Multicystic and diffuse malignant peritoneal mesothelioma in children.

BACKGROUND: Malignant and multicystic peritoneal mesotheliomas are extremely rare tumors in children, developing from mesothelial cells. No specific guidelines are available at this age. METHODS: We performed a retrospective analysis of all identified children (< 18-year-old) treated in France from 1987 to 2017 for a diffuse malignant peritoneal mesothelioma (DMPM) or a multicystic peritoneal mesothelioma (MCPM). RESULTS: Fourteen patients (5 males and nine females), aged 2.2 to 17.5 years, were included. The most frequent presenting symptoms were abdominal pain, ascitis, and alteration in the general condition. Eight patients had epithelioid mesothelioma, three had biphasic mesothelioma, and three had MCPM. Eight patients with DMPM diagnosis received cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC). Among them, six patients had neoadjuvant systemic chemotherapy, one patient, post-operative chemotherapy, and one patient CRS and HIPEC only. Three patients received only systemic chemotherapy. All patients with MCPM had only surgery. After a median follow-up of seven years (2-15), six patients (6/11; one death) with DMPM and two patients (two/three) with MCPM had a local and distant recurrences. CONCLUSION: Peritoneal mesothelioma in children is a rare condition with difficult diagnosis and high risk of recurrence. Worldwide interdisciplinary collaboration and networking are mandatory to help diagnosis and provide harmonious treatment guidelines.

Technical standardization of laparoscopic repair of Morgagni diaphragmatic hernia in children: results of a multicentric survey on 43 patients.

BACKGROUND: This study aimed to standardize the surgical correction technique of congenital Morgagni diaphragmatic hernia (CMDH), analyzing the results of an international multicentric survey. METHODS: The medical records of 43 patients (29 boys, 14 girls) who underwent laparoscopic repair of CMDH in 8 pediatric surgery units in a 5-year period were retrospectively reviewed. Their average age was 3.3 years. Ten patients (23.2%) presented associated malformations: 9 Down syndrome (20.9%) and 1 palate cleft (2.3%). Thirty-five patients (81.4%) were asymptomatic, whereas 8 patients (18.6%) presented symptoms such as respiratory distress, cough or abdominal pain. As for preoperative work-up, all patients received a chest X-ray (100%), 15/43 (34.8%) a CT scan, 8/43 (18.6%) a barium enema and 4/43 (9.3%) a US. RESULTS: No conversion to open surgery was reported. Average operative time was 61.2 min (range 45-110 min). In 38/43 (88.3%) patients, a trans-parietal stitch was positioned in order to reduce the tension during the repair. In 14/43 cases (32.5%), the sac was resected; in only 1/43 case (2.3%) a dual mesh of goretex was adopted to reinforce the closure. Average hospital stay was 2.8 days. The average follow-up was 4.2 years, and it consisted in annual clinical controls and chest X-ray. We recorded 2 complications (4.6%): one small pleural opening that required no drain and one recurrence (2.3%), re-operated in laparoscopy, with no further recurrence. CONCLUSIONS: To the best of our knowledge, this is the largest series published in the literature on this topic. Laparoscopic CMDH repair is well standardized: The full-thickness anterior abdominal wall repair using non-resorbable suture with interrupted stitches is the technique of choice. Postoperative outcome was excellent. Recurrence rate was very low, about 2% in our series. We believe that children with CMDH should be always treated in laparoscopy following the technical details reported in this paper.

Indications for laparoscopy in the management of intussusception: A multicenter retrospective study conducted by the French Study Group for Pediatric Laparoscopy (GECI).

INTRODUCTION: Surgical management of intussusception is required for cases where enema reduction fails. Some articles report an advantage of the laparoscopic over the open approach, but complications such as serosal tearing and frank perforation have been described. We aim to determine the best indication of laparoscopy in the decisional management tree of intussusception. PATIENT AND METHOD: This is a retrospective chart review of all children with intussusception who failed hydrostatic enema reduction and who underwent immediate laparoscopic management. Cases were compiled from 7 pediatric surgical centers (French Study Group of Pediatric Laparoscopy) between 1992 and 2005. Data collected included age, duration of symptoms, findings on initial assessment, level of intussusceptum after attempted hydrostatic enema reduction, type of laparoscopic approach, operative time, conversion to open surgery, etiology, and postoperative complications. Two groups were analyzed-conversion to open surgery or not. RESULTS: Sixty-nine patients (48 males and 21 females) were reviewed. In total, 22 patients required a conversion to open surgery (31.9%). Eleven of these were converted because of failure of laparoscopic reduction. The risk for conversion to open surgery is directly linked to the length of time between onset of symptoms and diagnosis (1.6 vs 3.1 days for conversion group, P = .048), the presence of signs of peritonitis on the initial clinical assessment (10.6% vs 41% in conversion group, P = .003), and the presence or absence of a pathologic lead point (17% vs 50% in conversion group, P = .004). CONCLUSION: The child seen early after the onset of symptoms (<1.5 days) with no signs of peritonitis is the best candidate for a laparoscopic approach in management of intussusception requiring surgery. Particular attention must be paid to not miss a pathologic lead point, especially as most tactile cues are lost.