Purpose: To consider the strategy of surgical treatment for patients with intestinal failure (IF) by evaluating our experiences retrospectively.

Method: Eight patients with IF, who were referred to our institute as candidates for cadaveric intestinal transplantation (ITx) between April 2010 and April 2016, were included in this study. Their data were gathered from their charts and analyzed.

Results: Their primary diseases were total colon aganglionosis (n = 1), chronic idiopathic intestinal pseudo-obstruction syndrome (CIIPS) (n = 2), and short bowel syndrome (SBS) (n = 5). The causes of SBS were SMA embolization, graft loss after ITx, necrotizing enterocolitis (NEC), malrotation, and ulcerative colitis with primary sclerosing cholangitis. Two patients died of liver failure. On two patients among the remaining six, we performed enteroplasty/STEP, and four of the six were registered as candidates for ITx. On one of the registered patients, we performed ITx. On another of the candidates, we performed balloon enteroplasty for the stricture of the remaining bowel detected after being registered, and the patient was removed from the waiting list after the surgery. The remaining two are still waiting for transplantation. The lengths of the remaining intestine were less than 15 cm in the fatal cases.

Conclusion: Patients with the residual small bowel of less than 15 cm had poor outcomes owing to the acute deterioration of liver function. (1) Ultra-SBS, (2) severe IFALD, and (3) loss of access venous routes seem to be definitive factors for becoming a candidate for ITx.

A 13-year-old boy presented with complaints of abdominal pain for two days and bloody stool for one month. The patient underwent Computed Tomography (CT) and diagnosed as having intussusception (ileum colon type), and was referred to our hospital. The mass lesions found on CT images were considered to be the leading point of intussusception. The patient underwent open reduction surgery to clarify the leading point of intussusception. There was no sign of intestinal necrosis and it was easily redacted using Hutchinson procedures. There were several large lymph nodes (LNs) in the mesentery of the terminal ileum with a 20 mm LN at the leading point of intussusception. Bloody stool was observed on postoperative day 1, but no recurrence of intussusception was found by abdominal ultrasonography. The patient underwent colonoscopy on postoperative day 4, which revealed the presence of ulcerative colitis. Intussusception usually occurs in patients under 1 year of age and is very rare in patients over 6 years of age. These patients usually have an organic leading point of their intussusceptions. Even then, intussusception caused by ulcerative colitis in older children is very rare and reports of such cases are difficult to find.

Swyer-James syndrome (SJS) is a rare condition characterized by increased permeability of one pulmonary or lung lobe and loss of pulmonary blood vessel shadow in chest X-ray. Recently, the number of case reports of bilateral or patchy lesions have been increasing owing to the development of computed tomography (CT). We encountered a case of bilateral SJS in a 7-year-old boy and performed thoracoscopy-assisted pulmonary resection. The boy, who had histories of repeated pneumonia, bronchitis, asthma, and infection of a left apical lung cyst, was referred to our hospital. Chest CT showed lobar emphysema with multiple cysts in the left upper lobe and complete atelectasis in the left lower lobe, as well as whole expansion and patchy emphysema in the right lung, in addition to obstruction of the peripheral bronchus in the left upper lobe and narrowing of the left pulmonary vessels. Therefore, we diagnosed him as having SJS. We performed thoracoscopy-assisted partial resection of the left upper lobe and left lower lobectomy, considering ventilation failure due to recurrent cyst infection or shunt blood flow in complete atelectasis. He was discharged on the 15th postoperative day, and no rehospitalization due to respiratory tract infection or asthma attack occurred until 10 months after surgery. We should consider SJS in cases of repeated respiratory infections from the infantile period, and then consider lung resection if a patient suffers from infectious cyst formation or respiratory distress, even in childhood.

The patient was a 11-year-old girl who started vomiting from about 8 years of age. Since postprandial vomiting persisted after 10 years of age, she was examined thoroughly. Delayed esophageal emptying of contrast medium was noted on esophagography, and the integrated relaxation pressure (IRP) of the lower esophageal sphincter was high (63.8 mmHg) on high-resolution manometry. On the basis of these findings, the patient was diagnosed as having esophageal achalasia. The preoperative Eckardt score was 6. We performed peroral endoscopic myotomy (POEM), a new treatment for esophageal achalasia that has had favorable outcomes in adults. A submucosal tunnel was prepared from the 5-o’clock direction of the esophageal body. The external longitudinal muscle layer was conserved, and a 16 cm incision was selectively made in the inner circular muscle. Here subjective symptoms rapidly disappeared after surgery, and her Eckardt score and IRP improved to 0 and 17.7 mmHg, respectively. A proton-pump inhibitor (PPI) was administered for one month after surgery. At 3 months after surgery, mild esophagitis of Los Angeles classification A was observed on upper digestive tract endoscopy, but regurgitation symptoms were absent and her overall condition was favorable with a body weight gain of 2 kg.

