Departments of 1Anesthesiology and Critical Care, 2Gastroenterology and Hepatology, and 3Transplantation Surgery, Mayo Clinic, Rochester, MN 55905, USA*Corresponding author
Accepted for publication: November 2, 2000
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Abstract |
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Br J Anaesth 2000; 86: 4314
Keywords: liver, transplantation; liver, haemangiomatosis; lung, diseases
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Introduction |
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Report of patient |
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Over the years, as his liver tumour grew, the patients condition gradually worsened. Two months before transplantation, he became weak and lethargic, with dyspnoea on minimal exertion, persistent abdominal discomfort, and peripheral oedema. His abdomen was massively distended, and he had to remain upright at all times because of shortness of breath. Examination showed jugular venous distention and markedly diminished lung air-entry.
The chest radiograph (Fig. 1A) showed bilateral raised hemidiaphragms with small lung fields. Computerized axial tomography (CT) (Fig. 2A) of the thorax showed a compressed pulmonary vascular bed, especially in the lung bases, and abdominal CT (Fig. 2B) showed massive hepatomegaly. Transthoracic echocardiography was normal except for a mildly dilated right atrium, moderately dilated right ventricle, and an elevated pulmonary artery systolic pressure estimated at 56 mm Hg. There was no evidence of right to left shunt.
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The surgical dissection was complicated by adhesions from the previous cholecystectomy. The liver was densely adherent to the abdominal wall in the areas near the previous incisions. Near the site of the previous thoracoabdominal incision, the distal portions of the ribs were embedded within the hepatic parenchyma and haemangioma. The abdominal wall was excised bilaterally in a wedge-shaped fashion between the umbilical area and the flank. While removing the liver from its attachment to the abdominal wall, significant bleeding was encountered, and thus the portal vein was ligated, starting the anhepatic phase. (The hepatic artery had been ligated earlier in an effort to decrease potential arterial bleeding.) The liver was removed, leading to improvement in ventilation (tidal volume 300 ml and rate 16 min1 with peak inspiratory pressures of 30 cm H2O, to tidal volumes of 500 ml without an increase in pressures). Pulmonary artery pressures decreased to 29/15 mm Hg. After removal of the liver, there was redundancy of the previously stretched diaphragm. The anhepatic phase lasted 142 min and reperfusion was tolerated without significant incident. Total transfusion requirements during surgery were to 40 units banked red blood cells, 79 units Cell Saver (Hemonetics, Braintree, MA, USA), 50 units of fresh frozen plasma, 30 units of platelets, and 60 units of cryoprecipitate. A rapid transfusion device (Hemonetics) was used to give fluids and blood products. The resected liver weighed 19 kg and measured 63x45x51 cm. Large parts of redundant abdominal wall skin, subcutaneous tissue and muscle were resected before abdominal closure. The bulk of the tumour was confirmed histologically to be cavernous haemangioma.
The patient was transferred to the intensive care unit (ICU). Continued intra-abdominal bleeding caused haemodynamic instability. Haemostasis was secured after repeat laparotomy, with subsequent haemodynamic stability.
On the second post-operative day the patient was alert and co-operative and a trial of weaning from intermittent mandatory ventilation was attempted. While on continuous positive airway pressure (CPAP) of 5 cm H2O and pressure support of 10 cm H2O, his CO2 increased to greater than 13 kPa. Bedside pulmonary mechanics were obtained and these showed maximal inspiratory and expiratory pressures of 18 and +2 cm H2O, respectively. Over 8 days in the ICU, the patients maximal inspiratory and expiratory pressures improved to 20 and +40 cm H2O, respectively. His spontaneous vital capacity improved from 500 to 1450 ml during the same period of time. The patient was weaned to spontaneous ventilation with 5 cm H2O CPAP and pressure support of 10 cm H2O. He mobilized in the ICU while attached to a portable ventilator and improved sufficiently to allow extubation of his trachea 8 days post-operatively. Post-extubation blood gases (FIO2 0.21) were PaO2 9.2 kPa, PaCO2 6.6 kPa, pH 7.39 and serum bicarbonate concentration 30 mEq litre1. He left hospital 2 weeks after transplantation.
Pulmonary function tests 1 month after surgery, showed marked improvement in his restrictive lung pattern (Table 1) with FEV1 increased to 1.93 litre (45% predicted). Subsequently, lung function and overall quality of life have continued to improve. One year later, his chest x-ray (Fig. 1) showed improvement in lung volumes and a rounded contour to the diaphragm. Arterial blood gases (room air) showed a PO2 of 12.1 kPa, PCO2 6.1 kPa, HCO3 28 kPa and pH 7.4. His transplanted liver continues to function satisfactorily.
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Discussion |
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Giant haemangiomas may be managed expectantly especially if surgical resection is thought to be technically difficult or if the patient has minimal or no symptoms.12 16 When patients become symptomatic the options for therapy include hepatic artery embolization17 and surgical resection. There have also been reports of the use of corticosteroids and radiotherapy in the management of the lesions.18 Orthotopic liver transplantation (OLT) has been used for giant liver haemangiomas, the principal indications being pain, functional debility, and risk of rupture. There are less than 15 such patients reported in the literature. In our patient, the principal reason for transplantation was the severe restrictive lung disease.
