Purpose: The incidence of congenital jejunal stenosis is about 5% of small intestinal atresia and stenosis. We report 4 cases with congenital jejunal stenosis treated in our institution, and review 16 cases previously reported on literature.
Methods: We reviewed the medical records of our 4 cases with jejunal stenosis: the perinatal findings and clinical courses were analyzed. Furthermore, the neonatal condition, age on diagnosis, site of stenosis, type of stenosis, process of diagnosis, and treatment methods were analyzed in 20 (our 4 and previously reported 16) cases.
Results of our 4 cases: Our findings revealed the following: Prenatal ultrasonography demonstrated intestinal dilatation in 1 case. There were no other perinatal problems. Age on diagnosis ranged from 21 days to 7 months, and only 1 case was diagnosed during the newborn period. Body weights on diagnosis were 2,794 g, (-2.8 SD), 6,685 g (-1.5 SD), 2,970 g (-3.6 SD) and 5,868 g (-2.1 SD), respectively. All cases presented with vomiting, and had been suffering from poor weight gain. Based on findings of the upper gastrointestinal (UGI) studies, they were diagnosed as having stenoses of the proximal jejunum. Operation was performed on all cases. Post-operative stricture developed in a site controlled by balloon dilatation. The long term prognosis is good.
Result of 20 cases: All cases indicated signs of stenosis at the upper jejunum. Most stenoses were membranous. While 10 cases had some episodes of neonatal vomits, only 3 of them were diagnosed during the neonatal period. Eleven cases were presented for persistent vomiting, upper abdominal distension, and poor weight gain during infancy. The UGI studies were performed on 13 cases, and duodenography on 2. One case was diagnosed with cine MR imaging. One case underwent endoscopic dilatation. Operation was performed on 13 cases, and no serious operative complication was reported.
Conclusion: Although jejunal stenosis is a congenital anomaly, many cases are diagnosed after the newborn period. In infants with persistent vomiting and poor weight gain, UGI studies or duodenography should be performed to rule out congenital jejunal stenosis.

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Purpose: To investigate the benefit of the single incision laparoscopic surgery (TANKO) in the treatment of pediatric appendicitis, we conducted a comparison study between TANKO and conventional three-port laparoscopic appendectomy (CLA).
Methods: Of 40 consecutive pediatric patients who underwent laparoscopic appendectomy for the last four years, 15 were performed with CLA, and the remaining 25 with TANKO including three methods of different concepts; transumbilical laparoscopy-assisted appendectomy, total laparoscopic appendectomy, and so-called hybrid TANKO. The severity of the disease, operative time, hospital stay, complications, and satisfaction of the scar, were retrospectively compared.
Results: The successful ratios were 100% in CLA and 88% in TANKO. There were four children with complicated appendicitis (CA) in the CLA group, while none in the successful TANKO group. The operative time of TANKO group was significantly shorter than that of CLA group; however, those treated by the total laparoscopic TANKO appendectomy needed a duration similar to that of CLA group. Although the significance disappeared when CA patients were excluded, hospital stay of the TANKO group was shorter than the CLA group. Most patients of both groups had no complications, and were satisfied with their scars, except one boy in the TANKO group who complained of reoperation for his deformed umbilics.
Conclusions: Our data suggest that TANKO appendectomy is applicable and useful in pediatric patients, and is not inferior to CLA. In TANKO, however, additional ports should be placed without hesitation in a difficult case such as CA, and it is necessary to pay enough attentions to prevent postoperative deformation of the umbilicus.

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Purpose: Recurrent tracheoesophageal fistula (RTEF) is an intractable complication in children, who receive treatment for esophageal atresia (EA) with tracheoesophageal fistula (TEF). We aimed to demonstrate the usefulness of biotissue patches in the treatment of RTEF.
Methods: Six patients presenting with RTEF after performed radical operation, underwent implantation by the biotissue patch between 2001 to 2011; these included 4 patients who received the operation for EA at our department and 2 patients who received the repeated operations for RTEF at other hospitals. The age at operation for the RTEF was between 4 months and 5 years 4 months.
Results: We performed the closing RTEF technique for six cases that used implantation of biotissue graft between the esophagus and trachea. Used biotissue grafts were pedunculate pleurae in 2 cases, pedunculate pericardia in 2 cases, a free pericardium in 1 case, a pedunculate omentum in 1 case, an isolated latae fascia and auricular cartilage in 1 case. In one patient, who underwent a previous open-heart surgery, RTEF was repeated. A small free pericardium graft was used unavoidably in the 2nd operation. The auricular cartilage was used for the tracheal side, and the free latae fascia was used for the esophageal side in the 3rd operation. There is no evidence of recurrence in any patients for 1 to 9 years in the follow-up.
Conclusion: In cases of intractable RTEF, the biotissue graft should be selected on the basis of the patient's medical history, surgical history, and degree of inflammation, and should be implanted as extensive as possible.

