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Caesarean delivery under general anaesthesia for a woman with undiagnosed tuberous sclerosis complex and lymphangioleiomyomatosis.

Tuberous sclerosis complex (TSC) is a rare autosomal dominant disorder characterised by seizures, cutaneous lesions and hamartomatous lesions in various organs (1). The association between TSC and lymphangioleiomyomatosis (LAM) is well known. LAM is a rare, progressive cystic disease of the lung that occurs sporadically or in association with TSC, affecting almost exclusively females of childbearing age (2,3). Pregnancy in these women is frequently associated with an increased incidence of pulmonary complications (3). We report the anaesthetic management and complications of caesarean delivery in a parturient with undiagnosed tuberous sclerosis complex and lymphangioleiomyomatosis.

A 33-year-old multiparous woman was admitted to our hospital for caesarean section because of posterior placenta praevia totalis at 39 weeks' gestation. Chest radiograph showed minimal cardiomegaly and subtle miliary or interstitial processes in both lungs. Her haemoglobin concentration was 8.0 mg/dl but other laboratory findings were within normal limits. Oximetry (Sp[O.sub.2]) observed after anaesthetic induction was 70%. Nitrous oxide was immediately stopped and 100% oxygen used, but Sp[O.sub.2] remained at 60 to 80%. About seven minutes into surgery, the foetus was delivered with Apgar scores of 7 at one minute and 9 at five minutes. After reversal of muscle paralysis, the Sp[O.sub.2] increased to 90% with an inspiratory oxygen fraction (Fi[O.sub.2]) of 1.0. After emergence from anaesthesia, the patient appeared awake and oriented, but she was transferred to the recovery room intubated because her breathing was rapid and shallow. When the tracheal tube was suctioned in the recovery room, the tube lumen filled with bright red blood. Emergent chest X-ray was taken and right internal jugular vein catheterisation was performed to allow central venous pressure measurement. Her central venous pressure was 0 cm[H.sub.2]O and her chest X-ray showed pulmonary congestion. Her respiration rate decreased to normal rates after frequent suctioning of the tracheal tube and the Sp[O.sub.2] was maintained above 95% at an Fi[O.sub.2] of 0.35. She was extubated and transferred to the intensive care unit. A chest CT showed lymphangioleiomyomatosis in both lungs and an abdominal CT on the second postoperative revealed bilateral renal angiomyolipomas. At the age of 20, she had experienced several episodes of seizures. On postoperative physical examination there were ungual fibroma and hypopigmented maculae (formerly known as ash-leaf spots) on the skin of her toes. After histological examination, the patient was diagnosed with tuberous sclerosis complex and lymphangioleiomyomatosis. Her postoperative course was uneventful. She was discharged on the eighth postoperative day without complication.

TSC is a multisystem, autosomal dominant disorder affecting children and adults, resulting from mutations in one of two genes, TSC1 (encording hamartin) or TSC2 (encording tuberin) (1,4). The diagnostic criteria for TSC consist of a set of major and minor diagnostic features (1). The incidence is one in five to 10,000 live births, with one-third of cases familial and two-thirds sporadic from new mutations (5). Sporadic LAM is rare, with a prevalence of approximately one in 1,000,000 people in the population. It is much more common in patients with the genetic disease tuberous sclerosis, where signs of LAM can be identified in up to 40% of adult females (2). LAM features hamartomatous proliferation of smooth muscle in the lungs, mediastinum and abdomen (2,6). In the lungs involvement of airways, blood vessels and lymphatics extends to the alveolar interstitium causing cystic change and into pulmonary veins causing haemorrhage. Several authors (3,6) have demonstrated an association between pregnancy and an increased risk of complications. Johnson and Tattersfield found that in seven LAM pregnancies there were five cases of either single or recurrent pneumothorax or chylous pleural effusion, the complication rate during pregnancy being 11 times higher (6). Among 50 LAM-affected females in the UK, of whom 28 had been pregnant, only three (14%) were known to have the condition before becoming pregnant and four (15%) were diagnosed during pregnancy. The mode of delivery is dictated by obstetric and medical considerations (3). McLoughlin et al (3) suggested that epidural analgesia during labour might reduce hyperventilation and excessive changes in intrathoracic pressure during contractions. Our patient had previously had a normal spontaneous vaginal delivery and required elective caesarean section due to placenta praevia. She had no respiratory difficulty or abnormal laboratory findings. General anaesthesia was planned and it was not until the first postoperative day that her past full history was determined. This information might have prompted biopsy for ungual fibroma and hypopigmented maculae (formerly known as ash-leaf like spots) on the skin of her toes before the operation and if the diagnosis had been known we could have planned combined spinal-epidural anaesthesia, as recommended by McLoughlin et al (3).

In conclusion, this patient with pulmonary LAM developed the life-threatening complication of pulmonary haemorrhage after general anaesthesia, but fortunately recovered fully. More thorough history taking and physical examination might have avoided these complications.




Seoul, Korea


(1.) Crino PB, Nathanson KL, Henske EP. The tuberous sclerosis 1. complex. N Engl J Med 2006; 355:1345-1356.

(2.) Johnson SR. Lymphangioleiomyomatosis. Eur Respir J 2006; 2. 27:1056-1065.

(3.) McLoughlin L, Thomas G, Hasan K. Pregnancy and 3. lymphangioleiomyomatosis: anaesthetic management. Int J Obstet Anesth 2003; 12:40-44.

(4.) Roach ES, Delgado MR. Tuberous sclerosis. Dermatol Clin 4. 1995; 13:151-161.

(5.) Rose VM, Au KS, Pollom G, et al. Germ-line mosaicism 5. in tuberous sclerosis: how common? Am J Hum Genet 1999;64:986-992.

(6.) Johnson SR, Tattersfield AE. Clinical experience of 6. lymphangioleiomyomatosis in the UK. Thorax 2000;55:10521057.
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Article Details
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Title Annotation:Correspondence
Author:Cho, S.Y.; Kim, K.H.; Jeon, W.J.
Publication:Anaesthesia and Intensive Care
Article Type:Clinical report
Geographic Code:4EUUK
Date:Jan 1, 2009
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