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I. ДИАГНОСТИКА И ЛЕЧЕНИЕ
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DIAGNOSIS AND SURGICAL TREATMENT OF LUNG HYPOPLASIA
ABOUT THE AUTHORS
Shirtaev Bakhytzhan Kerimbekovich MD, PhD, the branch manager of toracic and child surgery department NSSC named by Syzganov A. N. e-mail: [email protected] Sundetov Mukhtar Magzomovich -MD, PhD, thoracic surgeon. Kasenbayev Ruslan Zhumakhanovich -Thoracic surgeon. Voronin Denis Sergeevich -children's surgeon.
Keywords
pulmonary hypoplasia
Shirtaev B.K., Sundetov M.M., Kasenbaev R.J., Voronin D.S.
National Scientific Surgery Center under the name of A.N.Syzganov, Almaty, Kazakhstan
Abstract
The article presents the results of diagnosis and surgical treatment of 21 patients with pulmonary hypoplasia. Established that the immediate and late results of surgical treatment of children better than adults.
вкпе гипоплазиясыньщ диагностикасы мен хирургияльщ eMi
АВТОРЛАР ТУРАЛЫ
Ширтаев Бахытжан Керимбекович -м.г.к., А.Н.Сызганов атындагы YFXO торакалды жене балалар хиругиясы бел/м/н/н менгерушю e-mail: [email protected] Сундетов Мухтар Магзомович -м.г.к., торакалды хирург. Касенбаев Руслан Жумаханович -торакалды хирург. Воронин Денис Сергеевич -балалар хирург/.
Туйш сездер
вкпе гипоплазиясы
Ширтаев Б.К.,Сундетов М.М., Касенбаев Р.Ж., Воронин Д.С.
A. H. Cbi3f3H0B атындаш улттык, шлыми хирургия орталыш, Алматы, К,азак,стан
Ацдатпа
Макалада вкпе гипоплазиясы бар 21 наукастын диагностикасымен хирургиялы^ емдеу нэтижелер1 кврсетлд1. Балаларга жасалынран хирургиялыц ем ересектерге цараеанда жак,сы нэтиже кврсетуде.
Диагностика и хирургическое лечение гипоплазии легкого
ОБ АВТОРАХ
Ширтаев Бахытжан Керимбекович -к.м.н., зав. отделением торакальной и детской хирургии ННЦХ им.
А.Н.Сызганова. e-mail: [email protected] Сундетов Мухтар Магзомович -к.м.н., торакальный хирург. Касенбаев Руслан Жумаханович -торакальный хирург. Воронин Денис Сергеевич -детский хирург.
Ключевые слова
гипоплазия легкого
Ширтаев Б.К.,Сундетов М.М., Касенбаев Р.Ж., Воронин Д.С.
Национальный научный центр хирургии им. А.Н. Сызганова, Алматы, Казахстан
Аннотация
В статье представлены результаты диагностики и хирургического лечения 21 пациента с гипоплазией легкого. Ранняя диагностика и оперативное лечение у детей улучшает отделенные результаты.
ВЕСТНИК ХИРУРГИИ КАЗАХСТАНА № 3-2016
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Among the operated patients with congenital disorders of the lung hypoplasia is 1.3-15.7% of cases. Malformation of lung characterized by uniform size decreases lung lobe or segment without significant disruption of the structure as a result underdevelopment of the bronchi and lung parenchyma [1, 2, 3, 4, 5].
In our clinic for the last 30 years, 21 patients were operated with pulmonary hypoplasia, which is 2.1% of the operated congenital lung disease. If among children and adolescents number of women and men was similar, among adult patients was dominated by men (table 1).
This pathology is often diagnosed in childhood. According to our data, the first signs of defect in 61.9% of patients manifested from early childhood, and at 23.8% in the subsequent years of life. In one case, the disease was asymptomatic and was detected during routine examination. By the time of the survey in 2 (9.4%) patients the duration of clinical symptoms was less than 5 years.
