| ObjectiveThis study aimed to explore clinical features, diagnosis, treatment,and outcomes of children’s pulmonary sequestration (PS) to propose theprefer way of diagnosis and treatment.Material and methodsClinical records of30children with PS in Children’s Hospital ofChongqing Medical University between April1994and April2013wereretrieved, and the differences in clinical characteristics between ELS andILS were compared with SPSS19.0.Results1. Gender and AgeA total of30confirmed cases (12males and18females; gender ratio:1:1.5) were collected in this study. The ages ranged from13days+1hourto14year-old (average first diagnosed age:52.4±49.3months). Amongthem,1case (3.3%) aged~28days,6cases (20%) aged~1year-old,10cases (33.3%) aged~3year-old,4cases (13.3%) aged6year-old,8cases(26.7%) aged~13years old, and1case (3.3%) aged more than13year-old. 2. Symptoms and Finding on physical examination27patients (90%) showed nonspecific symptoms: fever (21cases,70%), cough (20cases,66.7%), expectoration/phlegm sound in the throat(13cases,43.3%), The following symptoms were also showed: fever+cough (16cases,53.5%), fever+expectoration/phlegm sound in the throat(12cases,40%) and fever+cough+expectoration/phlegm sound in thethroat (10cases,33.3%).3PS patients (10%) were absence of symptoms.21cases(70%) showed abnormal signs, decreased breath sound was foundon their affected side of15patients (50%),a few moist rales coarse wereheard in6patients (20%),and9patients (30%) did not exhibit positivesigns.3.Findings on image studieschest radiography, chest color Doppler ultrasound, chest CT plainscan, CT-enhanced scan, CT-enhanced+vascular three-dimensionalreconstruction, retrograde aortography and bronchofiberscope wereperformed in19,13,26,6,9,3and1patient, respectively.16patients(53.3%) were confirmed before operation, among them,9,4and3patientswere confirmed by enhanced-CT scan+vascular three-dimensionalreconstruction, enhanced-CT scan and DSA, respectively.3patients (10%)were not diagnosed before operation and then were found duringpulmonary lobectomy because they were accompanied by pulmonary cyst.11patients (45.8%) were misdiagnosed before operation.8,2and1patients were misdiagnosed with pulmonary cyst, tumour and tumourbullae of lung respectively.4.Difference between ELS and ILS 24patients (17intralobar sequestration and7extralobar sequestration)received excision, and2patients were given intervention closure, and4patients underwent conservative treatment.17cases of intralobarsequestration(ILS) can be summarized as follows:6males and11females,average first diagnosed age:52.4±49.3months, absence of symptoms:1case(found on physical examinations),positive signs:12cases(decreasedbreath sound9cases/moist rales coarse4cases), lesion site: left lowerlobe(9cases)/right lower lobe(8cases), complicated malformations:5cases（29.4%）,branch of abnormal supplying arteries:1branch(14cases)/2branches(1case)/5branches(1case)/uncertain(1case), source of abnormalsupplying arteries: thoracic aorta (14cases)/aorta abdominalis (1case)/intercostal artery (1case)/uncertain origin (1case), venous drainage: leftinferior pulmonary vein (7cases)/right inferior pulmonary vein (8cases)/unclear (2cases), average length of stay after operation:10.4±1.9days,average closed thoracic drainage time after surgery:3.6±1.0days;7casesof extralobar sequestration(ELS) can be summarized as follows:3malesand4females, average first diagnosed age:31.4±29.1months, absence ofsymptoms:1case(found on prenatal ultrasound),positive signs:7cases (allshowed decreased breath sound), lesion site: left costophrenic angle (5cases)/right costophrenic angle (2cases), complicated malformations:4cases（57.1%）,branch of abnormal supplying arteries:1branch(6cases)/uncertain(1case), source of abnormal supplying arteries: thoracic aorta (5cases)/aorta abdominalis (1case)/uncertain origin (1case), venousdrainage: left inferior pulmonary vein (2cases)/azygos vein (1case)/unclear (4cases), average length of stay after operation:9.3±1.3days,average closed thoracic drainage time after surgery:3.0±0.9days.5.Prognosis There was no operative mortality. Encapsulated pleural effusionoccurred in one patient as only post-operation complication. All weredischarged after successful treatment. Postoperative pathologicalexamination showed that the main manifestations were pulmonaryhypoplasia and chronic inflammation.1patient was found to havemycobacterium tuberculosis during acid-fast staining and then wasdiagnosed with combined tuberculosis. Only18patients (surgicalchildren:16cases, the patients receiving the intervention therapy:2cases)with PS were followed-up successfully, no complications of recurrentrespiratory tract infections or others were observed.Conclusion1. PS in children more easily occurred in the left lung, most of themshowed clinical manifestations before13years old. Recurrent respiratorytract infections were common. Clinical symptoms of pulmonarysequestration are not typical, the sum of missed diagnosis andmisdiagnosis rate was very high, and easy to be confused with pulmonarycyst, pneumonia, pulmonary tumor etc. Clinicians, especially pediatriciansneed to raise awareness of pulmonary sequestration urgently.2. There is no exactly difference between ELS and ILS in terms ofgender，first diagnosed age，symptoms，signs，the incidence of two lobes，complicated malformations，branch and source of abnormal supplyingarteries, venous drainage, average length of stay after operation, averageclosed thoracic drainage time after surgery. It is difficult to point theclinical types just by clinical features. Compared with ILS,ELS showedmore decreased breath sound and less merged multiple malformations.congenital heart disease and diaphragmatic hernia were most common congenital deformity which were associated with PS. Venous drainagethrough inferior pulmonary veins were common. ELSs were mainlylocated in the costophrenic angle, while ILSs the lower lobe, especially theleft lower lobe.3. Finding abnormal feeding artery from systemic circulation is thekey to confirm PS, color Doppler ultrasound and chest radiography can beused for routine screening, three-dimensional CT for the preferredconfirmation. It is necessary to perform gastrointestinal barium, cardiaccolor ultrasound and other examinations to exclude the possibility ofcombined malformations such as diaphragmatic hernia, bronchial gastricfistula, esophagotracheal fistula for the children who had been confirmed.Ultrasonography has its value in neonatal and prenatal diagnosis.4. Surgical resection is still preferred once a definite diagnosis wasmade, Pulmonary lobectomy was mainly used for ILS, while the lesionscan only be removed for ELS. it was not the absolute contraindication thatno classification was performed before operation or the trend of artery/vein was unclearly displayed. The surgery is safe, mortality orpost-operation complications were rare.Postoperative histopathologicalexamination is performed mainly to exclude whether other lesions (e.g,fungus, tuberculosis infection and even malignant change, etc.) arecombined. |
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