Purpose: A congenital portosystemic shunt (CPSS) may cause hyperammonemia, pulmonary vascular problems, and hepatic nodules such as focal nodular hyperplasia (FNH). We evaluated our CPSS patients treated surgically.

Methods: Between January 2004 and October 2018, ten CPSS patients were surgically treated. Age and episode for diagnosis, type of shunt, clinical manifestations, indication, age at surgery, mode of surgery, and prognosis were evaluated.

Results: There were three boys and seven girls with the median age of 0 months ranging from 0 to 5 years old. The most frequent reason for diagnosis was a positive result in neonatal mass screening for galactosemia. The types of shunts were port-caval shunt in five and patent ductus venosus in four. Regarding clinical manifestations, portopulmonary hypertension (PPHTN) was observed in five, hepatopulmonary syndrome (HPS) in two, and rapid growth of FNH in two. In patients with PPHTN, liver transplantation was performed in three and shunt ligation in two. In those with HPS, liver transplantation was performed in one, shunt ligation in one, and left lateral segmentectomy in two with rapid growth of FNH. One patient with HPS treated by shunt ligation showed recurrence of the intrahepatic portosystemic shunt after the ligation and PPHTN occurred. Liver transplantation was required four years after ligation. After surgical treatment, all the patients with pulmonary vascular problems improved, and four patients who received liver transplantation became free from medication. Two with FNH treated surgically have been doing well without recurrence.

Conclusions: Surgical treatments were effective for CPSS patients. The indication and method of operation should be determined individually on the basis of the clinical status of patients and the morphology of the shunt.

Purpose: The aim of this study was to confirm the efficacy of completely divided colostomy of the sigmoid colon in the management of anorectal malformations in our institution.

Methods: The cases of 10 patients who underwent completely divided colostomy of the sigmoid colon for anorectal malformations at our hospital between April 2009 and June 2016 were studied. We retrospectively investigated complications related to the colostomy, the diameter of the distal rectum before radical surgery, and urinary tract infections. The diameter of the distal rectum was evaluated using the anteroposterior diameter on a preoperative examination.

Results: All 10 patients were male. Four had prostatic fistulas, and six had bulbar fistulas. The following colostomy-related complications were observed: surgical site infections, 0; prolapse, 0; stenosis, 0; difficulty setting up the stoma pouch, 3. There were no difficulties in pulling through the rectum owing to restrictions caused by the colostomy during the radical surgery in none of the patients. In addition, none of the patients required colostomy reconstruction. A urinary tract infection was detected before the radical surgery in one patient. The median diameter of the distal rectum was 1.75 cm.

Conclusions: Completely divided colostomy of the sigmoid colon caused few complications, and our results suggest that it did not dilate the distal rectum.

Purpose: Foreign body aspiration into the airway in children leads to a serious condition that requires prompt management. A definitive diagnosis cannot always be obtained from chest X-ray images, as only 10% of foreign bodies are radiopaque. Furthermore, the removal of foreign bodies using a bronchoscope is technically difficult owing to the narrow airway in children.

Methods: Fifteen cases of foreign body aspiration at our institution were collected and analyzed retrospectively.

Results: Most of the patients (66.7%) were male, and 80% were under two years of age. Only three patients were diagnosed on the basis of symptoms. One patient was asymptotic. Chest X-ray showed radiopaque bodies in four patients. The most common foreign bodies were beans (40%). Computed tomography was performed in seven patients, and foreign bodies were detected in five patients. In six patients, no foreign bodies were detected by imaging modalities. The foreign body was removed in six patients using rigid bronchoscopy. In three patients, food-based foreign bodies were removed by irrigation and suction using flexible bronchoscopy because food-based foreign bodies were dissolving.

Conclusions: Foreign body aspiration in children shows nonspecific symptoms. Chest X-ray is not sufficiently sensitive, and although computed tomography is a sensitive modality, it sometimes shows false-negative findings. Detailed discussions regarding the aspiration history and symptoms are the most important factors for achieving a definitive diagnosis. Bronchoscopy should be considered for cases suspected of involving foreign body aspiration.

Purpose: In this paper, we report the activities of a nonprofit organization (NPO) that supports the growth of children who have undergone surgery and discuss their postoperative QOL.

Methods: A questionnaire survey involving 34 pediatric patients who had undergone surgery was conducted to examine their health-related distress/difficulties and methods of collecting medical information.

Results: In October 2015, a general meeting was held to establish the NPO Growth Support for Children after Surgical Operation, and in February 2016, the organization was officially approved. To date, it has organized health consultation sessions and open lectures for citizens. In the questionnaire survey, responses were obtained from all of the 34 pediatric patients. Of the 11 patients aged 10 or older with bile duct dilatation, three had an increased risk of cancer and three had postoperative calculus. Of the 10 patients younger than 10 with bile duct dilatation, three had an increased risk of cancer and two had abdominal pain. As for the method of collecting medical information, 32 of the 34 patients collected such information from the hospital/doctor in charge, whereas 22 used the Internet.