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Purpose: Inflammation in mild acute appendicitis may be relieved by conservative treatment, although recurrence remains a problem. We investigated the risk factors of recurrence with surgical specimens and follow-up ultrasonography (US) after conservative treatment of acute appendicitis.
Methods: The medical records of 126 consecutive patients with acute appendicitis treated conservatively between January 2005 and December 2010 were reviewed. We examined 1) pathology of the surgical specimens, and 2) followup US findings one month after conservative treatment.
Results: Recurrence was confirmed in 36 of 126 patients. The average period of recurrence was 258 days (range 13-1,505 days), and relapse occurred in 31 treated cases (86.1%) within one year. 1) Pathological findings showed stenoses of the appendix in 25 (69.4%), and swelling of the appendiceal tip in 18 (50.0%) treated cases. 2) Followup US after conservative treatment was established in 65 cases, in which 17 cases relapsed. The recurrence rate was significantly higher in patients who showed a swollen tip in US one month after discharge.
Conclusions: Recurrence of acute appendicitis after conservative treatment was higher in patients with stenosis of the appendix, and swelling of the appendiceal tip in the follow-up US.

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A case of intestinal malrotation with spontaneous pneumoperitoneum was reported. A 6-day-old male infant was born at 38 weeks gestation with a birth weight of 2,506 g. He was admitted to our medical center because of vomiting, and pneumoperitoneum was noted on X-ray and CT scans. Laparotomy revealed no gastrointestinal tract perforation, however intestinal malrotation was found. Ladds procedure was performed and the postoperative course was uneventful. A case of intestinal malrotation with pneumoperitoneum is rare, and it is suspected that pneumoperitoneum seems to have been induced by intestinal malrotation.

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A 9-year-old boy was referred to our institute due to vomiting and recurrent attacks of abdominal pain. Ultrasonographic examination showed intussusceptions. A contrast enema reduction revealed ileocecal intussusceptions; however, pathological lesion could not be detected. Intussusception recurred 4 times. At the third episode of intussusception, a contrast enema reduction revealed ileoileocecal intussusceptions; however, we could not detect a pathological leading lesion. Therefore, after the fourth contrast enema reduction, computed tomography examination with filing contrast material in the intestine was performed, and a shadow defect was revealed at the distal ileum. Laparoscopy assisted surgery was then performed. During the operation, a tumor mass palpable at the ileum, existed 50 cm above the ileocecal valve, and the mass was resected. Microscopic examination revealed a heterotopic gastric mucosa. Although no diverticular projection was observed in this case, vitello-intestinal remnant was strongly indicated because there was an attached mesodiverticular artery. We must keep in mind that the heterotopic gastric mucosa without Meckel's diverticulm may be a cause of intussusception, although it is rare. Furthermore, careful inspection and palpation are necessary to detect the lesion during operation.

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A boy was born at 33 weeks 6 days gestation by cesarean section with a birth weight of 2,210 g. His growth was unremarkable by breast feeding. On postnatal day 53, he had an acute abdomen with fever and free air on X-ray. Although emergency laparotomy was performed, the site of perforation was not found. He was doing well after operation; however, he was referred again on postnatal day 86 because of paralytic ileus. Although he responded well to conservative treatment, symptoms recurred on the day 5 after admission. Although milk-specific IgE and allergenspecific lymphocyte stimulation tests were negative, cow's milk allergy was suggested through a careful history taking. Thereafter, oral intake of cow's milk was strictly prohibited, and breast feeding without maternal intake of cow's milk followed. Thereafter, he fared well and was non-eventful. Milk allergy should be considered when an infant presents intestinal perforation of unknown origin.

