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Eur J Cardiothorac Surg 2005;28:50-55
© 2005 Elsevier Science NL

Thoracic surgery in children

Department of Thoracic and Vascular Surgery, Universitätsklinikum Ulm., Steinhövelstr. 9, 89070 Ulm, Germany

Received 13 November 2004; received in revised form 8 March 2005; accepted 10 March 2005.

^* Corresponding author. Tel./fax: +49 2361 9386336. (Email: peterkanngiesser{at}bdc.de).

	Abstract

Top Abstract 1. Objective 2. Patients and method 3. Results 4. Postoperative course 5. Conclusion References

 
Objective: Children rarely undergo thoracic surgery. When they do, the procedures fall into five main groups: oncologic indications, immune defects, malformations, infections and trauma. In addition to considerations associated with the underlying indication, the different proportions of the anatomical structures in children require special modifications in both diagnostics and surgical technique compared to corresponding procedures in adults. Methods: Of a total 2137 thoracic surgical procedures performed between 1992 and 2001, 49 were performed in children (n=37; age: 3 months–15 years; median age: 8 years). Indications for surgery included underlying oncologic disease (n=20), immunodeficiency (n=5), thoracic or pulmonary malformation (n=6) and trauma (n=3). Patients' postoperative clinical course was analyzed retrospectively for all 49 procedures. Pre- and postoperative pulmonary function test results are available for 16 children. Data regarding quality of life were documented in 24 children. Results: The following procedures were performed: 27 atypical resections, seven lobectomies, one pneumonectomy, three decortications, four mediastinotomies or mediastinoscopies and seven other procedures. Six procedures represented second or third procedures in the same patient. Two of six patients with immune defects died during the perioperative period. Eleven of 20 oncologic patients (55%) have remained free of recurrent disease. Quality of life, as assessed by the Karnowski index in 24 children, was at least 80%. Conclusions: Thoracic surgical procedures in children with underlying benign disease are associated with a good prognosis and high quality of life scores. Surgical treatment of pulmonary metastases is a feasible component of the overall oncologic therapy concept and can offer the only opportunity for curation for a selected group of patients. Because of high postoperative mortality, however, the indication for diagnostic thoracotomies in children with immunodeficiencies and poor general health should be weighed critically.

Key Words: Thoracic surgery • Children • Metastases • Immune defect

	1. Objective

Top Abstract 1. Objective 2. Patients and method 3. Results 4. Postoperative course 5. Conclusion References

 
Children rarely undergo thoracic surgery. When they do, the indications for such surgery frequently differ from those underlying thoracic surgical procedures in adults. Furthermore, because pediatric patients present with a number of disease entities that do not fully correspond with pathology seen in adult patients, the typical distinction between benign and malignant disease [1–3] would benefit from further differentiation.

Our departmental practice has been to class thoracic surgical procedures in children to five main groups. The first group consists of oncologic patients. In comparison to adults, children present most frequently with metastatic disease and less often with primary malignancies of the thorax. Most commonly seen in our collective were metastases of nephroblastomas, osteosarcomas and pulmonary manifestations of other malignant processes, such as Hodgkin's disease or acute leukemic disorders. The remaining classes include patients with immune defects, and those suffering from malformations, infections or trauma.

A second area of distinction between thoracic surgical procedures in children compared to those performed in adults relates to differences in surgical technique required by the special physiological and anatomical characteristics of children. On one hand, the smaller size of children's anatomical structures restricts access while, on the other hand, special attention must be paid to structures that are important for the child's continued normal somatic growth.

The present retrospective study gives an overview of pediatric patients presenting with diseases of the lung, mediastinum and thorax wall treated in our department between January, 1992 and January, 2001. In addition, we report on functional results and the children's long-term clinical course.

	2. Patients and method

Top Abstract 1. Objective 2. Patients and method 3. Results 4. Postoperative course 5. Conclusion References

 
Of a total 2137 thoracic surgical procedures performed between January, 1992 and January, 2001, 49 were performed in children (n=37; 20 males, 17 females; median age: 8 years; age range: 3 months–15 years). These included six second operations, one third operation and five cases in which two procedures were performed at the same surgical session.