Conclusion: The results suggest that pediatric patients who receive outpatient consultation regularly after surgery and their families have difficulty in sufficiently consulting their doctors about their distress/difficulties during the consultation. Their tendency to use the Internet and SNS more frequently to collect medical information was also noted. Through its activities, We will continue to listen to complaints of postoperative pediatric patients to clarify and reduce their distress and difficulties in daily life.

Purpose: Several investigators have reported that ductal plate malformation (DPM) in the liver is characteristic of persistent embryonal ductular strictures and the embryonic subtype of biliary atresia (BA). We investigated the relationship between liver fibrosis and DPM in BA.

Methods: Thirty-two BA patients underwent Kasai operation from 1976 to 2017. We compared two groups: Group A had DPM and Group B did not. Biopsies obtained during the initial Kasai procedure were histopathologically analyzed in terms of the degree of portal-portal (P-P) bridging and the existence of portal-central vein (P-C) bridging. The degree of P-P bridging was defined as follows: Grade 1, P-P <50%; Grade 2, >50%; Grade 3, nodular architecture. We also evaluated the age at the time of Kasai operation, the number of patients who became jaundice-free (JF; total bilirubin <2.0 mg/dl), and the time taken to become JF. Statistical comparisons of multiple data were carried out using the chi-square test and Student’s t-test. Regression analysis was carried out with the significance level set at p <0.05.

Results: There were nine patients in Group A and 23 patients in Group B. There was no significant difference in P-P bridging (p = 0.281). Seven patients had P-C bridging in Group A and eight in Group B (p = 0.028). The ages at the time of Kasai operation were 67.3 ± 17.8 days in Group A and 89.0 ± 29.7 days in Group B (p = 0.050). There were no significant differences in the number of JF patients (p = 0.599) and the time taken to become JF (p = 0.252).

Conclusions: DPM tended to be associated with early age of operation and having P-C bridging.

We experienced treating two cases of Currarino syndrome in which defecation function was improved by dilated large bowel resection. Their constipation was associated with intestinal dilation following anorectal malformation surgery. Case 1: A boy aged four years and five months was diagnosed as having Currarino syndrome with rectal atresia, sacrococcygeal partial defect, and presacral meningocele as its components. Rectal anastomosis and meningocele resection were performed at the age of 4 months after colostomy, and the colostomy was closed at 7 months of age. An enema, laxative, and herbal medicine were used, but the dilation from the rectum to the sigmoid colon became severe. It was difficult to control defecation, and the expanded intestinal tract was resected. As of 6 years of age, he uses only laxative and herbal medicine. Case 2: A boy aged four years and three months was diagnosed as having Currarino syndrome with anal atresia, sacral malformation, and sacral lipoma as its components. After a colostomy, he underwent anoplasty at six months of age, and the colostomy was closed after one year and one month. He needed an enema and an intestinal lavage after surgery, and the expansion from the rectum to the sigmoid colon became worse, so the dilated intestinal tract was resected. At the age of 12, he has self-defecation but sometimes uses an enema. Hypoperistalsis due to chronic intestinal dilation associated with anorectal malformation was considered to be the cause of constipation.

We herein report the case of a patient in which gastric transposition was effective for refractory esophageal stenosis induced by the accidental ingestion of a strong alkali. A 6-year-old girl had a history of esophageal stenosis due to corrosive esophagitis caused by the accidental swallowing of a strong alkali at 3 years of age. The patient was treated by balloon dilatation and radial incision and cutting (RIC). However, her esophageal stenosis relapsed; thus, gastric transposition was planned. The esophagus was removed by a thoracoscopic approach. The posterior mediastinal tunnel was formed by blunt dissection from cervical and abdominal incisions, and the cervical esophagus and stomach were anastomosed in the neck. After the gastric transposition, she was able to take food orally. Regular endoscopic examination was performed for the observation of anastomosis, and dilation was performed for mild anastomotic stenosis. Her physical growth was within the normal range, and no retardation was recognized after the reconstruction procedure. Corrosive esophagitis is chemical esophagitis induced by a corrosive agent swallowed such as a strong acid or alkali. Scarring esophageal stenosis is a late complication of corrosive esophagitis. Balloon dilatation is the first choice for the treatment of esophageal stenosis, but conservative treatment sometimes has limited efficacy. Surgical esophageal reconstruction should be considered for uncontrollable cases. Gastric transposition is considered a physiological and safe procedure for the treatment of severe esophageal stenosis.

A 13-year-old girl was treated for congenital heart disease and polysplenia. Bloody vomiting occurred during hospitalization for a cardiac catheter test at the age of 12 years. She was brought to our department, and she was diagnosed as having gastric volvulus complicated by wandering spleen on the basis of abdominal CT findings. She was treated conservatively by depressurization using a nasogastric tube and closely followed-up. However, gastric volvulus recurred multiple times over a short period; therefore, laparoscopic gastropexy and splenectomy were performed. An inversed stomach and spleens of various sizes were observed intraoperatively. Some of the spleens that were fixed to the retroperitoneum were preserved, and the other wandering spleens were excised. Gastropexy was carried out with three points of gastric fixation to the abdominal wall. A nonrotation-type intestinal malrotation was recognized, and prophylactic appendectomy was performed. The postoperative course was excellent, and three years have passed without recurrence. Polysplenia often complicates various cardiovascular and intraabdominal organ malformations such as biliary atresia or intestinal malrotation, but gastric volvulus is very rare. Since radiological images vary among individual cases, careful consideration of imaging results is needed for choosing the appropriate treatment strategy.

