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22
II. ХИРУРГИЯ
HEPATOPULMONARY SYNDROME IN A CHILD WITH EXTRAHEPATIC PORTAL HYPERTENSION
UDC 616.149-008.341.1
Shirtaev B., Kospanov N., Zharasbayev A., Zharylkapov N., Zhunisov N., Kasenbayev R.
National Scientific Center of Surgery named after A.N. Syzganov, Almaty
ABOUT THE AUTHORS
Shirtayev Bakhytzhan Kerimbekovich - head of the department of thoracic surgery for children and adults, can. med., a high level certificate physician; Kospanov Nursultan Aidarkhanovich -head of the department of angiosurgery, can. med., a high level certificate physician
Abstract -
This publication describes the clinical observation of an 8 years old girl with extrahepatic portal Key words
. , . „, i «. , t- ■ ,4 ■ hepatopulmonarysyndrome,
hypertension complicated by hepatopulmonary syndrome. In the postoperative period a positive dynamics portai hypetenSion: nver
was noted. However, the final conclusion will be made in a long-term period. cirrhosis.
Гипертензиясымен баланьщ гепатопульмоналды синдромы
Шертаев Б., Коспанов Н., Жарасбаев А., Жарылкапов Н., Жушсов Н., Касенбаев Р.
А.Н. Сь^анов атындаш ¥лттык шлыми хирургиялык, орталыш, Алматы
Ацдатпа —--
Бул макалада гепатопульмоналды синдромымен аскынеан 8 жастагы кызбаланын клиникалык Туи'н свздер
. „_ „.. . „ ... гепатопульмональды синдром,
тексерун сипаттаиды. Операциядан кеишп кезецшде дурыс динамика баикалган. Дегенмен тупкшкл портащы гипертензия
корытынды кеишп кезде жасалады. бауыр циррозы.
ГепатопульмональныИ синдром у ребенка с внепеченочноИ портальной гипертензии
Ширтаев Б., Коспанов Н., Жарасбаев А., Жарылкапов Н., Жунисов Н., Касенбаев Р.
Национальный научный центр хирургии имени А.Н. Сызганова, Алматы
ОБ АВТОРАХ
Ширтаев Бахытжан Керимбекович
- заведующий отделением торакальной хирургии для детеИ и взрослых, к.м.н., врач высшеИ категории, хирург, shlrtaevb@gmall.com; Коспанов Нурсултан АИдарханович
- заведующий отделением ангиохирургии, к.м.н., врач высшеИ категории, хирург, Kospanov.nursultan@gmall.com
Аннотация
Эта статья описывает клиническое обследование девочки 8 лет с внепеченочноИ портальной ги-пертензиеИ, осложненноИ гепатопульмональным синдромом. В послеоперационном периоде была отмечена положительная динамика. Однако окончательный вывод будет делаться в дальнеИшем.
Ключевые слова
гепатопульмональныИ синдром, портальная гипертензия, цирроз печени.
The relationship between liver and lung was first noted by Fluckiger (1884), based on his observations of women with cirrhosis, cyanosis and fingers as "drumsticks". This relationship was formulated almost a century later, when Kennedy and Knudson (1997) described the «hepatopulmonary syndrome» which is characterized by hypoxemia caused by the expansion of pulmonary vessels [1].
The clinical picture of hepatopulmonary syndrome [GPS] is defined by the presence of chronic liver disease, usually with signs of portal hypertension, hyperdynamic type of blood circulation and hypoxemia.
The most frequent manifestation of hepatopulmonary syndrome - is dyspnea. Pathognomonic symptom is the appearance or worsening of dyspnea during the moving from the horizontal to the vertical position of the body - platipnoe [2]. Quite often in patients with hypoxemia during the inspection pulmonary cyanosis, changes in the distal phalanges of the type "drumsticks" and "watch glasses" can be observed. In addition, the skin of the trunk are observed typical in cirrhosis "spider veins", which are regarded as a manifestation of systemic vasodilation.
For diagnosis hepatopulmonary syndrome in patients with chronic liver disease need to confirm the presence of arterial hypoxemia and intrapulmonary vasodilatation . To assess the level of blood oxygenation it is necessary to measure blood oxygen saturation by pulse oximetry, and a study of arterial blood gas analysis. At GPS the "ortodeoksy" is observed - decrease of PaO 2 by more than 3-10 mm Hg during the transition from the horizontal to the vertical position of the body. "Gold standard" to identify intrapul-monary vasodilatation recognized two-dimensional transthoracic contrast echocardiography [3].. As the contrast solution is used forming the microbubbles larger than 15 microns. After intravenous injection microbubbles visualized by echocardiography in the right heart chambers. Because the diameter of the microbubbles is larger than the diameter of intrapul-monary capillaries, they do not reach the left heart chambers. In these parts, contrast is adjudged in the presence of intracardiac shunts, the expansion of intrapulmonary capillaries or in the presence of arteriovenous shunting. Visualization of contrast in the left heart chambers for 3 heart rate from the moment of intravenous injection indicates intracardiac shunting (defects of interventricular septum or interatrial septum). The appearance of microbubbles in the 4-6 th heartbeat indicates intrapulmonary shunting of blood within the hepatopulmonary syndrome. More sensitive transesophageal echocardiography contrast - the microbubbles become already visible in the upper pulmonary veins, but this method has significant limitations in the presence of varicose veins of the esophagus in patients with cirrhosis.
