weeks earlier such that he could no longer walk and was admitted for investigation and a neurosurgical opinion. By now he had no power or sensation in the left leg, and in the right leg had Grade 2 hip¯exion, Grade 3 knee extension, no power at the ankle and a suspended sensory loss from L3 to S1. MR of the neuraxis demonstrated a moderate supratentorial hydrocephalus and a Chiari II malformation with the tonsils down to C2 though with no compression at the foramen magnum. In the spine (Figures 1 and 2) there was an extensive intradural mass lesion lying posterior to the neural tissue at the level of the myelomeningocoele repair and a moderate size thoracolumbar syrinx above this. The di erential diagnosis of this lesion was felt to be either a dermoid cyst or a tumour. Comments by participantsWhat is your di erential diagnosis? M Spencer (General Practitioner) points out that he sees very few cases of spina bi®da at any age and ®nds this di cult, but highlights possibilities such as a problem arising as a direct late consequence of his myelomeningocoele or secondary mechanical or musculoskeletal consequences due to abnormal posture and use of the lower body. As such he would consider disc prolapse as well as`non-back' causes of back pain on the di erential including urinary tract infection and constipation.DL Douglas (Orthopaedic Surgeon) also pays heed to incidental pathologies adding meningioma or AV malformation to the list. He mentions that patients with spina bi®da and asymmetric neurology frequently have back deformities such as scoliosis. He w...

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Fetal Surgery for Myelomeningocele: Panacea or Peril?

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