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A colonic diverticulitis, which is rarely experienced during childhood, requires differential diagnosis with an acute abdomen, such as an acute appendicitis. We present a case of pediatric diverticulitis of the ascending colon diagnosed by the dynamic CT and treated by simple diverticulum excision. An 11-year-old girl complained of right lower abdominal pain. An ultrasonography revealed 40 × 30 mm size of low echoic lesion, with a fecalith-like structure in her lower right abdomen; findings suspected of periappendiceal abscess. Dynamic CT showed 50 × 30 mm size of tumor like lesion containing a fecalith at the mesentery side of the lower ascending colon with no swelling of the appendix. An urgent operation was performed under the diagnosis of ascending colon diverticulitis. Excision of diverticulum, including the surrounding granular tissue, was performed for a greenish yellow fecalith embedded in the membrane of intestinal tract. The postoperative course was uneventful, and was followed by barium enema 2 months after operation. Dynamic CT seemed to be useful for the diagnosis of colonic diverticulitis. Simple diverticulum excision is considered to be sufficient enough for patients with this disease, and intestinal tract excision should be avoided in a cases of solitary diverticulitis.

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We report our experience of thoracoscopic diaphragmatic procedures using Lapa-Her-Closure^TM under artificial pneumothorax in 2 cases.
Case 1 was a 9-month-old boy with right-sided diaphragmatic eventration. Three trocars were used. Under artificial pneumothorax, a Lapa-Her-Closure^TM needle with nonabsorbable suture was threaded through the grasped diaphragm, with two forceps to complete two-line continuous running and horizontal mattress sutures successfully.
Case 2 was an 8-month-old girl with right-sided Bochdalek hernia with a sac. Four trocars were used. At the dorsal side without muscle, the needle threaded through the supra- and infra-costal space and a knot was tied subcutaneously to anchor the muscle of hernia edge to the costal bone. A total of 9 sutures were needed to close the defect.
This procedure was simple, safe, cosmetically acceptable, and suitable for children with diaphragmatic disorders.

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Adrenocortical carcinoma (ACC) is rare in children. The prognosis of ACC is poor, especially in advanced disease stage. Herein we report a pediatric case of advanced ACC with pulmonary metastasis.
An 8-year-old boy with large abdominal mass was admitted to our hospital. Pubic hair and penile hypertrophy were observed on admission. A CT scan demonstrated a large tumor in the left adrenal grand and 3 lung nodules in the right upper lobe, right lower lobe, and left lower lobe. After tumor biopsy, he was diagnosed with ACC stage IV. Thus, he underwent resection of the left adrenal gland tumor. After the operation, he received adjuvant chemotherapy including mitotane, an antiadrenal agent for ACC. The lung metastases remained unchanged and new lesions were not found during his clinical course. He underwent resection of the right and left lung metastases 7 and 16 months after the primary operation, respectively. He was free from recurrence as of 28 months after the admission.
While there is no established treatment protocol for advanced ACC in children, complete resection of the tumor with adjuvant chemotherapy including mitotane would be effective.

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Congenital subglottic stenosis is a rare anomaly caused by thickened cricoid cartilage. We tried ablating the thickened parts of the cricoid cartilage with KTP laser under direct vision, and attempted to preserve the tracheal opening by inserting a Tracheal Opening Retainer (TOR) during laryngotracheoplasty (LTP) with costal cartilage grafting. We could successfully enlarge the subglottic lumen without any complications. TOR was removed 55 days, and the tracheal openig was closed surgically 1 year and 2 months after LTP. It was suggested that ablation with KTP laser was safe and effective to vaporize the thickened cricoid cartilage and that retaining the tracheal opening by TOR at LTP was effective for safely providing an alternative channel for respiration during extubation of the nasotracheal stent tube.

