Description

1. There is a posterior dislocation of the left hip with wide separation of the pubic symphysis and faecal loading of the rectum and sigmoid
colon.

2. There is neurofibromatosis material invading the left hip joint, and similar material arising from the sacrum and impinging on the rectum.

3. She has extensive pelvic neurofibroma disrupting the normal pelvic anatomy causing pubic diastesis, and extending in to the acetabulum. Hip dislocation is normally associated with high trauma injuries (typically road traffic collisions and sporting injuries); the presence of neurofibromatosis in the acetabulum, displacing the head of the femur explains why this dislocation occurred with only minimal trauma. There have been previous reports of hip dislocation in neurofibromatosis due to either anatomical abnormalities or neuropathic arthropathy. Her longstanding constipation was caused by neurofibroma partially obstructing the rectum.

4. Initial management should include early relocation of the hip and thromboprophylaxis. Debulking surgery may reduce the risk of repeat dislocations or others problems due to the pelvic mass, although removing the entire neurofibroma is rarely possible as they are extensive and infiltrative. This patient sadly developed acute on chronic bowel obstruction and died of complications of surgery to relieve this.