Emergency salpingectomy for ruptured ectopic pregnancy in patient with Melnick-Needles syndrome: a rare otopalatodigital syndrome

摘要

Melnick-Needles syndrome (M-NS) is a rare genetic disorder which primarily affects skeletal developments. M-NS may also affect the cardiorespiratory and renal systems. A 35 kg patient presented complaining of abdominal pain. Following a positive pregnancy test and ultrasound examination, she was diagnosed with a ruptured ectopic pregnancy. She had a significant background history of M-NS, obstructive sleep apnoea (with narrow gauge tracheostomy in situ), obstructive lung disease and scoliosis. She received fluid resuscitation, and the case was managed using an open salpingectomy and clot evacuation under combined spinal/epidural anaesthesia. Anticipated difficulty in securing a definitive airway was pivotal to choosing a regional anaesthetic technique. The operation was successful and the patient recovered well. This was a unique surgical and anaesthetic challenge due to abnormal facial, spinal and abdominal morphology compounded by the time-critical emergency nature of the case. Individually tailored perioperative management is frequently required for patients with rare syndromes.