Michèle Larroquet

Carcinoid and mucoepidermoid bronchial tumours in children

The aim of the study was to determine the characteristic features and outcome of carcinoid or mucoepidermoid tumours in children. A retrospective analysis of all patients treated for a carcinoid or mucoepidermoid tumour in France between 1984 and 2001 was performed. There were 11 cases of carcinoid tumour and 6 cases of mucoepidermoid tumour. The mean age of the patients was 10.5±3.0 years, with a range of 5 to 15 years. Twelve and 6 patients presented with evidence of bronchial obstruction and haemoptysis, respectively. Fibre optic bronchoscopy confirmed the presence of a bronchial tumour in all cases and endobronchial biopsies were diagnostic in 11 of 12 cases. A chest CT scan revealed the presence of a hypervascular tumour in 8 of 12 patients. The distribution of the location of the tumours was equal between the right and the left lung, and, in 9 cases, the airways were totally occluded by the tumour. Complete surgical resection (lobectomy in 15 patients and pneumonectomy in 2 patients) was performed in all cases without pre-operative chemotherapy or radiotherapy. The mean duration of follow-up was 4.0±3.0 years. In 2 patients, auscultation assymetry and an episode of haemoptysis revealed the recurrence of a mucoepidermoid tumour, successfully cured by removal of the tumour and chemotherapy and radiotherapy in one child. No death was observed. Conclusion:Pulmonary carcinoid and mucoepidermoid tumours are rare in children. Bronchoscopic removal should not be performed. With aggressive surgical therapy, the prognosis is excellent. Fibre optic bronchoscopy confirms the presence of an endobronchial mass. A biopsy is needed for diagnosis and complete surgical removal is the treatment of choice. Long-term results are excellent but a clinical follow-up is recommended.

Intraoperative localization of neuroblastoma in children with 123I- or 125I-radiolabeled metaiodobenzylguanidine

BACKGROUND This study describes a novel method of intraoperative localization of neuroblastoma with a gamma-detecting probe, to detect in situ tumor binding of radiolabeled 123I- or 125I-metaiodobenzylguanidine (MIBG) and improve the quality of tumor resection. METHODS Fifty-eight children underwent 66 surgical procedures with intraoperative detection of radiolabeled MIBG. All patients with positive MIBG scintiscans at diagnosis were included in the study. A tumor/background ratio exceeding 2:1 at the time of operation was considered positive, indicating a significant uptake of MIBG, compatible with the presence of malignant cells. The surgeons were requested to evaluate the contribution of the method to the surgical procedure. Sensitivity and specificity of the method with either 123I-labeled MIBG or 125I-labeled MIBG, on the basis of correlations between probe findings and pathologic analysis of 288 resected specimens, were determined. RESULTS Intraoperative detection was helpful in 65% of surgical procedures, allowing a better definition of tumor limits and extension to locoregional nodes or detection of small and nonpalpable tumors in sites with difficult surgical access, especially during operation for relapse. The detection was not contributory in 35% of the procedures (well-localized tumors, thoracic neuroblastoma for technical reasons, highly differentiated tumors as ganglioneuroma, and tumors with mainly necrosis or fibrosis). The sensitivity of 123I and 125I was the same (91% and 92%), but the specificity of 125I (85%) was significantly higher than that of 123I (55%) (p < 0.005). CONCLUSIONS First, this study demonstrates the feasibility of intraoperative detection, with radiolabeled MIBG, of neuroblastoma in children. We advocate the use of 125I rather than 123I. Second, the method is useful to improve the quality of macroscopic resection in widespread neuroblastoma with nodal involvement, in sites with difficult access, and in operations for relapse.

