Purpose: The tunneled central venous catheter (CVC) is the life line for pediatric intestinal failure patients. Catheter-related bloodstream infections (CRBSIs) were one of the most serious catheter-related complications. Ethanol lock therapy could prevent removal of CVC. Here, we review our cases of ELT and the effectiveness of ELT for CRBSIs. Methods: From January 1, 2008, through June 30, 2010, twenty one cases underwent ethanol lock therapy for 5 days in addition to systemic antibiotic therapy to treat tunneled CVC-related bloodstream infections. The patient's records were retrospectively reviewed. Results: ELT was successful in seventeen of 21 cases (81%) without recurrence. Gram-positive organisms were detected in 71%. Gram-negative organisms and yeasts were isolated in 19% and 20%, respectively. All cases of yeasts infection were successfully treated. No previously reported adverse reactions or adverse effects were observed. Conclusions: This study suggested that the ethanol lock therapy with systemic antibiotic therapy is an effective and safe method for CRBSIs, recovering the infected CVC and decreasing the need for catheter removal.

View full abstract

Purpose: To evaluate outcomes of sutureless abdominal wall closure in gastroschisis. Methods: Clinical records of all patients who underwent abdominal wall closure between 1990 and 2010 in our hospital were retrospectively reviewed. Five patients who underwent sutureless closure were compared to a historical group of nine patients who underwent suture closure. End points were intubation period, time to starting oral feeds, time to full oral feeds, hospital stay, and postoperative complications during hospitalization or follow-up period. Results: Time to full oral feeding with sutureless closure was significantly shorter than with the traditional method (19.6 vs 45.1 days, p=0.04). There was no difference in intubation period (8.6 vs 21.2 days, p=0.28), time to starting oral feeds (10.8 vs 27.1 days, p=0.08), or hospital stay (34.6 vs 69.3 days, p=0.08). There was one postoperative complication with sutureless closure requiring additional care. There were five complications (56%) with traditional closure. One patient in the sutureless closure group (20%) required surgery for complications within the follow-up period. Additional surgery was also performed on five patients (56%) who underwent traditional suture closure. Conclusions: Sutureless abdominal wall closure is useful as it has a lower rate of complications and requires a shorter hospital stay.

View full abstract

Purpose: Long-term outcome of modified Duhamel procedure (Z-shaped colorectal side-to-side anastomosis) for Hirschsprung's disease (HD) is reported. Methods: Between 1978 and 2010, 123 children who had the aganglionosis from the rectum to the colon underwent a modified Duhamel procedure with Z-shaped anastomosis using a linear stapling device. A questionnaire on anorectal function was sent to the patients more than 4 years old. Questions were asked on use of laxatives, stool frequency, diarrhea, feeling of rectal fullness, rectal sensation, severity of perianal erosion, soiling, and incontinence. Responses were obtained from 72 of 111 patients (65%). Among these, 65 patients at the age 4-34 years, excluding the 7 patients with trisomy 21 and mental retardation, were evaluated by means of a scoring system. The maximum possible score was 40 points (Excellent; 36-40, Good; 31-35, Fair; 26-30, Poor; 0-25). Results: In group I (4-6 years old: n=7), the mean score was 33.1 (Excellent, 3; Good, 2; Fair, 1; Poor, 1). In group II (7-12 years old: n=11), the mean score was 37.7 (Excellent, 10; Poor, 1). In group III (13-18 years old: n=18), the mean score was 38.8 (Excellent, 17; Good, 1). In group IV (19-34 years old: n=29), the mean score was 38.9 (Excellent, 29). Forty-six patients more than 13 years old (98%) had excellent results. In the 29 patients more than 19 years old, anorectal function was normal; among them, five patients occasionally required laxatives for constipation. No patients had soiling or incontinence. Conclusion: Our series revealed that the long-term results of this procedure were satisfactory.

View full abstract

We report a case of congenital anterior urethral diverticulum accompanied by complications of the urinary tract at the time of diagnosis, in which staged surgical treatments were performed. A male infant had repeated urinary tract infections since he was 7 months old. He was referred to our hospital as a voiding cystourethrogram at 11 months old showed severe bladder deformity, right VUR (grade 4) and urethral diverticulum located in the pendulous part. We speculated that a lower urinary tract obstruction due to anterior urethral diverticulum induced retrograde high pressure on the bladder and right VUR. Cystourethroscopy was performed at 12 months old and revealed a membranous stenosis at the distal lip of the diverticulum; therefore endoscopic resection of the membrane was done. However, no reduction of the diverticulum was observed after that. We performed diverticulectomy when he was 17 months old, resulting in the disappearance of urethral dilatation. However, as no improvement was observed for the right VUR, an antireflux operation (Cohen procedure) was added at the age of 24 months. Renal scintigraphy showed no deterioration of split renal functions after these operations.