On admission 95.2% of patients complained that established the existence of a more or less pronounced inflammation in the lungs .In 11 children found to delay in growth and development. Seven children were malnutrition with deformity of the chest, with narrowing of intercostal spaces and atrophy of the pectoralis major muscle. At the entire lung hypoplasia (9 patients) breathing on the affected side was weak or with dry and moist rales.
On radiographs with hypoplastic lung observed decrease transparency in the areas of lung tissue disorders (detected in 52.4% of patients), the change in the architectonics of the bronchi (in 57.1%), depletion of lung pattern (33.3%), narrowing of intercostal spaces (in 38.1%), a high standing dome of the diaphragm on the affected side (at 42.8%). Normal lung hypertrophy, its transparency increased (47.6%) (picture 1).
Computed tomography of the lungs, giving the image a three-dimensional space, with precise measurements, with difficulties verify the diagnosis accurately localize hypoplasia, prevalence, the condition of the bronchi and parenchyma. 9 patients, because of the uncertainty nature of the pathological process, performed computed tomography of the lungs. In the four cases were found uniformly sharply reduced lungs with proportionally lent bronchi that is visualized only within the root and do not reach the cortex (Picture 2). At hypoplasia in 5 patients near to vicariously larger normal lobe was detected reduced lobe.
Fibrobronchoscopy was performed in 18 patients (8 adults and 10 children). Endoscopic picture was different depending on the prevalence of hypoplasia and nature of endobronchitis. On the basis of the endoscopic picture in 11 (61.1%) patients were able to establish hypoplasia: observed rough deformation and bronchodilation of the affected lobe, that at the segmental level is not clear, mucous had corrugated appearance, and bronchial lung unaffected departments were shifted to the affected side . In 5 patients (27.8%), the trachea was shifted to the affected side, bronchial lumen was normal, but they were close together, was a decrease respiratory mobility. Inflammatory stenosis of the lobar bronchi detected in two (11.1%) patients. In the event of a suspected lesion of the entire lung and to determine the extent of surgery was performed in 8 patients angiopulmonography. At angiopulmonography in 6 patients revealed hypoplasia of the pulmonary artery and a sharp impoverishment of all the vessels of the lung (Picture 3).
In two patients found the narrowing of the pulmonary artery. In all patients, the capillary phase was delayed. Increase in the average dynamic pressure in the trunk of the pulmonary artery to 22 mm. hg. art. found only in one patient with a simple hy-poplasia of the left lung, the remaining pressure in the pulmonary circulation was normal.
On the basis of radiation and endoscopic methods was installed next localization pulmonary hypoplasia (Table 2).
Thus hypoplasia often localized in the left lung - 15 (71.4%) cases. Defeat the entire lung was in 9 (42.8%) patients, two lobes in one patient, and the rest revealed hypoplasia of lung lobe. In one adult
Sex children and adolescents % Adult %
Female 7 53,8 3 37,5
Male 6 46,2 5 62,5
Total 13 100 8 100
Picture 1
Chest radiograph of the patient T., 17 years old
Hypoplasia of the left lung
Table 1
distribution of patients by sex
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Table 2
Localization of lung hypoplasia (lobe)
children Adult
Localization of lung hypoplasia (lobe) (n=13) % (n=8) %
The left lung 5 38,4 2 25
upper lobe 2 25
lower lobe + lingular segments 1 7,7 1 12,5
lower lobe 4 30,8
The right lung 2 15,4
upper lobe 1 7,7 1 12,5
lower lobe 1 12,5
lower and middle lobe 1 12,5
Total 13 100 8 100
Picture 2
CT scan of the chest of the patient T., 17 years old
Picture 3
Angiopulmonogramma at hypoplasia of the left lung
patient hypoplasia of the lower lobe combined with acquired bronchiectasis of lobar segments. Due to prolonged recurrent exacerbations of purulent process in the lower lobe appeared in lobar segments secondary bronchiectasis, which further led to the expansion of the volume of surgery.
