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‘Butterfly vertebrae and neuroaxial blockade’: A rare case of Alagille syndrome and caesarean section.
Abstract Number: F5C-10
Abstract Type: Case Report/Case Series
Alagille syndrome is a rare autosomal dominant genetic disorder that affects the liver,heart,kidney and other systems of the body .It presents in infancy and can pose a number of anaesthetic challenges.It is associated with liver disease,hepatomegaly,biliary atresia and in severe cases liver transplantation may be necessary.Congenital cardiac disease can arise including pulmonary artery stenosis,tetralogy of Fallot and ventricular septal defects.One unique feature of Alagille syndrome includes an unusual ‘butterfly’ shape of one or more bones of the spinal column.
This is the first case describing safe neuroaxial anaesthesia care for caesarean section in a patient with Alagille syndrome.We present the case of a 32year old multiparous woman that presented to our high risk multi disciplinary clinic at 31weeks gestation.She had a diagnosis of Alagille syndrome.Her diagnosis was only established in 2005 after the birth of her first child that was diagnosed with Alagille syndrome.
The patient had a number of features related to her syndrome.She had a previous atrial septal defect repaired in 2007 and has pulmonary artery stenosis but was asymptomatic.She was attending an adult congenital cardiac clinic annually.She had characteristic facies; posterior embryotoxon and ‘butterfly’ shaped vertebrae associated with her diagnosis.
During the patient’s visit to our clinic the patient expressed a wish to have a spontaneous vaginal delivery and to avoid epidural analgesia.The option of remifentanil as alternative analgesia was discussed.Discussion surrounding the event of an emergency caesarean section was had.After examining the patient’s back, palpating her lower lumbar region in the midline using bony landmarks, it was felt that an attempt of spinal anaesthesia would be reasonable.We confirmed this by using ultrasound.Of note,the patient’s most recent fetal anomaly scans also revealed features strongly suggestive of Alagille syndrome.
At 37weeks gestation,the patient experienced labour pains,and had a number of unprovoked fetal decelerations.A category two caesarean section was called and the patient underwent uneventful spinal anaesthesia.A standard spinal anaesthetic was administered and successful on first attempt.Surgery was uncomplicated.
This was the first case report to demonstrate the safe use of neuroaxial blockade in these patients for caesarean section.Only one previous case report of a patient with Alagille syndrome undergoing a caesarean section under general anaesthesia has been described .This case highlights the importance of a thorough pre operative assessment and that the use of ultrasound and prior spinal imaging may be invaluable in the perioperative planning of these patients.
1.US National Library of Medicine:https://ghr.nlm.nih.gov/condition/alagille-syndrome
2.F.C.Rahmoune,M.Bruyère,M.Tecsy,D.Benhamou.Alagille syndrome&pregnancy:anaesthetic management for cesarean section:IJOA:Oct2011Vol20Issue4,355–358