Anaesthesia in a parturient with Noonan's syndrome

• Published in: Canadian journal of anesthesia Vol. 45; no. 4; pp. 332 - 336
• Main Authors: GRANGE, C. S, HEID, R, LUCAS, S. B, ROSS, P. L. E, DOUGLAS, M. J
• Format: Journal Article
• Language: English
• Published: Toronto, ON Canadian Anesthesiologists' Society 01.04.1998; Springer Nature B.V
• Subjects: Adult; Analgesics; Anesthesia; Anesthesia depending on patient's condition; Anesthesia, Obstetrical; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Biological and medical sciences; Childbirth & labor; Female; Humans; Intubation; Medical sciences; Noonan Syndrome - blood; Noonan Syndrome - physiopathology; Nursing; Pregnancy; Pregnancy Complications - blood; Pregnancy Complications - physiopathology; Human; Nervous system diseases; Diseases of the osteoarticular system; Fentanyl; Cardiovascular disease; General anesthesia; Opiates; Labour; Self administration; Narcotic analgesic; Puerperium; Hemorrhage; Obstetrics; Genetic disease; Case study; Analgesia; Chemotherapy; Pain; Treatment; Noonan syndrome; Female; Delivery; Osteochondrodysplasia
• ISSN: 0832-610X; 1496-8975
• DOI: 10.1007/BF03012024

To report the management of labour analgesia and subsequent anaesthesia for postpartum bleeding in a 19-yr-old parturient with Noonan's syndrome. The patient presented in active labour at 36-wk gestation. She was known to have Noonan's syndrome and had been assessed regularly throughout pregnancy. Features of the syndrome exhibited by the patient included typical facies, chest skeletal abnormalities, pulmonary valve dysplasia, mental retardation and lymphoedema. In addition, she had Factor XI deficiency (0.46 mg.L-1) and thrombocytopenia (92 x 10(9).L-1), previously unreported in a parturient with this syndrome. Although epidural analgesia may have been considered the labour analgesic technique of choice, the risk of epidural haematoma caused by her bleeding diathesis made this unacceptable. This risk was balanced against the possibility of a potentially difficult intubation due to facial abnormalities, should emergency operative delivery become necessary. Labour analgesia was provided with intravenous patient controlled opioid analgesia (fentanyl 25 micrograms bolus, five minute lockout) despite her mental retardation. Dilatation and curettage required general anaesthesia after intubation with awake direct laryngoscopy using cautious sedation. Noonan's syndrome is characterised by multi-system involvement, requiring thorough preoperative assessment of cardiovascular, skeletal, haematological and central nervous systems. Clotting and platelet defects considerably restrict the possible analgesic and anaesthetic options for labouring patients with this syndrome.