Научная статья на тему 'Diagnosis and surgical treatment of congenital cystic adenomatoid malformations of the lung'

Diagnosis and surgical treatment of congenital cystic adenomatoid malformations of the lung Текст научной статьи по специальности «Клиническая медицина»

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Ключевые слова
кистозная гипоплазия легких / хирургическое лечение / сongenital cystic adenomatoid malformations of the lung / surgical treatment

Аннотация научной статьи по клинической медицине, автор научной работы — Eshmuratov T., Shirtaev B., Sundetov M., Batyrhanov М., Zharylkapov N.

Диагностика и хирургическое лечение кистозной гипоплазии легких Ешмуратов Т.Ш., Ширтаев Б.К., Сундетов М.М., Батырханов М.М., Жарылкапов Н.С., Жунисов Н.А., Елеусизов А.М., Акимниязова Б. Б., Касенбаев Р.Ж. Национальный научный центр хирургии им. А.Н.Сызганова г.Алматы, Республика Казахстан В статье представлены итоги диагностики и хирургического лечения 247 пациентов с кистозной гипоплазией легких в ННЦХ им.А.Н.Сызганова. Показаны результаты применения экстирпации бронхов при кистозной гипоплазии легких. Установлено, ранняя диагностика и оперативное вмешательство улучшают отдаленные результаты хирургического лечения.

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Diagnosis and surgical treatment of congenital cystic ad enomatoid malformations of the lung Esmuratov T., Shirtaev B., Sundetov M, Batyrhanov М, Zharylkapov N, Zhunisov N, Eleusizov A, Akimniyazova B, Kasenbaev R. National Scientific Center of Surgery Almaty, Kazakhstan In this paper results of diagnostic and surgical treatment for 247 patients with сongenital cystic adenomatoid malformations of the lunglung at the National Scientific Center of Surgery are presented. Outcomes of bronchi extirpation at the сongenital cystic adenomatoid malformations of the lung are described. It was determined that the early diagnostics and operative intervention in the childhood age improve the outcomes of the surgical treatment in the follow up period

Текст научной работы на тему «Diagnosis and surgical treatment of congenital cystic adenomatoid malformations of the lung»

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Вестник хирургии Казахстана №3, 2014

УДК 617-089

Diagnosis and surgical treatment of

congenital cystic adenomatoid malformations of the lung

Eshmuratov T., Shirtaev B., Sundetov M, Batyrhanov М, Zharylkapov N, Zhunisov N, Eleusizov A, Akimniyazova B, Kasenbaev R.

National Scientific Center of Surgery. Almaty, Republic of Kazakhstan

Аннотация

Диагностика и хирургическое лечение кистозной гипоплазии легких

Ешмуратов Т.Ш., Ширтаев Б.К., Сундетов М.М., Батырханов М. М., Жарылкапов Н. С., Жунисов Н.А., Еле-усизов А.М., Акимниязова Б. Б., Касенбаев Р.Ж.

Национальный научный центр хирургии им. А.Н.Сызганова

г.Алматы, Республика Казахстан В статье представлены итоги диагностики и хирургического лечения 247 пациентов с кистозной гипоплазией легких в ННЦХим.А.Н.Сызганова. Показаны результаты применения экстирпации бронхов при кистозной гипоплазии легких. Установлено, ранняя диагностика и оперативное вмешательство улучшают отдаленные результаты хирургического лечения.

Ключевые слова: кистозная гипоплазия легких, хирургическое лечение

Congenital cystic adenomatoid malformations of the lung (CCAM) is the most common (80%) pathology of lung malformations, in which multiple cavity in the lung and a violation of bronchial drainage create favorable conditions for the development of suppurative processes. Diagnosis and timing of surgical treatment of CCAM still remains an actual problem [1, 2, 3, 4, 5].

Since 1980 in our clinic diagnosed and operated on 247 patients with CCAM, which accounted for 29% of all patients with congenital lung malformations. The age of patients ranged from 2 to 56 years . Number of children (46%) and adults (53.8%) was similar. In 4.1% of cases, the disease is asymptomatic and detected during prophylactic radiological surveys. The first signs of the disease in the majority (71.3 per cent) patients were observed from early childhood in the form of constant coughing, frequent bronchitis and pneumonia. All of them are in different periods were examined in TB dispensaries, 11 (4.4%) of them were long-term treated with anti-TB drugs.

The only non-invasive radial method that allows correctly estimate volume and prevalence of pathological changes in the lungs, is computed tomography [6]. Using a computer tomograph examined 68 patients aged 4 to 56 years old with suspected CCAM. The verification was carried out according to bronhografii and surgery. Sensitivity of the method in detecting CCAM was 88.8% and specificity - 78.6%. Six false-negative results were obtained due to the fact that CCAM was mistaken for congenital bronchiectasis. False-positive results were obtained with an abscessed upper lobe bronchiecta-sis and bullous emphysema. Thus, most of discrepancies occurred in the differential diagnosis between CCAM and

bronchiectasis, but this did not affect the treatment strategy.