The anaesthetic management of OLT is challenging and in this patient the difficulties were compounded by several unusual features. First, despite the fact that his airway looked favourable, there was concern that, haemodynamically, because of aortocaval compression, the patient would not tolerate a rapid sequence induction. In addition, the patient could not lie flat because of dyspnoea. Awake fibreoptic endotracheal intubation was performed, and inhalation induction of anaesthesia using sevoflurane followed. In spite of a slow change to the supine position the patient did become hypotensive, but this was responsive to fluid and vasopressor administration. Left lateral tilt could also have been used to decrease aorto-caval compression, in a fashion analogous to the use of left uterine displacement in pregnancy.
Second, his pulmonary hypertension (PA systolic pressure 56 mm Hg on pre-operative testing) was a concern. It was felt that, in the current patient, this was almost entirely because of mechanical factors caused by his massive liver lesion and would be reversible post-transplant. Pulmonary artery pressures did decrease (mean pulmonary artery pressure 3320 mm Hg) after the patients liver was removed.
Finally, for a number of reasons, the patient was likely to breathe poorly after surgery. His already elevated bicarbonate concentration (49 mEq litre1, ref. 2228) and the massive blood transfusion requirements containing citrate, caused severe metabolic alkalosis and central hypoventilation. The stretching of the diaphragm by the large liver conferred some mechanical advantage pre-operatively but once the liver was removed the diaphragm became lax.19 In addition, his malnourished state (a result of pre-operative early satiety from gastric compression and consequent decreased caloric intake) and compressed lungs from tumour mass impaired respiratory function. Each of these factors could prolong mechanical ventilation, but extubation of the trachea was possible 1 week after the transplantation.
In summary, we present a patient with massive hepatic haemangiomatosis, which caused a restrictive-type of respiratory failure. Transplantation was successful in spite of concerns of elevated airway pressures, pulmonary hypertension, severe metabolic alkalosis, inherent surgical difficulties and possible aorto-caval compression. Transplantation improved the patients respiratory status and overall quality of life.
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References |
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2 Colonna JO, Olthoff KM, Seu P, Busuttil RW. Liver resection for primary neoplasms. Adv Surg 1992; 25: 30929[Medline]
3 Kasabach HH, Merritt KK. Capillary haemangioma with extensive purpura. Report of a case. Am J Dis Child 1940; 59: 106370
4 Chui AK, Vass J, McCaughan GW, Sheil AG. Giant cavernous haemangioma: a rare indication for liver transplantation. Aust NZ J Surg 1996; 66: 1224[ISI][Medline]
5 Crespo Uriguen M, Martinez Torres D, Miyar Gonzalez A. Diffuse giant cavernous haemangioma of the liver: apropos of two cases. Rev Esp Enferm Aparato Dig 1988; 73: 28992
6 Ducerf C, Rode A, Adham M, et al. Hepatic outflow study after piggyback liver transplantation. Surgery 1996; 120: 484487[ISI][Medline]
7 Klompmaker IJ, Slooff MJ, van der Meer J, de Jong GM, de Bruijn KM, Bams JL. Orthotopic liver transplantation in a patient with a giant cavernous haemangioma of the liver and Kasabach-Merritt syndrome. Transplantation 1989; 48: 14951[ISI][Medline]
8 Mabrut JY, de la Roche E, Adham M, Ducerf C, Baulieux J. Peritoneovenous diversion using the LeVeen shunt in the treatment of refractory ascites after liver transplantation. Ann Chir 1998; 52: 6127[ISI][Medline]
9 Mora A, Cortes C, Roige J, Noguer M, Camps MA, Margarit C. Orthotopic liver transplant for giant cavernous haemangioma and Kasabach-Merritt syndrome. Rev Esp Anestesiol Reanim 1995; 42: 714[Medline]
10 Longeville JH, de la Hall P, Dolan P, et al. treatment of a giant haemangioma of the liver with Kasabach-Merritt syndrome by orthotopic liver transplant: a case report. HPB Surg 1997; 10: 15962
11 Brouwers MA, Peeters PM, de Jong KP, et al. Surgical treatment of giant haemangioma of the liver. Br J Surg 1997; 84: 3146[ISI][Medline]
12 Farges O, Daradkeh S, Bismuth H. Cavernous haemangiomas of the liver: are there any indications for resection? World J Surg 1995; 19: 1924[ISI][Medline]
13 Russo MW, Johnson MW, Fair JH, Brown RS. Orthotopic liver transplantation for giant hepatic haemangioma. Am J Gastrol 1997; 92: 19401[ISI][Medline]
14 Tepetes K, Selby R, Webb M, Madariaga JR, Iwatsuki S, Starzl TE. Orthotopic liver transplantation for benign hepatic neoplasms. Arch Surg 1995; 130: 1536[Abstract]
15 Woltering MC, Robben S, Egeler RM. Hepatic hemangioendothelioma of infancy: treatment with interferon alpha. J Pedatric Gastroenterol Nutr 1997; 24: 34851
16 Weimann A, Ringe B, Klempnauer J, et al. Benign liver tumours: differential diagnosis and indications for surgery. World J Surg 1997; 21: 98390[ISI][Medline]
17 Yamamoto T, Kawarada Y, Yano T, et al. Spontaneous rupture of haemangioma of the liver: treatment with transcatheter hepatic artery embolization. Am J Gastroenterol 1991; 86: 164549[ISI][Medline]
18 Reading N, Forbes A, Nunnerley H, Williams R. Hepatic haemangioma: a critical review of diagnosis and management. Q J Med 1988; 253: 43145
19 Duchenne GB. Physiology of Motion. Philadelphia: JB Lippincott, 1949; 45260