Patients' underlying pathology and the respective indications for surgery were classed to five main groups. The first and largest group consisted of oncologic patients (n=20, 20,5%) and here we differentiated between primary tumors, metastases and diffuse pulmonary manifestations of different malignant disorders. One patient presented with a ganglionic neuroblastoma (Stage IIa INNS). Six patients each suffered from pulmonary metastases of nephroblastoma (Stage IV SIOP) or osteosarcoma, while one patient each showed pulmonary metastases derived from rhabdomyosarcoma and a germ-cell tumor (dysgerminoma Stage IV). The time of presentation of metastases in these patients varied between 0 and 48 months after first presentation of the disease. The group with diffuse pulmonary manifestations of other malignant disorders included two patients each with Hodgkin's disease (Stage IV Ann Arbor) or acute lymphocytic anemia (ALL) and one patient with acute myeloic leukemia (AML). Group II consisted of children undergoing thoracic surgery in the management of an immune defect (n=5, 14%). These children presented with respiratory insufficiency due to a primary or secondary immune defect and required lung biopsies to confirm the diagnosis. Group III included children undergoing surgery for treatment of a pulmonary or thoracic malformation (n=6, 17%). Patients undergoing thoracotomy for treatment of infectious diseases involving the lung made up group IV (n=3).

Finally, group V consisted of three patients undergoing thoracic surgery secondary to trauma. Patient characteristics are summarized in Table 1 .

	3. Results

Top Abstract 1. Objective 2. Patients and method 3. Results 4. Postoperative course 5. Conclusion References

 
3.1. Surgical procedures
During the observation period, a total of 49 thoracic surgical procedures were performed in 37 children. Six children underwent two or three procedures and eight procedures were performed simultaneously with another procedure.

Atypical resections were performed in 27 cases. Indications included metastatic disease (n=15); diagnostic biopsy in cases of diffuse pulmonary infiltration in patients with leukemia (n=2); diagnostic biopsy in patients with an immune defect (n=3); malformation (n=4); infection (n=1); and trauma (n=2). Multiple resections (up to 10) were performed in four children. Six children underwent atypical resections as a second or third surgical procedure.

Seven children underwent lobectomy. These included three children with pulmonary metastases of osteosarcoma (n=2) or nephroblastoma (n=1); one child with pulmonary malformation (bullous emphysema); one child with pulmonary infiltrates in a combined immune defect syndrome; one child with a gunshot wound; and one child with a stab wound. In addition, bilobectomy was performed in one case due to metastases of rhabdomyosarcoma and pneumonectomy in another due to metastases of osteosarcoma. Three decortications were performed. Indications included so-called ‘captured lung’ following recurrent pneumonia (n=2) and pleural fibrosis in a patient with an immune defect syndrome (n=1). Two children underwent diagnostic mediastinoscopy, including one child of the immune defect group with mediastinal lymphomas and one child with mediastinal lymphomas in Hodgkin's disease. Two mediastinotomies were performed. Indications included mediastinal lymphomas in ALL (n=1) and Hodgkin's disease (n=1).

Other procedures included two exploratory thoracotomies in children with pulmonary metastases following atypical resection and after chemotherapy of a nephroblastoma or an osteosarcoma; cartilage resection in the malformation group; and two extirpation of malignant tissue in patients with neuroblastoma (n=1) and lymphoma (ALL; n=1).

Six patients underwent a second operation. Indications included surgery for metastases (osteosarcoma) in three children, some of whom had undergone multiple resections (up to eight). One child who had undergone atypical resection of pulmonary metastases of nephroblastoma was referred for diagnostic thoracotomy following chemotherapy. One child required decortication 10 days after empyema clearance following resolution of abscess-forming pneumonia. Finally, one child with tension pneumothorax secondary to bullous emphysema required a second operation (atypical resection and partial pleurectomy). This child's first surgery included an atypical resection.

One patient with osteosarcoma who had undergone two resections of pulmonary metastases was referred for diagnostic thoracotomy following completed chemotherapy. The procedure was performed as part of a third operation (Table 2 ).

	4. Postoperative course

Top Abstract 1. Objective 2. Patients and method 3. Results 4. Postoperative course 5. Conclusion References

The 30-day mortality for hospitalized patients stood at 8% (n=3). Two patients who expired within the first 30 postoperative days suffered from an immune defect. The third child, who suffered from a B-cell AML, developed a pneumonia. All three of these children had been on a ventilator with a FiVi of 1.0. The thoracic surgical procedure in all three of these children was performed to obtain an open biopsy specimen.

A total of nine additional children (24%) died during the entire follow-up period. These included eight childen in the oncologic group and one child with an immune defect, who had undergone lobectomy for treatment of a pulmonary abscess.

