Recurrent congenital diaphragmatic hernia: A single center experience.

Congenital Diaphragmatic Hernia (CDH) occurs in 1:4000 live births. It's morbidity and mortality rates are significant. Recurrence is one of the recognized complications occurring after a successful initial repair. Poorly known are the rate of recurrence and factors that may influence the re-herniation. Poorly described are its morphology and treatment. Medical records of 95 neonates with CDH who had undergone repair at tertiary pediatric teaching hospital between 2007-2018 were retrospectively reviewed. Prior to surgery, peri-operative and recurrence data were collected. The follow-up was 1 to 12 years after the initial repair. Recurrence occurred in 22/84 (26%) patients. In 5 patients we observed more than one recurrence, total number of 27 (32%) re-herniations; 16/22 (72%) recurrences occurred within first 12 months; 7/27 (26%) recurrences were symptomatic which gives the symptomatic recurrence rate 8.3%; 20/27 (74%) were asymptomatic. There were no significant differences between groups considering LHR, gestational age, birth weight, severity of pre-surgery and post-surgery stabilization period. Significantly higher Apgar scores were in the group without recurrence. Among subjects with recurrent CDH majority had a left-sided defect, no hernia sac, no liver herniation, diaphragm was primarily sutured, abdominal wall primarily closed. Even though none of those factors was significantly related with the recurrence. Recurrence in CDH remains a significant issue for long-term surgical morbidity, especially more apparent in the first year of life. As long as the risk factors of re-herniation remains unclear, the most important seems to be routine follow-up protocol allowing for CDH recurrence detection.

Solid pseudopapillary tumor of the pancreas (Frantz's tumor): two case reports and a review of the literature.

INTRODUCTION: Solid pseudopapillary tumor of the pancreas is extremely rare in children; it usually occurs in young women between 18 and 35 years of age. It comprises less than 3% of pancreatic tumors. It is of low malignancy; however, it may be locally aggressive. Surgical resection is the treatment of choice and its prognosis is excellent. CASE PRESENTATION: Two Caucasian girls, 15 and 12 years of age were diagnosed with tumor of the pancreas. The first patient had severe abdominal pain. In the second case the tumor was asymptomatic, detected incidentally during ultrasound. Computed tomography confirmed pancreatic mass. In the first case, apart from the tumor located in the head and the body of her pancreas, focal change in her right kidney was found, which was an indication to biopsy that confirmed solid pseudopapillary tumor. In the second patient the tumor was located in the body of her pancreas, with portal vein occlusion and well-developed collateral circulation. In the first patient a pancreatoduodenectomy (Traverso-Longmire) was performed; there was no mass in her right kidney. In the second case, distal pancreatectomy and splenectomy were performed. In both cases histopathology revealed solid pseudopapillary tumor resected radically. Our first patient's postoperative course was uneventful. In the second case, her postoperative course was complicated by necrosis of the remaining pancreatic head that needed pancreatoduodenectomy. Follow-up at 28 and 26 months revealed no evidence of tumor recurrence or metastases on magnetic resonance imaging. CONCLUSIONS: Typical radiological appearance of solid pseudopapillary tumor is an indication for surgery. The treatment of choice is tumor resection with sparing of pancreatic tissue. In one of our two cases we performed a preoperative biopsy because of an uncharacteristic mass in her right kidney. In our second patient, necrosis of her spared pancreatic head meant that we could not preserve pancreatic tissue. Our whole diagnostic process, treatment and possible complications analysis should be of interest and noteworthy not only to surgeons as the treatment of choice is radical resection, but also to pediatric oncologists because of differentiation from other pancreatic tumors in children.

Desmoid tumor of the pancreas: a case report.

INTRODUCTION: Desmoid tumor is a rare, benign, usually asymptomatic fibromatous lesion. The etiology is unknown and the diagnosis is based on histopathological examination. The treatment is complete resection of the tumor. Pancreatic desmoid tumor is extremely rare. In the literature there have been only 11 cases described, most of them as solid or solid-cystic masses. We report the case of a patient with an isolated cystic pancreatic desmoid tumor that is, to the best of our knowledge, the second reported case. CASE PRESENTATION: A 13-year old Caucasian boy presented with recurrent pain of two months' duration in the left hypochondrium of his abdomen. An ultrasound examination and computed tomography scan revealed the presence of a cystic mass located in his splenic hilum, tightly adjacent to the pancreatic tail. A splenic cyst was suspected. Operative findings showed a 10 x 10 cm cystic mass tightly connected to the pancreatic tail and left colonic flexure, adherent to the spleen, splenic vein and artery. Distal splenopancreatectomy with en bloc resection of the left colonic flexure was performed. Histological analysis confirmed that the resection was complete. The mass had infiltrated the pancreatic parenchyma. All tumor cells were positive for anti-beta-catenin staining characteristic for desmoid tumor. No abnormalities in the spleen and colon were found. CONCLUSIONS: Isolated sporadic pancreatic desmoid tumor with cyst formation is extremely rare and its diagnosis can be difficult, especially because of uncharacteristic symptoms and radiological findings, as in our patient. This case report should be of interest not only to surgeons, as the treatment of choice is radical resection, but also gastroenterologists, considering it is in close relation with familial adenomatous polyposis, and oncologists as the reason for differentiation with other pancreatic tumors.