Comparison between US and MRI in the prenatal assessment of lung malformations

BackgroundThe contribution of MRI in the prenatal evaluation of congenital lung abnormalities (CLA) has not been extensively investigated.Objective(1) To compare diagnostic accuracy and assessment of prognostic factors between US and MRI in CLA and (2) to assess the diagnosis agreement between prenatal imaging and postnatal diagnosis.Materials and methodsWe included 23 consecutive fetuses who underwent concomitant US and MRI during gestation as well as postnatal CT and surgery (n = 22).ResultsUS-MRI sets were performed at median gestational age of 26 (n = 16) and 34 (n = 22) weeks. Postnatal diagnoses were 11 congenital pulmonary airway malformations (CPAM), 4 bronchopulmonary sequestrations (BPS), 6 hybrid lesions and 2 cysts. US and MRI agreement was significantly better during the second trimester than during the third one (P = 0.02). Disagreements were related to missed cysts (n = 5), mediastinal shift (n = 6) and vessels (n = 5). US and MRI diagnosis agreement was present in 20 cases, including 5 cases of misdiagnosis. US and MRI were concordant with postnatal diagnosis in 17 and 16 cases, respectively.ConclusionIn our series, no clear superiority of MRI over US in the prenatal evaluation of CLA was demonstrated, but US better demonstrated systemic feeding vessels and MRI cysts and normal lung adjacent to the lesion.

Laparoscopic diagnosis of contralateral patent processus vaginalis in children under 1 year of age with unilateral inguinal hernia: Comparison with herniography

PURPOSE The effectiveness of laparoscopic diagnosis of contralateral patent processus vaginalis (CPPV) in children with unilateral inguinal hernia was evaluated. METHODS Ninety-three consecutive children under the age of 1 year were operated on for a unilateral inguinal hernia. A contralateral CPPV was diagnosed by laparoscopy via the inguinal hernia sac before ligation. The laparoscopy results of this technique were correlated with those of herniography or inguinal exploration. RESULTS Laparoscopy was performed on 88 patients; sensitivity was 71% and specificity 89%. The only complication arising from the procedure was wound infection in two patients. CONCLUSION This method is a simple, safe, and accurate procedure for selecting children for contralateral surgical exploration.

Pleuroperitoneal shunt in the management of chylothorax caused by thoracic lymphatic dysplasia

Three cases of intractable chylothorax secondary to thoracic lymphatic dysplasia were treated by pleuroperitoneal shunt insertion. These cases included one with Gorhams syndrome, and one case with a bilateral chylothorax and chylous ascites. Pleuroperitoneal shunts allowed an adequate internal drainage in all cases, alleviating protein and lymphocyte losses caused by recurrent pleural taps. In the third case with chylous ascites, valved shunts were used to avoid reflux between the peritoneal cavity and the pleural space. Such palliative therapy did not change the bad prognosis of these patients with lymphatic disorders but improved the childrens quality of life.

An idiopathic congenital chylothorax: surgery or conservative management?

A 3-month-old infant was admitted to the respiratory unit for dyspnoea and vomiting after her second DTaP-Polio vaccine shot. The chest X-rays showed a white right lung with a left mediastinal shift. A pleural aspiration assessed the diagnosis of chylothorax. A conservative treatment was initiated with a fat-free diet and pleural aspirations. As this treatment was ineffective, a total parenteral nutrition was started at day 11, plus increasing doses of Octreotide. As the chylothorax persisted at day 50, a pleuroperitoneal shunting was performed but a pleurodesis was finally necessary. The child was discharged from the hospital 6 weeks after the surgery. Ten months later, her physical and biological conditions were normal and her chest X-rays dramatically improved. This case highlights the difficult management of infant chylothorax. Although conservative treatment has to be tried first, surgical procedures as pleuroperitoneal shunting and rarely pleurodesis have to be discussed.