Early diagnosis and prompt surgical treatment of lung hypoplasia avoids subsequent inflammatory changes in the normal parts of the lungs and arteriovenous shunting. Therefore, the presence of lung hypoplasia we consider the indication for surgical treatment.
Lungs hypoplasia frequently detected in childhood, so their frequency of pneumonectomy was twice higher than in adult patients (Table 3)..
Lower lobectomy produced more in children, the upper - adult patients. This is probably due to the fact that a simple upper lobe hypoplasia occurs often asymptomatic or sparse short exacerbations that led to late diagnosis.
Adult patients had a longer anamnesis of disease, so they all have dense and extensive adhesions in the pleural cavity. This has led to an increase in trauma and duration of the operation, required especially careful selection of vessels and bronchi. In two cases (9.5%) due to sharply thickened pleura expressed adhesive processes, displacement of the mediastinum and cover hilar elements by heart performed transpericardial pneumonectomy.
Postoperative bronchial fistulas more probable at long-term ill patients with malformations associated with the lungs and bronchus structural disorders, or nonspecific chronic or tuberculosis process. We have developed a method of treat-
Table 3
The nature of surgical interventions in hypoplasia lung (lobe)
Type of surgery children % Adult % Total %
pneumonectomy 7 53,8 2 25 9 42,9
Bilobectomy 1 12,5 1 4,7
lobectomy:
upper 1 7,7 3 37,5 4 19,1
lower 4 30,8 1 12,5 5 23,8
combined resection 1 7,7 1 12,5 2 9,5
Total 13 100 8 100 21 100
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ВЕСТНИК ХИРУРГИИ КАЗАХСТАНА № 3-2016
Figure 4
Picture of the method of closing the stump of a major bronchus after pneumonectomy or lobectomy
ing bronchial stump to prevent bronchial fistula. This method is performed as follows (Picture 4). After pneumonectomy with separate processing elements of the root suture on the cult of the bronchus additionally fortified by suture as shown in the picture and folding bronchus contraction ends and ligation ligature back first, and then in front of bronchial stump.
Postoperative complications and deaths was not. All patients were discharged in satisfactory condition.
In the late period from 1 year to 14 years were examined 12 (57.1%) operated. Long-term results
of surgical treatment of hypoplasia we assess as good - in 10 (75%), satisfactory - in 3 (25%) patients. Unsatisfactory results were not.
Thus, early surgical treatment of a simple hypoplasia prevents the development suppurative processes in the lung pathological shunting of blood and pulmonary hypertension. Removal of localized chronic suppurative focus leads to a reduction of inflammatory changes in the bronchial tree up to their complete disappearance The risk of postoperative complications increases with the duration of the clinical manifestations and age of the patient.
References
1. Abrams M.E, Ackerman V.L, Engle W.A. Primary unilateral pulmonary hypoplasia: neonate through early childhood - case report, radiographic diagnosis and review of the literature. J Perinatol. Oct. 2004; 24(10):667-70.
2. Kant S. Unilateral pulmonary hypoplasia. A case report. Lung India. 2007; 24:69-71.
3. Sunam G, Ceram SJ. Pulmonary artery agenesis and lung hypoplasia. Eur J Gen Med. 2009;6:265-7.
4. Delgado-Peca Y.P., Torrent-Vernetta A., Sacoto G. et al. Pulmonary hypoplasia: An analysis of cases over a 20-year period. An Pediatr (Bare). 2015 Nov 25. pii: S1695-4033(15)00405-1.
5. Calzolari F., Braguglia A., Valfra L. et al. Outcome of infants operated on for congenital pulmonary malformations. Pediatr Pulmonol. 2016 May 27. doi: 10.1002/ppul.23472.
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