Depending on the changes occurring in the bloodstream, there are two types of GPS: Type 1: minimal changes - vasodilatation at precapillary level, where indicators of the blood oxygenation significantly improved after inhalation of 100% oxygen.. Type 2: severe vascular changes - the presence of arteriovenous shunts. In this case, oxygenation of the blood does not improve after inhalation of 100% oxygen [4].
Because the pathogenesis of GPS is not clear, pathogenetic treatment does not exist. Symptomatic therapy in most cases does not lead to the desired results. According to the literature, attempts to increase oxygenation and reduce shunting of blood using indomethacin, norfloxacin, oc-treotide, plasmapheresis is not efficacious[2,5,6]. In the literature there are reports that the application of Transjugular intrahepatic portosystem-ic shunt (TIPS) at a GPS reduces its clinical manifestations.
The use of TIPS in such cases improves the condition of patients before liver transplantation [7,8, 9]. In addition, several authors consider SBS as an independent indication for liver transplantation. In 85% of patients with GPS oxygenation of the blood indicators are normalized within the first year after surgery [4, 10]. It follows that the main treatment GPS is orthotopic liver transplantation [2, 11, 12, 13, 14]. Forecast in the absence of liver transplantation is not favorable. Survival of patients within one year after diagnosis of GPS without liver transplantation ranges from 16 to 38% depending on the degree of hypoxemia [15].
Hepatopulmonary syndrome in adults is widely discussed in the literature, however, the description of the syndrome in children is rare. In this publication we would like to share with our experience in the treatment of 8 years old girl with extrahepatic portal hypertension complicated with hepatopulmonary syndrome.
In July 2014 in our center a 8 years old patient has been hospitalized with complaints of episodes of vaginal and intestinal bleeding, abdominal pain, dyspnea on slight exertion and postural changes, cyanosis of the lips and fingers.
From anamnesis at the age of 41 days after the birth of first arose intestinal bleeding. Later she had been repeatedly surveyed and treated in children's hospitals of Astana and Almaty with diagnoses: "Meckel's diverticulum. Intestinal polyps. Cow's milk protein intolerance, gastrointestinal allergy, Ulcerative colitis, Extrahepatic portal hypertension". During the last 2 years signs of hypoxemia have joined. Episodes of intestinal and vaginal bleeding occurred weekly and were stopped by conservative measures. At admission general condition was severe, due to hypoxemia. Dyspnea occurs after a few steps, at change of position of the body - severe weakness.
36
ВЕСТНИК ХИРУРГИИ КАЗАХСТАНА № 1-2015
INDICATOR ARTERY VEIN NORM
pH 7,441 7,414
pC02 26,2 29,4 35,0-45,0 mmHg
p02 47,4 43,1 80,0-100,0 mmHg
ctHb 145 155 g/i
sO2 80.5 74.1 %
FO2Hb 79.1 72.9 %
FCOHb 1.0 0.9 %
FHHb 19.1 25.5 %
FMetHb 0.8 0.7 %
Indicators of blood gases
Skin clean, pale color, cyanosis of the lips and fingertips, fingers as drumsticks, nails in the form of watch glasses, asthenic constitution.
The complex survey produced doppler echo-ography and angiography of the liver. Blockade of the portal circulation at the trunk of the portal vein, below the confluence of the splenic vein, as a consequence of esophageal varices and splenomegaly was not. Severely the lower and upper mesenteric vein expanded. Shunting of portal venous system in the inferior vena cava occurs through the inferior mesenteric vein and veins of the rectum.
Given the frequency of vaginal and intestinal bleeding, in order to reduce portal hypertension, it was decided to perform mesocaval venous bypass.
The patient was operated, the mesocaval venous bypass was performed «side to side». Intraoperative: venous vessels of the mesentery small and large intestines expanded, tensed, and were tortuous. Inferior mesenteric vein dramatically expanded to 2530mm, tense. In the pelvis on the left lateral channel are many convoluted collaterals. In the projection
References
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2. Abrams G., Fallon M. The Hepatopulmonary syndrome. Clin. Liver Dis. - 1997. Vol. 1. p. 185-200.
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8. Chevallier P., Novelli L., Motamedi J.-P. et al. Hepato-
of the merger superior and inferior mesenteric veins at the lower edge of the head of the pancreas initial section of the portal vein extended to 40mm, tensed. In the mesentery of the colon and small intestine there were many hyperplastic lymph nodes (due lymphostasis). Superior mesenteric vein expanded to 15mm. The diameter of the inferior vena cava in the infrarenal section was 20mm, 15mm diastasis between the vessels. After mobilizing sections of the superior mesenteric vein and inferior vena cava created mesocaval anastomosis side to side (prolene 6/00) diameter of 11mm. The postoperative period was uneventful. Ph indicators in the early postoperative period were without significant changes. Histological examination of liver tissue - no signs of cirrhosis and fibrosis.
Currently 4 months passed after surgery. The general condition in the dynamics is better, no vaginal and intestinal bleeding, dyspnea after walking over a distance of 200-300m. In the future, the girl will be examined in more details to address the further treatment tactics.
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