Recurrence of metastatic disease after a disease free interval (DFI) was observed in seven patients during the observation period (osteosarcoma, n=5, DFI between 2 and 29 months; nephroblastoma, n=1, DFI: 2 months; rhabdomyosarkoma, n=1, DFI: 2 months).Two patients with osteosarcoma exhibiting pulmonary recurrence of metastases underwent a second operation (DFI: 2 and 7 months). All children in the oncologic group developing recurrent metastatic disease died due to the consequences of their malignancy within the follow-up period. One patient with pulmonary metastases of a germ-cell tumor died from multi-organ failure resulting from secondary AML. Median survival from the documented date of disease recurrence was 4 months (range: 6 weeks–9 months). Table 3 summarizes the causes of death for patients expiring during the observation period.

4.1. Pulmonary function test results
Pre- and postoperative pulmonary function test results are available for 16 patients. Postoperative pulmonary function tests were performed on the 30th and 60th postoperative day.

Results of postoperative pulmonary function tests showed a median improvement in vital capacity of 8% (range: 0–15%) and of 7% in FEV1 (range: 0–10%) in the group of children who had undergone one or multiple atypical resections. Five patients underwent postoperative pulmonary function tests following lobectomy. These children showed a median reduction in vital capacity of 25% (range: 10–40%) and a 25% drop in FEV1 (range: 10–30%). As expected, the patient who underwent pneumonectomy showed a 50% reduction in pulmonary function after 3 months.

Other surgical procedures, such as decortication and thorax wall resections showed median improvements in vital capacity of 10% and of 20% in FEVi. There was no difference in pulmonary function measured on the 30th or 60th postoperative day (Table 4 ).

	5. Conclusion

Top Abstract 1. Objective 2. Patients and method 3. Results 4. Postoperative course 5. Conclusion References

 
The indications underlying thoracic surgical procedures in pediatric patients are many and varied. The majority of indications are oncologic in nature, the majority of which being resections of pulmonary metastases. Consideration of the different disease entities is difficult in a review paper [1]. In our opinion, classifying indications to five groups (oncology, immune defect, infection, malformation, trauma), as we have done, helps to clarify the situation.

5.1. Indication
The indication for surgical resection of both solitary and multiple pulmonary metastases was established within the framework of a multidisciplinary oncologic concept. In this group, there were three indication for a thoracic surgical procedure.

Curative intention with complete resection of malignant tissue: in some cases this requires multiple resections, which proved feasible. Palliative resection in the sense of tumor debulking is not associated with any prognostic advantage.

Following chemotherapy, the lung is explored for remaining viable malignant tissue as part of a multidisciplinary management concept. This means that, even if radiologic imaging demonstrates complete resolution of a metastasis as a result of chemotherapy, the affected lung is palpated and the site of the metastasis is resected. If viable tumor tissue, however, is detected, chemotherapy must be re-started [12].

In cases of metastasizing osteosarcoma in which radiology demonstrates only unilateral pulmonary lesions, it is recommended that patients undergo surgical exploration of both lungs, since palpation by an experienced thoracic surgeon is the most sensitive method for discovering even the smallest metastases that may no longer be considered viable based on histologic criteria [12,15]. An aggressive approach to the treatment of pulmonary metastases is justified when the criteria for establishing a curative intention are met [10,16]. In our series one child with pulmonary metastases of a primary osteosarcoma underwent 10 atypical resections and has remained disease free during the subsequent follow-up period. Second thoracotomies may also be justified in patients with recurrent disease. Snyder et al. [11] McCarville et al. [10] report good results in children who had undergone five to six thoracotomies for management of pulmonary metastases of osteosarcoma.

Four of five children with an immune defect underwent a thoracic surgical procedure for purely diagnostic reasons. In these children, no reason for worsening in respiratory function could be identified. In none of these cases was the diagnostic thoracotomy with biopsy associated with any therapeutic consequence for the children involved; instead, they only contributed to their discomfort. Two of these children, who preoperatively required respiration at a FiVi of 1.0, died within the first 30 days. One other child died 18 months after the procedure. While the research group of Jaklitsch et al. [6] report similar results, there are reports in the literature of an actual diagnostic benefit in up to 60% of patients and a therapeutic benefit in 30%. A modified therapeutic regimen was associated with clinical improvement in these septic children [8]. Oyarzun et al. [7] report a diagnostic benefit in cases of perinatally acquired HIV infection with pulmonary involvement. In that study, the findings of surgical biopsy resulted in all children undergoing appropriate management. Based on our experience, the indication for thoracic surgery in children with an immune defect should be critically weighed, particularly when preoperative respiratory insufficiency already requires respiration with high FiVi. In such cases, the indication for diagnostic thoracotomy should be made earlier in the course of the patients' disease.

