Regional Anesthesia for Cesarean Section in a Parturient with Noonan's Syndrome

Dadabhoy, Zerin P.; Winnie, Alon P.

doi:10.1097/00000542-198804000-00031

Cited by 18 publications

(10 citation statements)

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“…Regional anaesthesia may be technically difficult in Noonan's syndrome because of kyphoscoliosis, lumbar lordosis, and a relatively narrow spinal canal, which contains a normal sized spinal cord [4]. In our case, the age of the patient, the presence of a hypertrophic obstructive cardiomyopathy, and the nature of the surgery precluded the use of regional anaesthesia [9, lo], although it was noted that lumbar puncture for myelography was accomplished with ease.…”

Section: Discussionmentioning

confidence: 90%

“…In Noonan's syndrome 30-50% of the patients have pulmonary stenosis [4]. Right ventricular failure could be precipitated by an increase in pulmonary vascular resistance or excessive administration of intravenous fluids.…”

Section: Discussionmentioning

confidence: 99%

“…It is a rare clinical entity and represents the phenotype of Turner's syndrome with normal chromosome studies [ 3 ] . These patients have facial, cardiovascular and skeletal abnormalities which may pose problems to the anaesthetist during surgery [4]. The most common congenital cardiac lesion is pulmonary stenosis, either alone or in combination with a septal defect, which is usually atrial.…”

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confidence: 99%

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Anaesthesia in a patient with Noonan's syndrome and cardiomyopathy

Campbell

Bousfield

1992

Anaesthesia

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SummaryA 5-year-old girl with Noonan's syndrome and a hypertrophic cardiomyopathy presented for myelography and subsequently, laminectomy. The anaesthetic management of the patient is described and the potential problems of the facial, cardiovascular and skeletal abnormalities associated with the condition are discussed. Key words Anaesthesia; paediatric.Complications; Noonan's syndrome, cardiomyopathy.Noonan's syndrome was first described by Noonan and Ehmke in 1963 [I, 21. It is a rare clinical entity and represents the phenotype of Turner's syndrome with normal chromosome studies [ 3 ] . These patients have facial, cardiovascular and skeletal abnormalities which may pose problems to the anaesthetist during surgery [4]. The most common congenital cardiac lesion is pulmonary stenosis, either alone or in combination with a septal defect, which is usually atrial. In addition, it is now being recognised that many of these children also have a hypertrophic cardiomyopathy [5]. There have been no reports of general anaesthesia in this combination of Noonan's syndrome and cardiomyopathy. Therefore we describe the management of such a patient who required anaesthesia for two separate procedures. Case historyA 5-year-old girl presented for investigation of a spastic diplegia. Family history was unavailable since she had been adopted at the age of 3 months, but she had appeared normal at birth. At 7 months she underwent pulmonary valvotomy for a dysplastic pulmonary valve. Heart muscle biopsy at that time showed evidence of a hypertrophic cardiomyopathy. Her growth was slow, remaining below the third centile, and at the age of 18 months a diagnosis of Noonan's syndrome was made.At 3 years, following growth hormone injections, she was noted to walk on tip-toes. Over the next 2 years she developed increasing spasticity in both legs, and a t 5 years she was admitted for CT scan and myelography under general anaesthesia.The patient was an alert, friendly child who weighed I5 kg and whose height was 96 cm. She had features typical of Noonan's syndrome, namely hypertelorism, downward slanting eyes, low set ears, webbing of the neck and a low hairline. Physical examination of the chest showed a median sternotomy scar, a grade II/W systolic murmur at the left sternal edge conducted to the aortic area, blood pressure of 90/40mmHg and a heart rate of 80 beatsmin-'. Current medication consisted of propranolol 20 mg three times daily.Echocardiography was reported to show evidence of hypertrophic cardiomyopathy and dynamic left ventricular outflow tract obstruction. The ventricular septal thickness was 2.5 cm while the posterior left ventricular wall thickness was 1.24 cm. Ventricular contraction, however, was within normal limits. Chest X ray showed left ventricular enlargement with increased pulmonary vascular markings. The electrocardiogram showed Q waves in leads 11, 111, aVF, V5 and V6, with left axis deviation and left ventricular hypertrophy.She was premedicated 90 min pre-operatively with trimeprazine syrup 30mg and droperid...

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Section: Discussionmentioning

confidence: 90%

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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Anaesthesia in a patient with Noonan's syndrome and cardiomyopathy

Campbell

Bousfield

1992

Anaesthesia

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“…If patient has severe cardiac disease, anesthesiologist should be aware of their impaired cardiac function. Regional anesthesia may be technically difficult with the kyphoscoliosis and lumbar lodosis [4].The epidural and subarachnoid spaces are difficult to detect because of the abnormal curvature of the spine. Six cases of anesthetic management in a parturient with Noonan syndrome under both general and regional anesthesia have been reported [4][5][6][7][8][9].…”

Section: Introductionmentioning

confidence: 99%

“…Regional anesthesia may be technically difficult with the kyphoscoliosis and lumbar lodosis [4].The epidural and subarachnoid spaces are difficult to detect because of the abnormal curvature of the spine. Six cases of anesthetic management in a parturient with Noonan syndrome under both general and regional anesthesia have been reported [4][5][6][7][8][9]. Although previous reports have suggested that anesthesiologists should evaluate a patient early during the prenatal period we had no opportunity to assess the patient until emergency Cesarean section was selected, and we had to deal with issues associated with anesthesia as they arose [4][5][6][7][8].…”

Section: Introductionmentioning

confidence: 99%

Anesthesia for Emergency Cesarean Section in a Parturient with Noonan Syndrome

Hamada¹

2013

J Anesth Clin Res

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Patients with Noonan syndrome are characterized by many anesthetic problems, primarily related to potential difficult airway and technical issues with regional anesthesia. We describe the anesthetic management of a difficult case of a parturient with Noonan syndrome requiring emergency Cesarean section under spinal anesthesia. We had to evaluate the patient within a limited amount of time because of fetal distress. Although spinal injection was easily accomplished, it resulted in inadequate sensory block requiring supplemental analgesics and sedatives. B AFigure 1: Standing lumbar spine anteroposterior (A) and lateral (B) X-ray images in the nonpregnant state showing kyphoscoliosis.

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