Ana Lourdes Luis

Objectives: To assess the efficacy of thoracoscopy and the outcome for children with thoracic neurogenic tumors. Methods: We performed a retrospective review of 15 European centers between 2000 and 2020 with patients who underwent thoracoscopy for a neurogenic mediastinal tumor. We assessed preoperative data, complications, and outcomes. Results were expressed with the median and range values. Results: We identified 119 patients with a median age of 4 years old (3 months–17 years). The diameter was 5.7 cm (1.1–15). INRG stage was L1 n = 46, L2 n = 56, MS n = 5, M n = 12. Of 69 patients with image-defined risk factors (IDRF), 29 had only (T9–T12) locations. Twenty-three out of 34 patients with preoperative chemotherapy had an 18 mm (7–24) decrease in diameter. Seven out of 31 patients lost their IDRF after chemotherapy. Fourteen had a conversion to thoracotomy. The length of the hospital stay was 4 days (0–46). The main complications included chylothorax (n = 7) and pneumothorax (n = 5). Long-term complications included Horner’s syndrome (n = 5), back pain, and scoliosis (n = 5). Pathology was 53 neuroblastomas, 36 ganglioneuromas, and 30 ganglioneuroblastomas. Fourteen had a postoperative residue. With a median follow-up of 21 months (4–195), 9 patients had a recurrence, and 5 died of disease. Relapses were associated with tumor biology, histology, and the need for chemotherapy (p = 0.034, p = 0.04 and 0.020). Conclusion: Our results show that thoracoscopy is safe, with good outcomes for thoracic neurogenic tumors in selected cases. Surgical outcomes are related to the IDRFs, whereas oncologic outcomes are related to tumor histology and biology.

Infants and rats with congenital diaphragmatic hernia (CDH) have malformations of the heart and the great arteries caused by neural crest (NC) dysregulation during embryogenesis. Abnormally narrow jugular veins have been found in babies during cannulation for ECMO. However, the venous system has not been examined in depth so far. We hypothesized that abnormal patterning and/or size of the thoracic veins could occur in rats with CDH. This hypothesis was tested by microscopic magnetic resonance imaging (MMRI), a high-resolution tool able to detect subtle changes of vessels in small animals. Fetuses from pregnant rats fed either 100 mg i.g. nitrofen or vehicle on E9.5 were recovered near term. A 7 T MMRI system with a coronal multislice fast spin echo sequence allowed diagnosis of CDH (n = 19), and T2 SE high-resolution sequences made assessment of the pattern and width of cervico-thoracic veins possible. Values were corrected for body size by dividing them by the length of thoracic vertebrae T3–T5. The results in nitrofen and control (n = 11) groups were compared by non-parametric tests (*p < 0.05). Congenital diaphragmatic hernia fetuses were smaller than controls (4.5 ± 0.26 vs. 5.3 ± 0.2 g*). The widths (corrected for body size) of left external, both innominate, right superior vena cava and azygos veins were significantly smaller in CDH rats than in controls. The cervico-thoracic veins are normally patterned but abnormally narrow (except the internal jugulars) in rats with CDH. The same embryonic NC dysregulation that accounts for cardiovascular malformations could also explain these venous anomalies in CDH.

The research on congenital diaphragmatic hernia (CDH) is often carried out on the nitrofen fetal rat model in which most investigations involve microdissections and fastidious assessment of serial sections of different anatomic areas. Current microscopic magnetic resonance (MMR) equipment allows detailed anatomic studies of alive, fresh or fixed fetuses. The purpose of the present study was to demonstrate that CDH itself and most of the associated malformations are adequately imaged and measured by MMR. Fetuses from pregnant rats treated with either i.g. vehicle (control, n = 10) or 100 mg nitrofen (only those with CDH, n = 18) on E9.5 were recovered on E21 (term = E22) and total body was scanned by MMR under sedation in a 7 T MRI system (Bruker Medical, Ettlingen, Germany). CDH was detected with a coronal multislice fast spin echo sequence with a long repetition time and short effective echo time. Oblique MPR and 3D reconstructions were used. All studies were processed with attention to the hernia and its contents and the structure of the tracheobronchial tree and the lung, the heart and great vessels, the thymus and cervico-thoracic vertebrae. The findings in both groups were compared. Congenital diaphragmatic hernia, lung hypoplasia and parenchymal features were clearly depicted. Tracheal ring anomalies were also demonstrated. The thymus was significantly smaller in CDH pups (2.9 × 1 × 2.4 mm) than in controls (4 × 1.3 × 2.8 mm) (p < 0.01). MRI was particularly performant for imaging cardiovascular anomalies: 4 double aortic arches, 3 Fallots, 3 right aortic arches, 3 ventricular septal defects and 1 aberrant subclavian artery. Microscopic magnetic resonance involves refined and expensive equipment but it provides a powerful research tool for the study of CDH and other malformations in rat fetuses. Further work on this area is warranted.

Aim: Aim of this study was to assess the long-term results of the treatment of total colonic aganglionosis (TCA) with 2 different techniques in terms of growth, continence and quality of life (QOL). Patients and Methods: Forty-one patients treated for TCA between 1972 and 2007 were reviewed retrospectively with special attention paid to the length of aganglionosis, complications, growth, continence and QOL. Until 1992, patients underwent subtotal colonic resection and side-to-side ileosigmoid anastomosis (modified Martin). Since 1992, straight ileo-anal pull-through was preferred. At the end of follow-up (median 18 years, range 1–35), the height and weight, continence and QOL (scoring feeding habits, school/work performance, family life and professional development) were assessed by clinical visit or phone interview. Results: Twenty-eight patients were male and 13 female. Six had total intestinal aganglionosis and were excluded from this review together with the 2 who died before definitive treatment. The 33/41 persons in whom aganglionosis had involved less than 50 cm above the ileocecal valve and who had been considered suitable for the reestablishment of transanal fecal flow were included. Eighteen children underwent a modified Martin and 15 straight ileo-anal pull-through. Postoperative intestinal obstruction occurred in 4 cases, prolapse and prolonged TPN requirement in 2, and wound disruption and fistula in 1. Thirteen patients (39 %) had postoperative enterocolitis. Two children died after operation (1 wound disruption with sepsis and 1 pneumonia). Out of 31 survivors, 57 % and 53 % were > p50 with regard to height and weight whereas only 15 % and 19 % were Conclusions: Patients with TCA amenable to reestablishment of the transanal fecal flow can have adequate growth, normal feeding, reasonably good continence and satisfactory QOL. However, complications and enterocolitis are frequent. A modified Martin's procedure was performed as well as straight ileo-anal pull-through with little influence on the long-term outcome.