Received date: November 14, 2015; Accepted date: December 23, 2015; Published date: December 28, 2015
Citation:Kasem E, Kassem H (2015) Surgical Treatment for Congenital Lung Parenchyma and Non Lung Parenchyma Disorder: Center Experience. J Pulm Respir Med 5:306. doi:10.4172/2161-105X.1000306
Copyright: © 2015 Kasem E, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
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Objective: Non parenchyma lung disorders are rare entity with life threatening outcome. Early surgerical intervention is the clue for life saving and avoid life threatening complications.
Methods: From Aug 2008 to dec 2014, 101 cases operated in Zagazig University Hospital for congenital non parenchyma disorder data are collected regarding preoperative, intra operative and post operative results.
Results: The mean age of our patients was 27 month (1 week - 120 month). F\M 61\40. Mean symptom is respiratory distress and frequent chest infection. Tracho-esophygeal fistula 24 cases, Congenital diaphragmatic hernia 17, Diaphragmatic event ration 6, congenital lobar emphysema 37, cystic adenomatoid malformation (n=9), pulmonary sequestration (n=7) and arteriovenous malformation (n=1).
Conclusion: Early surgerical intervention for parenchyma and non parachyanma l disorder is the primary curative to avoid life threatening complications.
Congenital malformations; Lung parenchyma; Congenital cystic adenomatoid malformation; Bronchogenic cyst; Congenital diaphragmatic disorder
CLE: Congenital Lobar Emphysema; CCAM: Congenital Cystic Adenomtoid Malformation; TOE: Tracheoesophageal Fistula
Congenital disorder of thoracic content is a rare entity, with estimated annual incidence 30 to 40 cases per 100.000 populations . The spectrum of these disorders include congenital lobar emphysema (CLE), congenital cystic adenomatoid malformations (CCAM), Pulmonary sequestration, anomalies of diaphragm and Tracheo-esophageal fistula [2-4]. The pathology of these disorders occurring from failure of primitive intestine and its differentiation into respiratory system . The presentation of these disorders rang from respiratory failure to imaging alterations in non symptomatic adult patients [5-7]. Prenatal diagnosis is helpful in early management. . CT chest is main diagnostic tool of the pathology and allow for proper surgical planning . Early surgery is the principal treatment for various forms of paraenchymal and non paraenchymal lung malformations. Pulmonary resection, lobectomy being the most common procedure [3,5,8,9] resections should be as conservative as possible. All pulmonary resections performed through thoracotomy. The other anomalies repaired either through thoracotomy or through abdominal incision .
Objective of our study present our experience with surgery in congenital lung disorder and other thoracic contents
We retrospective analysis 101 cases operated for congenital disorder of lung and other thoracic content in cardiac surgery department and pediatric surgery unit in Zagazig University Hospital from Aug 2008 to dec 2014. 107 Cases have been operated. Data analyzed regarding age, sex, symptoms of presentation and imaging test, side of the lesion and its location, surgical procedures used. post operative data include ICU, prolonged ventilation.
All patients operated through thoracotomy according to affected side. Under general anaesthesia patients positioned in lateral position. Emergent consideration taken for cases of congenital lobar emphysema. Posterior thoracotomy performed in 4th or 5th intercostals space. surgery done according to the congenital lesion, pulmonary resection have been performed for cases of congenital lobar emphysema, reeducation of abdominal content and closure of defect with mesh used for congenital diaphragmatic hernia, placation of diaphragm for congenital event ration and for Tracheoesophageal fistula ligation of fistula. Data analyzed, mean and median age were calculated, mean length of hospital stay and percentage and location of each disease.
The mean age was 4 month (1 week - 120 month). There was predominance of females with female to male ratio (61\40).
Mean symptom is respiratory distress except in 9 cases asymptomatic, (6 cases with CCAM, 1 case with diaphragmatic event ration, 1 lobar emphysema, 1 tracheooesophageal fistula).