It is difficult to obtain a satisfactory outcome in the repair of umbilical hernia with excess skin, because the excess tissue disturbs the satisfactory formation of a depression of the umbilicus. We performed a new surgical technique in three pediatric patients with such umbilical hernia. The technique consists of the following procedures: (1) The sac of the umbilical hernia was resected and the excess tissue under the skin around the umbilicus was trimmed off as thoroughly as possible, (2) the excess skin fold of the bottom of the umbilicus was then resected in a circular pattern and the defect in the skin was closed by purse-string suture, (3) the newly created bottom of the umbilicus was firmly anchored in the caudal rectus abdominis fascia by the same string of the purse-string suture to obtain a well-depressed umbilicus. One of these patients had a urachal remnant treated laparoscopically via the umbilical approach, and then the umbilical hernia was repaired by the method above. As the skin blood perfusion of the newly created bottom of the umbilicus was well maintained, there were no complications including skin necrosis or surgical site infection. The form of the umbilicus has remained in the well-depressed position more than 1 month after the operation. In addition, this new technique can be applied to patients with other diseases requiring surgery via the umbilical approach.

Internal hernia due to a congenital mesenteric defect can lead to intestinal obstruction or necrosis caused by strangulation and, in severe cases, to death as it has no hernia sac and allows a large portion of the small intestine to pass through its defect. However, the diagnosis has been considered to be difficult and the only way to confirm the diagnosis is via laparotomy. We experienced treating a case of a 7-year-old girl without any previous medical history, who was diagnosed as having a strangulated ileus due to internal hernia caused by a huge congenital mesenteric defect. She was referred to our hospital presenting with abdominal pain, vomiting and hyperglycemia (almost 600 mg/dl), and was suspected of having diabetic ketoacidosis. After some testing including abdominal CT scan with contrast, her above-mentioned diagnosis was confirmed and she underwent emergency surgery. During the operation, a mesenteric defect as huge as 15 cm in diameter was found over 235 cm of her small intestine, through which almost her entire small intestine passed. As a result of this condition, her intestine twisted and became necrotic. Therefore, we performed partial resection of the small intestine with ileocecal valve. The length of the residual intestine was 150 cm. After the surgery, she suffered from mild and frequent diarrhea for a while, but the symptom improved gradually and she can now eat sufficiently to maintain her body weight.

We report the case of a 16-year-old boy with trisomy 21. He underwent duodenojejunostomy for congenital duodenal atresia (apple-peel type) with annular pancreas at 12 days of age and hepaticojejunostomy for pancreaticobiliary maljunction at the age of 4 years. Frequent cholangitis and multiple intrahepatic bile duct stones occurred at the age of 14. Hepaticojejunostomy stenosis was suspected, and laparotomy was performed. Cholangiography revealed no hepaticojejunostomy stenosis. Hepaticojejunostomy was re-anastomosed for a wider anastomosis after intrahepatic cholangiolithotomy. At the age of 16, frequent cholangitis and liver damage occurred. Although CT and MRI revealed no cause of cholangitis, hepatobiliary scintigraphy revealed cholestasis in the loop of the jejunum; thus, dysfunction of the Roux loop was diagnosed. We performed partial resection of the loop and Roux-en-Y duodenojejunostomy for bile excretion. The clinical course of his illness was satisfactory after the operation. Evaluation of the function of the Roux loop is necessary to prevent cholangitis in the long-term follow-up.

An 11-year-old boy was suspected of having right scrotal hydrocele by his previous doctor because of continuous scrotal swelling. However, it was not a scrotal hydrocele but a testicular mass (45 × 25 × 23 mm, elastic hard). Ultrasonography showed small cysts clustered in the right testicular parenchyma. By MRI, multilocular cystic lesions were also found in the same part. Right renal aplasia and compensatory swelling of the left kidney were also observed. We suspected cystic dysplasia of the rete testis (CDRT) on the basis of the site, morphology, and characteristic urinary tract malformations, and performed mass nuclear exclusion. Histopathological findings of the excised specimens also showed that there were no heterotypic epithelial cells and that part of the normal testis network had expanded to the cystic lesions. It was a finding consistent with CDRT. The definitions of CDRT are unclear and there are few reports. Since it is derived from the rete testis, there is concern about the impairment of postoperative sperm-transporting ability, but in this case, it was expected that hormone productivity can be maintained by selecting tumor nucleus surgery. Currently, two and a half years have passed since the operation, but there has been no recurrence. It is considered that follow-up while taking note of recurrence is necessary in the future.

We report a case of a 0-day-old boy with omphalocele as determined by ultrasonography in the 24th week of gestation. He was born by vaginal delivery in the 35th week of gestation, and was subjected to siloplasty for the omphalocele. Microcalcification and marked intestinal adhesion were observed in the omphalocele sac, but the perforated site could not be specified and no fresh meconium leakage was seen. Therefore, the perforated site might have closed naturally. The silo was plicated daily after surgery, and a bilelike effluent was seen in the silo on day 4. Because reperforation of the gastrointestinal tract was suspected, a 2nd operation was performed on day 6. During the 2nd operation, a perforated Meckel’s diverticulum was observed, and wedge resection of Meckel’s diverticulum and silo replasty were performed. The postoperative course was uneventful, and abdominal wall closure was carried out on day 13. In the neonatal period, there are few cases of Meckel’s diverticulum perforation, and those associated with omphalocele are especially extremely rare. Furthermore, the clinical course of our patient was unique, given the reperforation in the neonatal period after natural closing in the fetal period.

