Article Text
Summary
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.
- anaesthesia
- ear, nose and throat/otolaryngology
- resuscitation
- obstetrics and gynaecology
- genetics
Statistics from Altmetric.com
Footnotes
Contributors This decision to plan, write and submit this case report was jointly made by MZJ, CFM, DK and IB. MZJ and CFM collected clinical details, patient consent and photography and drafted the manuscript. There was critical intellectual input and multiple revisions by IB and DK. MZJ, CFM, IB and DK all share and approve intellectual content and final approval of the version published. MZJ, CFM, DK and IB are in agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.