View full abstract

We report a case of a solid pseudopapillary neoplasm (SPN) of the pancreas. The patient was a 12-year-old girl with a history of proteinuria. Ultrasonography of the abdomen identified a large tumor located in the pancreatic head. Subsequent CT and MRI revealed the images consistent with SPN of the pancreas. Intraoperative findings showed a globular tumor located in the uncinate process of the pancreas. The upper side of the tumor was covered with the pancreas tissue, while bottom side projected outside of the pancreas. The tumor had complete fibrous encapsulation without any signs of external invasion. Therefore, we performed a total excision of the SPN. Macroscopically, the removed SPN was 13×11×10cm in size with a weight of 878g. Pathological examination confirmed the diagnosis of SPN. A pancreatic fistula developed after tumor resection. Leakage of pancreatic secretions appeared to originate from the dissected surface of the pancreas. The patient required long-term treatment with conservative therapy consisting of parental nutrition and administration of drugs such as protease inhibitors.

View full abstract

According to the Kasai classification, I cyst type biliary atresia (BA) is defined as that with hepatic hilum cyst and patent hepatic duct. Herein, we report an infant case of anomaly of the biliary tree which resembled I cyst type BA but with a patent common bile duct. A 2-month-old girl with jaundice and acholic stool was found to have a hepatic hilum cyst by ultrasound. She underwent an operation at 80 days of age with suspicion of BA. The first intraoperative cholangiography showed a hepatic hilum cyst with fine intrahepatic ducts which suggested I cyst type BA. However, the patent common bile duct from cyst to duodenum was demonstrated by operative finding and a second cholangiography. As a correctable hepatic duct did not exist, hepatic portoenterostomy was performed. Her jaundice was cured within 1 month after the operation and her postoperative course was uneventful without jaundice as of 13 months after operation. According to operative findings, cholangiography, and liver biopsy, we diagnosed this case as BA rather than congenital biliary dilatation. As this case did not fit with the definition of either I cyst type nor type III BA, we considered the case as unclassified type. Further study and an understanding on the classification of BA is necessary.

View full abstract

We report a case of possible carnitine deficiency in a 14-year-old boy. The patient had been in treatment for multiple organ failure due to severe appendicitis. It took 47 days to be weaned from continuous hemodiafiltration because of renal failure. Two other surgeries for multiple intestinal perforations after appendectomy were needed, and two stomas were finally made at 20 and 80cm anal side from the ligament of Treitz. A full support of total parenteral nutrition was necessary. Eighty-seven days after admission, heart failure was found by cardiac ultrasonography (ejection fraction: 37%). The cause was unclear other than micronutrient deficiency. Administration of transvenous sodium selenite and L-carnitine through the 60-cm jejunum between two stomas improved cardiac function after 3 weeks (ejection fraction: 68%). When cardiac failure was found, the serum levels of free carnitine and selenium were almost normal. However, an abnormally low level of serum free carnitine was observed after the closure of the two stomas. Considering that the serum low level of carnitine sometimes does not reflect the symptoms immediately, the carnitine deficiency could be the cause of cardiac failure. This is also supported by the fact that the case had multiple risk factors of carnitine deficiency including poor enteral feeding, massive intestinal discharge and prolonged dialysis.

View full abstract

A 3-year-old boy was admitted to a hospital because of abdominal pain and bloody stools. Abdominal ultrasonography and abdominal CT revealed a mass with calcification in the pelvis. He was transferred to this hospital with a diagnosis of appendicitis. A blood test showed mild inflammatory findings. The cystic lesion was thought to be a niveau in the pelvis. The mass was diagnosed to be a tumor in the pelvis, such as a teratoma, because a barium enema showed pressure on the rectal wall. Surgery was therefore performed on the next day based on a diagnosis of acute peritonitis, because the child had developed muscle guarding and new blood tests showed an increase in the CRP level to 16.3g/dl. Emergency surgery was performed due to an abscess in the pelvis in association with acute appendicitis. Surgery revealed a cystic mass with inflammatory changes on the dorsum side of the rectum, which was diagnosed to be a rectal duplication. A rectal duplication is relatively rare in children. Intestinal duplication often presents with an abdominal mass or ileus; however, the current patient presented with peritonitis. Therefore, no preoperative diagnosis could be successfully made in this patient because he presented with acute abdomen.