Diagnostic bronchoscopy performed to 235 patients. Observed dependence between the volume of the anatomical changes in the bronchi, nature and prevalence of inflammatory changes. In 58 (24,7%) patients on admission had bronchitis in the acute stage, and more than half (51.9 percent) bronchitis was under moderate exacerbation. Without exacerbation endobronchitis was 55 (23,4%) patients.

Bronchographic studies have shown that most often CCAM localized in the left lung - 36.2%. The most common combination was CCAM of lower lobe with ligula segments or middle lobe (35.2%), and most rare - a combination defeat of upper lobe with middle lobes (1.3%). Bilateral lesion of lung was detected in 21.6% of patients.

Objective information about the state of the pulmonary capillary blood flow gives radioisotope scanning. Examined 67 patients. In evaluating the scintigraphic data in 33 (49.3%) patients found to decrease in the accumulation of the radiopharmaceutical in the form of focal changes rounded shape , lung picture was deformed. In 34 (50.7%) patients showed a sharp decrease in the alveolar-capillary blood flow up to its complete lack of pathological areas of the lung tissue. In all cases, these data are consistent with the changes obtained during bronchoscopy and radiographic studies.

Angiography performed in 52 patients with CCAM. The complete absence of contrasting of the affected part of the lung was found in 6 (11.5%) patients. The most common angiographic sign of CCAM was thinning of branches of the pulmonary artery (59.6%). Uniform thinning of the pulmonary veins was detected in 29 (55.8%) patients. In 17 (32.7%) patients had a sharp delay in capillary and venous phases of contrasting.

At angiography determined pressure in the pulmonary artery. In all patients pulmonary artery pressure was within the normal range (mean dynamic blood pressure below 20 mm Hg), but in patients with a complicated course noted compared with a group of uncomplicated statistically significant (P <0.05) increase in mean dynamic pressure in the pulmonary artery.

Almost all patients were operated in the period of remission. Pulmonary hemorrhage in one patient required urgent operation . 247 patients underwent 251 (4 patients underwent one-stage bilateral operation) lung resections. The most frequently performed lobectomy (30.3%), combined resection (20.7%), and extirpation of the bronchi (17.9%). Surgery on his left lung were carried out in more than one and a half times more often.

Difficulties in determining the method of treatment in patients with unilateral localization of CCAM did not arise. These patients were in the majority - 179 (72.5%). The most common operations of the combined lung resection were lobectomy with segmental resection of the other lobe. These operations

Журнал Национального научного центра хирургии им. А.Н. Сызганова

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were carried out at the secondary irreversible transformations in neighboring bronchi (acquired bronchiectasis) or segmental cystic lesions of the other lobe. Also, while maintaining airiness and blood flow in the affected segments , to 45 (18.2%) patients underwent extirpation of changed bronchi , leaving the surrounding parenchyma. The most common variants were lower lobectomy of the left lung with the extirpation of bronchus of lingula segments (17 patients). This method was proposed by E.A.Stepanov (1969) as a method of surgical treatment of bronchiectasis in children [7]. The advantages of this operation are that the savings blood flow prevents the development of pulmonary hypertension, and saved pneu-matized segments act as a biological seal and prevent the occurrence of vicarious emphysema.

In total or with mosaic defeat of the lung, even in the presence of cystic changes in the other lung, pneumonectomy was performed. Pneumonectomy performed to 26 (10.5%) patients, of whom three had cystic changes in the other lung.

Much more difficult to find the indications for surgical treatment of bilateral CCAM. 46 patients with bilateral lesions of the lungs were performed stepwise pulmonary resection. The first stage of the operation was performed on the side of the largest defeat. The operation at the other lung was performed according to the the patient's condition in a few months or years. Of the 68 patients with bilateral lung involvement 53 (77,9%) first stage resection was performed most of the affected lung. 11 (16.2 %) patients was performed second stage - resection of the opposite lung in a period of several months to 8 years.

We performed 4 (5.9%) of simultaneous bilateral lung resections with CCAM. Operations were carried out through separate lateral thoracotomy. All one-stage bilateral and combined operations were no complications, postoperative period, with adequate anesthesia proceeded in the same manner as in the step-wise operations.

Early postoperative complications were observed in 24 (9.7% operated) patients. The most frequent complications were intrapleural bleeding (29.2% of complications) and postoperative wound abscess (37.5%). Noted a few cases of postoperative pneumonia and residual dry cavity. Early postoperative complications occurred more often after combined lung resections (33.4%), extirpation of the bronchi (20.8%) and lobectomy (20.8%), which coincides with the frequency of their performance.