A rare case of congenital intestinal atresia associated with Hirschsprung’s disease was described. A 0-day-old boy who was diagnosed as having small bowel atresia underwent intestinal resection with anastomosis. Postoperatively, he showed poor sucking of milk and abdominal distention. However, he soon recovered by enema and was discharged. In his one-month follow-up as an outpatient, he showed abdominal distention, and abdominal X-ray revealed dilation of the colon. Therefore, contrast enema was scheduled. However, before the examination, he required emergent hospitalization because of high fever due to enterocolitis. Then, he was diagnosed as having Hirschsprung’s disease on the basis of the findings of contrast enema and histopathological examination of rectal mucosa. At the age of four months, laparoscopy-assisted pull-through was performed and he had an uneventful postoperative course.

We report two cases of torsion of Meckel’s diverticulum (MD). Case 1: A 7-year-old boy was admitted owing to complaints of right lower quadrant pain and vomiting. Abdominal CT revealed fluid collection around a lumen structure similar to an appendix. We performed laparoscopic emergency surgery. Torsion of MD was found, and ileal resection including MD was performed laparoscopically. Case 2: A 6-year-old boy was admitted owing to complaints of sudden onset of abdominal pain and vomiting. Abdominal CT revealed dilation of the small intestine. Suspecting internal hernia, we performed laparotomy. An internal hernia through the twisted neck of MD was found. We detorsioned MD and performed ileal resection including the neck of MD. Torsion of MD at the neck is very rare. We herein describe our two experiences with pertinent bibliographical considerations.

A female infant was born 812 g in weight at 30 weeks of gestation. Breast milk feeding with Lactobacillus bifidus was initiated at 1 day of age. At 36 days of age, necrotizing enterocolitis developed and laparotomy was performed for intestinal perforations at 44 days of age. Multiple intestinal perforations were observed. The necrotic intestine was resected, and end-to-end anastomosis was performed, which resulted in about 50 cm in length of the residual small intestine. Breast milk was restarted 11 days after the operation, and the amount of breast milk was increased without problems. The general condition of the infant improved. At 90 days of age, however, diarrhea developed, and the milk was changed to MA-1 owing to suspected lactose intolerance. At 123 days of age, bloody stool, abdominal distension, and an increase in CRP level were observed. Milk allergy was suspected, and MA-1 was changed to an elemental formula, but no improvement was observed. After fasting and bowel rest, enteral nutrition was initiated, but the marked intestinal distension did not improve. Since gastrointestinal series confirmed the absence of intestinal obstruction, abnormal gas retention in the small intestine due to small intestinal bacterial overgrowth (SIBO) was considered, and metronidazole administration was initiated. After a few days, the amount of intestinal gas markedly decreased, and symptoms did not recur after the resumption of the elemental formula. In this patient, SIBO may have been caused by various factors such as poor intestinal peristalsis due to immaturity, necrotizing enterocolitis, or gastrointestinal allergy.

Stomal prolapse is a common complication of enterostomies. Re-enterostomy or fixation of prolapsed intestine is usually performed for stomal prolapse, but they are invasive for patients. In pediatric cases, stomas are often temporary, and thus nonsurgical treatment is sometimes selected. We report a case of stomal prolapse successfully managed with an ostomy pouch covered with a pantyhose. A 2-month-old boy with both trisomy 13 and tetralogy of Fallot had jejunostomy for hypoganglionosis on his birth day. He had been managed with mechanical ventilation because of heart failure. After extubation, stomal prolapse occurred. We tried to manage the patient with an ostomy pouch using a pantyhose and this treatment prevented stomal prolapse without intestinal damage until re-enterostomy. This method using a pantyhose affixed onto an ostomy pouch is easy to perform and is inexpensive. From our experience, this conservative method using a pantyhose may be very useful for the management of stomal prolapse.

We report a case of congenital segmental dilatation of the small intestine in a newborn. A 3,042 g boy was delivered vaginally at 39 weeks and 6 days of gestation. No abnormality was detected in his prenatal ultrasonography. Since he had abdominal distention, he showed poor oral intake from his birth, and his body weight decreased significantly in the first three days. Although abdominal radiography showed a dilatation of the intestine on his right upper abdomen, intestinal gas was also detected in the colon and rectum; therefore, congenital intestinal atresia was ruled out. An upper gastrointestinal tract contrast study showed a normal jejunum without dilatation, and a gastrografin enema showed a normal colon without any dilatation. A long tube was passed through the dilated area the following day to reduce the pressure of the dilated intestine. We diagnosed him as having congenital segmental dilatation of the small intestine from these findings. The dilated area of the intestine was resected, and his postoperative course was uneventful. Although congenital segmental dilatation of the small intestine is rare in congenital gastrointestinal diseases, it was possible to prepare sufficiently for surgery on this patient owing to a confirmed preoperative diagnosis.