The pathology and side of congenital anomaly are described in Tables 1 and 2 and for all patients chest x ray as routine and CT chest as confirmatory test. 11 cases required preoperative rigid bronchoscope for treatment of pneumonia (Figures 1 and 2).
|Cystic adenomatoid malformation|
|Congenital diaphragmatic hernia||17||16.8%|
|Congenital diaphragmatic event ration||6||5.9%|
Table 1: Pathological lesion in 101 cases.
|Side of anomalies||n||%|
|Left upper lobe||19||18.8%|
|Left lower lobe||2||0.198%|
|Right upper lobe||11||10.8%|
|Right middle lobe||10||9.9%|
|Right lower lobe||7||6.9%|
|Diaphragmatic event ration|
|Congentail Diaphgramatic Herina||17||16.8%|
Table 2: Side of pathology.
17 cases had post operative complications, (3 pneumonia, 4 pulmonary atelectasis 3 of them require bronchoscope for respiratory care, and 6 cases had pneumothorax after ICT removal, Managed conservative in 4 cases and 2 cases require another ICT., 4 cases has pleural effusion with re-insertion of intercostals tube).
Two patient out of 37 cases of CLE were operated while they are on mechanical ventilation, one case recovered another require prolonged mechanical ventilation die from sever respiratory infection and One case out of 24 cases of TOF was intubated and operated on mechanical ventilation. He survived after surgery.
The mean length of ICU stay was 5.4 (3-7 days) while total hospital stay was 11 day from (5-33 days) (Table 3).
|Post operative complications||n||%|
|Mean ICU stay||11 ( 5-33)day|
|Mean hospital stay||5.4(3-7)day|
Table 3: Post operative complications.
Congenital anomalies of lung parenchyma and thoracic content is rare entity that require surgical intervention in majority of cases to manage the symptoms and save of remaining lung tissues (1.3.5).
The estimated annual incidence of thoracic anomaly is 30=40 cases per 100.000 population  with predominance in female to male 2 to 1  same we reported in our study predominance of females than males 60% of our cases are females.
Respiratory disorder is the main presentation [5-7]. However, One case of our study was discovered during routine x-ray after traffic accident. She has diaphragmatic event ration (cases no 91). Recently many of cases diagnosed during prenatal care and so decision can be taken early or after close observation.
Surgery is the curative tool of diagnosed cases. Surgical strategy according to diagnosis case with (CLE) surgery go with symptoms, cases with mild symptoms require follow up while symptomatic cases planned for surgery, lobecttomy is the main main surgery depened on affected lobe. While cases with (CACM), Asymptomatic cases require close observation and fellow up to manage symptoms and avoid complications as in cystic adenoid malformation to avoid malignancy (bronchio alveolar carcinoma, rhabdomyosarcoma) [11,12]. No surgery carried as prophylactic for our cases, surgery is indicated on symptoms.
Pulmonary resection is the main surgical procedure for diagnosed case of congenital lobar emphysema, intra-pulmonary sequestration and congenital cystic adenomatoid malformation. While for extra pulmonary sequestration, sequestrectomy is indicated [13-16].
For cases diaphragmatic event ration, placation of diaphragm form thoracic side done [19,20] surgery is done once diagnosis confirmed and cases with tracheoesophgeal fistula surgery is done through right throcaotomy in 19 cases and by neck incision for H type fistula [21-23].
Three cases of TOF have right aortic arch . In our study we operate through thoracotomy according to affected side and we use 4th or 5th space according to affected lobe. Although in many center videoassisted thoracoscopy has been widely used for pulmonary resection. Upper pulmonary resection is most common type of resection for cases of CLE, while for cases of CAM, lower lobectomy is used as many studies [11-13,15,17].
We reported 5 cases with combined anomalies three cases with right aortic arch in cases of TOF, one case with right coronary artery fistula to right atrium and one case with deformed chest wall pectus excavatum. Some studies reported higher incidence of mixed pulmonary malformation which is not reported in our study and also reported high incidence of cardiac, esophageal and chest wall malformation, but the incidence in our study is lower than reported.
The reported post operative complications are mainly pleural in term of pneumothorax and parenchyma in form of atelectasis which require bronchoscope for management. This also reported in many series [13-15] which reported high incidence of pleural complications after pulmonary resection.
Surgery is the clue for congenital parenchyma lung disorder and non parenchyma disorder, surgery is applied as diagnosis confirmed carry the advantages of saving lung parenchyma and avoid life threatening complications or malignant conversion in some anomaly like CAM.
We want to express our appreciation to Dr. Nader Abdul Raheim Fayed for his valuable advise and great support.