In the late period in the period from 1 year to 22 years were examined 147 (59.5%) of the operated patients. Good results (the practical recovery) were observed in 64.1% of children, which is significantly higher than the results of surgical treatment of adult patients (P <0.05). Of satisfactory and unsatisfactory long-term results of significant differences is not established. All unsatisfactory results were observed in patients with bilateral lesions in the lungs and residual cysts in the operated lung.

Thus, for early diagnosis of CCAM need a comprehensive survey of patients, the active use of such highly informative and non-invasive methods, as computed tomography.

Extirpation of bronchi in CCAM has sanifying effect, and in disconnected segments saved airiness and blood flow, therefore, these segments serve as a biological seal and reduce the risk of pulmonary hypertension.

All one-stage bilateral and combined operations with cystic pulmonary hypoplasia were no complications, postoperative period, with adequate anesthesia proceeded in the same manner as in the step-wise operations.

Examination of patients in the late period showed a timely

diagnosis and early surgery reduces the risk of postoperative complications, improves long-term results.

Literature

1. Lezmi G, Hadchouel A, Khen-Dunlop N, Vibhushan S, Benachi A, Delacourt C. Congenital cystic adenom atoid malformations of the lung: diagnosis, treatment, pathophysiological hypothesis. //Rev Pneumol Clin. 2013 Aug;69(4):190-7. doi: 10.1016/j.pneumo.2013.06.001. Epub 2013 Jul 11.

2. Kotecha.S.Should.Asymptomatic congenital cystic adenomatous malformations be removed? the case against. //Paediatr Respir Rev. 2013 Sep;14(3):171-2. doi: 10.1016/j.prrv.2013.06.002. Epub 2013 Jul 17.

3. Макаров А.В., Сокур П.П. Врожденные аномалии бронхолегочной системы (диагностика, хирургическое лечение).// Украiнський пульмонологiчний журнал. 2003, №2. С..72-74.

4. Fauroux B. Congenital cystic adenomatous malformation (CCAM): remove or not, how can we progress? Paediatr Respir Rev. 2013 Sep;14(3):168. doi: 10.1016/j. prrv.2013.06.001. Epub 2013 Jul 17.

5. Peters RT, Burge DM, Marven SS. Congenital lung malformations: an ongoing controversy.//Ann R Coll Surg Engl. 2013 Mar;95(2):144-7. doi: 10.1308/003588412X13 373405387735.

6. Харченко В.П., Глаголев Н.А. Рентгеновская компьютерная томография в диагностике заболеваний легких и средостения. Москва, Медика, 2005, - 120с.

7. Исаков Ю.Ф., Степанов Э.А., Гераскин В.И. Резекция бронхов, как метод хирургического лечения бронхо-эктазий у детей.//Хирургия, 1975, №5. С.50-54.

Тужырым

Экпенн кистозды гипоплазиясын аныцтау жэне хирургиялыц емдеу

Ешмуратов Т.Ш., Ширтаев Б.К., Сундетов М.М., Батырханов М.М., Жарылкапов Н.С., Жунисов Н.А., Еле-усизов А.М., Акимниязова Б. Б., Касенбаев Р.Ж.

А.Н.Сызганов атындагы ¥лттыц хирургиялыц гылыми орталыгы

Мацалада вкпенiн кистозды гипоплазиясы бар 274 науцасты зерттеу жэне хирургиялыц емдеуд/'н нэтижелерi талцыланады. Бронхтарды экстирпациялау арцылы емдеу эд/Ън/'н цолдануы кврсетлдi. Хирургиялыц емдеуд/'н алыс нэтижелер/'н/'н жацсаруы вкпен н кистозды гипоплазиясын ерте аныцтау жэне оперативтi емдеуге байланысты.

Клтт/ свздер: вкпен/н кистозды гипоплазиясы, хирургиялыц емдеу.

Summary

Diagnosis and surgical treatment of congenital cystic ad enomatoid malformations of the lung

Esmuratov T., Shirtaev B., Sundetov M, Batyrhanov М, Zharylkapov N, Zhunisov N, Eleusizov A, Akimniyazova B, Kasenbaev R.

National Scientific Center of Surgery Almaty, Kazakhstan

In this paper results of diagnostic and surgical treatment for 247 patients with mngenital cystic adenomatoid malformations of the lunglung at the National Scientific Center of Surgery are presented. Outcomes of bronchi extirpation at the œngenital cystic adenomatoid malformations of the lung are described. It was determined that the early diagnostics and operative intervention in the childhood age improve the outcomes of the surgical treatment in the follow up period.

Key words: mngenital cystic adenomatoid malformations of the lung, surgical treatment

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