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We report three cases of children who underwent singleincision laparoscopic reduction of intussusceptions using E-Z access (Hakko Medical Co.). Case 1 was a 4-yearold boy with an ileocolic intussusception. Case 2 was an 11-year-old girl with recurrent ileocolic intussusception 10 hours after successful hydrostatic enema reduction. Case 3 was an 8-month-old girl with an ileo-ileocolic intussusception. A semicircular umbilical incision was applied with a Lap Protector^TM (Hakko Medical Co.). The E-Z access^TM (Hakko Medical Co) inserted with 3 ports was equipped to the top ring of the Lap Protector^TM. Intussusception could be reduced laparoscopically using two forceps followed by ileal-walking. All cases were free of postoperative complications, and were discharged 7 days after the operation. This single-incision laparoscopic method could therefore reduce intussusceptions without restriction of the wound or port sites. In addition, it is easy to convert laparoscopic surgery to laparoscope-assisted surgery, and vice versa. Note that laparoscopic surgery is also cosmetically superior other methods.

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We report a case of congenital dyserythropoietic anemia (CDA) type-II relieved from transfusion-dependency by splenectomy. A 1,224 g female infant was born at 28 weeks and 5 days of gestation by cesarean section due to nonreassuring fetal status. After birth, she suffered from severe anemia. She was suspected to have CDA by detailed examination, and underwent repeated transfusion. Finally she was diagnosed as CDA type-II from genetic analysis at age 2 years. Anemia caused by ineffective erythropoiesis required frequent transfusions. Her splenomegaly and gallstone appeared at age 2 years and 5 months. Splenectomy and cholecystectomy were performed at age 3 years. The spleen was 370 g in weight. Postoperative histopathological examination confirmed the diagnosis of severe hemostasis and hemosiderosis, suggesting hypersplenism as the cause. She was discharged 8 days after surgery. Postoperative course was uneventful and she was relieved from transfusion.

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Hemangioma is a rare cause of hepatobiliary obstruction in children. We report a child with obstructive jaundice caused by capillary hemangioma.
A 2-year-old boy was referred to our institution because of jaundice and acholic stool. There was absence of hepatomegaly or palpable mass on physical examination. Results of laboratory studies were as follows: total bilirubin, 8.7 mg/dl; direct bilirubin, 6.3 mg/dl; AST 271 IU/l; ALT 303 IU/l; CRP 0.2 mg/dl. Magnetic resonance imaging and ultrasonography showed severe stenosis of the common hepatic duct (CHD) and dilation of the intrahepatic and both hepatic ducts. Tumor markers were within the normal limits.
An exploratory laparotomy was performed because jaundice was developing rapidly. Intraoperative cholangiography via the gallbladder revealed complete stenosis of the common hepatic duct with normal choledochus. On palpation, there was partial thickening of the wall in the CHD with reference to the site of the stenosis. No patency was observed in the stenotic site after choledochotomy at the confluence of three ducts. A biopsy specimen was obtained from the thickened wall, and a drainage tube was threaded through the biopsy site because outflow of bile was observed at the site. Jaundice rapidly disappeared with improvement of liver function. A histopathological study showed benign hyperplastic lesions without malignancy and inflammation.
As repeated cholangiography through the drainage tube revealed no improvement of the CHD obstruction, we resected the extrahepatic biliary tract with reconstruction of hepaticojejunostomy 2 months thereafter. A histiopathological diagnosis of juvenile capillary hemangioma of the CDH was confirmed. He is curently doing well without jaundice 5 years after the operation.

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We experienced a rare case of cystic lymphangioma at the extra-peritoneal space of the anterior abdominal wall.
A 3-year/7-month-old boy was admitted due to suspected acute appendiceal abscess. He had a history of right communicated scrotal hydrocele repair in our institute 21 days before admission. On re-admission, the right scrotal hydrocele appeared normal. On biochemical analysis, the white blood cell count elevated to 17,590/mm³, and C-reactive protein increased to 12.11 mg/dl. IV fluid therapy with antibiotics was therefore started. A computed tomography revealed multi-cystic mass located at the right lower abdominal quadrant. An ultra-sound examination showed small multi-cystic mass located at the right groin connected to the abdominal mass. He was diagnosed as having an infected cystic lymphangioma after communicated scrotal hydrocele repair. Laparoscopic intervention was performed on 21 days after admission, including adheolysis of omentum from the cystic mass. The appendix appeared normal. Most of the cystic mass was removed through the wound of the previous hydrocele repair. OK-432 solution was also injected into the remaining cystic mass. Histologically, the removed mass was confirmed as cystic lymphangioma. Postoperative course was uneventful, and no recurrence was found in two years.

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