In cases of infection with intraparenchymal processes that may lead to atelectasis, we recommend that conservative management with antibiotics and intensive respiratory therapy be continued as long as possible. In cases of pleural involvement, we distinguish between early and late disease manifestations. In early manifestation, for example, with pleural effusion, we recommend the prompt placement of a thorax drainage. Should the patient experience a late manifestation, as in cases of unsuccessful drainage, the indication for surgery should be considered. Prompt decortication can counter the danger of pleural fibrosis and subsequent restriction.

The diagnosis of thoracic malformations is often preceded by unclear radiological findings. The indication for thoracic surgery should be favorably considered, particularly in view of establishing a diagnosis [2,4]. In cases of trauma involving the thorax, our own findings also suggest that the indication for surgery should be favorably considered.

5.2. Diagnostics
Besides the conventional chest X-ray, it is routine practice to obtain a CT examination, which remains the gold standard in pulmonological diagnostics. Modern scanners are able to detect pulmonary focal lesions as small as 3mm in diameter [13]. In selected cases, preoperative bronchoscopy may be warranted and should be preferred to a diagnostic procedure. Bronchoscopy permits evaluation of the mucosa and biopsies can be taken. Bronchoscopy also offers interventional capabilities, such as aspiration or the dilation of stenoses. Virtual bronchoscopy with three-dimensional reconstruction of thorax CT data is a gentle method very suitable for children. Using helical CT data, a computer generates images from which the structure of the bronchi, lungs and blood vessels, together with the position and size of the tumor can be recognized. Based on a volume-oriented, automatic segmentation of the bronchial tree and pathological changes, the computer generates a three-dimensional model. Virtual bronchoscopy can be performed in real time. Its advantage lies in the excellent evaluation of the lumen and in its capacity for post-stenotic evaluation of the bronchial system. A disadvantage is the inability to directly view the mucosa. Its diagnostic value at the present time, therefore, remains limited [14].

5.3. Surgical technique
The surgical technique of thoracotomy in children differs from that used in adults in several important steps. As in adults, we select the anterolateral approach. This is important in children because this approach spares the latissimus dorsi and thoracodorsal muscles, both of which are important structures for the growth of the chest wall. Whereas osteotomy of the ribs is necessary in adults, it can be avoided in children, since the intercostal spaces are easily spread. As is the practice with other research groups [5] and because they result in less tissue damage, we use Endo-Gia devices for resection of lung tissue. When resection is required, we attempt to preserve as much parenchyma as possible. In cases of bilateral metastases, we prefer a two-stage approach. The alternative, represented by sternotomy, has the disadvantage that the inferior lobes are not as accessible to palpation. A double-lumen tube cannot be used in children under 6 years of age. Compression of the lungs, however, facilitates surgical procedures on aerated lungs.

We endorse the use of thoracoscopy in children as has been reported by several research groups [3,9]. Our experience in this area is limited. We see the indication for thoracoscopic procedures in obtaining biopsy specimens for histologic study and for treatment of recurrent pleural effusions. Because palpation of the lung is not possible, we do not endorse its use in oncological patients.

5.4. Pulmonary function tests and postoperative quality of life
A survey of 24 children regarding their quality of life revealed almost no limitation in the children evaluated. The same holds true for pulmonary function assessed preoperatively and on postoperative days 30 and 60. Complete findings are available for 16 children. There was no significant reduction in function despite the fact that some children had undergone multiple atypical resections. Therapeutic procedures, such as decortication, actually resulted in improvements in pulmonary function. Thoracic surgery in children appears to be tolerated with no significant restrictions in pulmonary function or quality of life.

5.5. Conclusion
The classification of thoracic surgical procedures into five groups (oncology, immune defect, infection, malformation, trauma) is useful and permits adequate comparability of the children treated. If the correct diagnosis is established, precise preoperative studies are obtained and the surgical procedure is adapted to the specific requirements of the pediatric patient; thoracic surgery in children with underlying benign disease is associated with a low rate of complications and good chances of cure with a rapid convalescence. Metastasectomy is an important surgical technique in selected patients as a component of an overall oncologic therapy concept.

	References

Top Abstract 1. Objective 2. Patients and method 3. Results 4. Postoperative course 5. Conclusion References

 

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Ludger Sunder-Plassmann Permission Requests Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Kanngiesser, P. Articles by Sunder-Plassmann, L. Search for Related Content PubMed PubMed Citation Articles by Kanngiesser, P. Articles by Sunder-Plassmann, L. Related Collections Lung - cancer Lung - other Lung - basic science