CL086 - Chirurgie des néphroblastomes associés à de la néphroblastomatose (24 patients)

La nephroblastomatose (NBL) s’associe souvent a des nephroblastomes (WT). En 20 ans (12/1989-02/2010-Protocoles SIOP9, 93 et 2001), 24 enfants ont ete pris en charge en Chirurgie Viscerale pour NBL. La NBL etait bilaterale (19) ou unilaterale (5). L’indication chirurgicale etait portee uniquement en cas de WT, certain ou suspect, associe a la NBL, soit d’emblee (26 reins), soit lors de la surveillance (10 reins : 6 masses stables et 4 apparues ou progressant). Les 24 enfants ont ete operes d’un seul (12) ou des 2 reins(12). Sur les 36 reins operes, une chirurgie conservatrice(CC) a ete realisee pour 27 (75 %); il s’agissait histologiquement de 7 nodules de nephroblastomatose isolee sans WT et de 29 WT (7 bas risque, 15 intermediaire et 7 haut). Avec un suivi median de 61/2 ans (8 mois –17 ans), une recidive loco-regionale puis metastatique est survenue chez 2 enfants (8 %) apres CC, dont un haut risque blastemateux, decedes. Une insuffisance renale moderee (1) et/ou une HTA (2) etaient presentes dans 2 cas. Conclusion L’incidence du WT dans l’evolution de la NBL est elevee dans notre experience de centre chirurgical. Les masses apparaissant ou progressant sous chimiotherapie sont fortement suspectes de WT. La CC a ete possible dans 75 % des cas avec un taux de recidive de 7 %.

SFCP-004 – Urologie – Chirurgie des néphroblastomes associés à de la néphroblastomatose (23 patients)

La nephroblastomatose (NBL) est un processus proliferatif qui s’associe volontiers avec des nephroblastomes bilateraux. La SFCE a propose une approche therapeutique homogene combinant chimiotherapie et chirurgie. La chimiotherapie etait systematique et prolongee, et la chirurgie indiquee seulement en cas de nephroblastome, certain ou suspect, sur les donnees de l’imagerie, lors du diagnostic ou de la surveillance. Cette etude retrospective a pour but d’evaluer les indications et les techniques chirurgicales dans l’evolution de la NBL. Patients En 29 ans (01/1979-12/2007), 23 enfants ont ete pris en charge pour NBL, 10 garcons et 13 filles avec un âge median de 12 mois (6 mois–5 ans). Il s’agissait d’une NBL bilaterale (20) ou unilaterale (3). L’indication chirurgicale etait portee uniquement en presence d’un nephroblastome, certain ou suspect, associe a la NBL, soit d’emblee au moment de la prise en charge initiale (29 reins), soit lors de la surveillance (6 reins : 2 masses stables pendant la chimiotherapie et 4 apparues ou progressant apres la chimiotherapie). Resultats Sur 35 reins operes, une chirurgie conservatrice a ete realisee pour 27 reins (77 %) et une nephrectomie totale pour les 8 autres. Les 23 enfants ont du etre operes, d’un seul rein (11 enfants) ou des 2 reins (12); il s’agissait histologiquement de 31 nephroblastomes et 4 nodules residuels de restes nephrogeniques. Parmi les nephroblastomes, 7 etaient de haut risque, 18 de risque intermediaire et 6 de bas risque. Avec un suivi median de 4,5 ans (4 mois–15 ans), une recidive est survenue chez 6 enfants (26 %) dont 4 haut risque et 2 intermediaire, tous apres chirurgie conservatrice. Une recidive locale isolee est survenue chez 3 enfants et une recidive avec metastases chez 3 autres. Quatre enfants sont decedes (debut de traitement avant 1983 pour 3). Une insuffisance renale moderee (1) et/ou une hypertension arterielle (2) etaient presentes dans 2 cas. Conclusion L’incidence du nephroblastome dans l’evolution de la NBL est elevee dans notre experience de centre chirurgical. Les masses apparaissant ou progressant sous chimiotherapie sont fortement suspectes de nephroblastome. La recidive parait frequente apres chirurgie conservatrice dans les nephroblastome de haut risque.