View full abstract

A 5-year-and-10-month-old girl was admitted to our hospital because of pollakiuria and hematuria. Twice inguinal hernia operations including a relapse on the left side and once on the right side were performed on her in an adult hospital in infancy. Computed tomography and ultrasonography revealed cystolithiasis, then cystoscope was performed under general anesthesia. It showed the stone fixed at the left side of the apex of the bladder. Laparoscopy, performed at the same time, also revealed an adhesion between the bladder and the abdominal wall around the left internal inguinal ring. Therefore, we performed open cystotomy and removed the stone with a part of the mucosa of the bladder. The stone was centered by non-absorbable suture material, then it was strongly suspected that the left inguinal hernia operation in infancy was related to the stone. Five months after the cystolithotomy, a new bladder stone appeared at the same position in the bladder, then we removed the stone using cystoscope. In this report, we discuss cystolithiasis as a complication of the inguinal hernia operation in infancy.

View full abstract

A 2-year-old girl with sudden abdominal pain and vomiting was referred to our institution. She had mild abdominal distension, but no tumor was palpable. Only leukocytes increased slightly in the laboratory determination, and various tumor markers were negative. Ultrasonography showed an intra-abdominal giant lobular tumor. It was encapsulated and had fat-like density. The contrasting CT showed the capsule to be enhanced moderately. MRI depicted the tumor as high intensity in both T1WI and T2WI. It presented a decrease of the intensity equally in the fat suppression condition. We estimated it to be some kind of lipogenous tumor, and we operated on her. The tumor was present in mesojejunum and it was totally removed surgically with some jejunum. The tumor was 13×14×5cm in size and 531g in weight. We diagnosed it as lipoblastoma histologically. She recovered soon, and is doing well now. Lipoblastoma is a benign tumor, but its differentiation from liposarcoma is very difficult. It is rare that lipoblastoma arises in the mesenterium. When this lesion grows in the visceral cavity, it is hard to detect until it becomes a giant tumor. We present a case of mesenteric lipoblastoma, with a review of 10 other cases reported in Japan.

View full abstract

Most bronchogenic cysts present in the mediastinum, and subcutaneous bronchogenic cysts are rare. We herein report a case of subcutaneous bronchogenic cyst in the presternal region. A subcutaneous mass was noted at the age of three weeks. Soon after the mass enlarged and was infected. Although conservative therapy led to temporary improvement of infection, the mass was infected again. After relieving the infection, a surgical resection was performed. Operative findings revealed no communication with the mediastinum or the airway. Histologically, the inner surface of the cyst was covered with ciliated columnar epithelium. Smooth muscles were also found.

View full abstract

We report a case of a 13-year-old boy who was diagnosed with blue rubber bleb nevus syndrome (BRBNS) treated by excision of all of the hemangiomas of the gastrointestinal tract. He presented a hemangioma of the back from birth. He was treated by excision for multiple hemangiomas of the skin at the age of 6 years. He presented anemia and was diagnosed as having multiple gastric hemangiomas by gastroscopy at the age of 9 years. He suffered from chronic anemia due to gastrointestinal bleeding, and underwent endoscopic mucosal resection for 3 gastric hemangiomas at the age of 12 years. He presented multiple colonic hemangiomas by colonoscopy, so he was diagnosed with BRBNS. He presented multiple small intestinal hemangiomas which were considered a bleeding source, by bowel X-ray examination. Steroid and interferon therapy were not effective, so we treated him by excising for multiple hemangiomas of the gastrointestinal tract. Laparotomy revealed the following hemangiomas: 1 in the stomach, 31 in the small intestine, and 7 in the colon. We resected all hemangiomas of the gastrointestinal tract, by mucosal resection if possible, but by wedge resection for most of them. Total excision by the operation for hemangiomas of the gastrointestinal tract is recommended, but new hemangiomas may develop after surgery, so we stress to preserving as much of the intestine as possible.

View full abstract

The patient was an 8-year-old girl. She was suspected of having acute appendicitis and referred to our department. Her height was 123.3cm, and body weight was 23.9kg (obesity:+15.7%). There was no digestive symptom, such as vomiting or diarrhea. Spontaneous pain was noted in the right lower quadrant over the lateral abdominal region, but no mass was palpated and she was without fever. Tenderness and rebound tenderness were noted, but muscular protection was negative. Blood examination on admission: WBC 12,300/μl, CRP 6.98mg/dl. An ultrasonograph revealed a mass in the intra abdominal space, and a CT showed the swirling structure inside the mass. Primary torsion of the greater omentum was diagnosed and laparoscopic surgery was performed. The greater omentum formed a mass and adhered to the abdominal wall in the right upper abdominal region. The omental mass was resected. The postoperative course was favorable, and the patient was discharged on the 5th day after surgery. Primary torsion of the greater omentum should be considered as a disease to be differentiated on diagnosing appendicitis in children.

View full abstract