Pelvic lymphangiomas are rare among lymphangiomas. Incomplete resection often causes recurrence of the lymphangioma, and it is sometimes difficult to extirpate the lesion without complications. A 7-year-old boy presented with fever and painful urination. Ultrasonography and abdominal computed tomography (CT) with contrast enhancement revealed a huge cystic lesion occupying the pelvic cavity and a dilated right renal pelvis and ureter. The size of the cystic lesion was 10 × 7 × 11 cm. Urography revealed a narrow portion in the prostate bulb urethra. Cytological examination revealed that the aspirated fluid was lymphocyte-predominant. On the basis of these findings, the patient was diagnosed as having pelvic lymphangioma. We attempted to perform laparoscopic complete resection, but performed laparoscopic fenestration instead because the adhesion between the dorsal cyst and the prostate was very firm. We resected the cyst wall as much as possible, and covered the residual cyst lumen with a polyglycolic acid (PGA) sheet and fibrin glue spray. The postoperative course was uneventful, and the patient was discharged on the 14th postoperative day. This patient has been followed up for 3 years without recurrence and relapse.

The patient was 3-year-old girl who was admitted to a hospital owing to abdominal pain and vomiting, which was suspected as gastroenteritis. She was transferred to our department because a structure with a netlike appearance and containing air was observed in the duodenum by abdominal CT, suggesting ileus caused by a trichobezoar. The trichobezoar in the duodenum migrated to the intestine, resulting in intestinal obstruction, and the patient received gastric decompression using a gastric tube and fluid resuscitation in preparation for surgery. After we confirmed by endoscopy that there was no trichobezoar remaining in the stomach, a small incision was made at the umbilicus, the jejunum containing the trichobezoar was pulled out, and the trichobezoar was taken out through an incision on the intestinal wall. The postoperative course was uneventful, and the patient was discharged on day 8 after consulting with the psychiatric department. Our patient was the youngest case of intestinal obstruction by a trichobezoar in the literature. We achieved a safe and less invasive surgical treatment, and the course of the patient suffering from trichobezoar was unique.

Congenital portosystemic shunt (CPSS) can cause hepatopulmonary syndrome and encephalopathy, among other complications. Interruption of the shunt blood flow is the definitive treatment and can prevent such complications. However, there is no standardized treatment approach for CPSS because of the varied pathophysiological states that can manifest. We treated three cases of CPSS with three different approaches. A one-year-old girl was treated for shunt vessel transection by laparoscopic liver resection. A 10-month-old boy with congenital heart disease underwent shunt vessel banding by laparotomy. A 17-year-old boy underwent transcatheter embolization. CPSS may cause severe complications, such as progressive pulmonary hypertension. Therefore, even when no clinical manifestations are apparent, CPSS should be treated as early as possible. If hypoplasia of the intrahepatic portal system (IHPS) is mild or moderate and the portal venous pressure (PVP) under the occlusion test is <25 mmHg, shunt closure can be performed in a single step. If hypoplasia of IHPS is severe or the PVP is ≥25 mmHg, the patient is a candidate for 2-step shunt closure. Endovascular closure is the first option for treatment, whereas surgical closure should be considered second. We should carefully select our approach on the basis of the preoperative evaluation of the anatomy of the shunt vessel and IHPS.

We report two cases of lymphatic malformation derived from the mesentery of the small intestine. (Case 1) A female child aged three years and 4 months was brought to our hospital for sudden abdominal pain. Computed tomography (CT) showed a mesenteric cyst measuring 16 × 9 cm. Laparoscopic resection of the cyst was performed. The histopathological finding revealed that the cyst was a lymphatic malformation of the mesentery of the small intestine. (Case 2) A 17-year-old female who was diagnosed as having an ascending mesenteric cyst seven years ago visited our hospital owing to increasing frequency of abdominal pain. We performed laparoscopic resection of the cyst for relief of the abdominal pain without resection of the intestinal tract. The histopathological analysis revealed that the tumor was a cavernous lymphatic malformation arising from the mesentery of the small intestine. Tumor resection is usually performed for lymphatic malformation arising from the mesentery. Laparoscopic surgery for mesenteric lymphatic malformation is safe with a good field of view and less invasive with a minimal wound.

We present the case of a 1-year-old girl who presented to a local hospital with abdominal pain. A 10-cm calcified tumor was found in her right abdomen. She was suspected of having ovarian tumor torsion and was transferred to our hospital. We decided not to perform emergency surgery on the day of her admission, as blood flow could still be detected in the ovarian tumor. However, the next day, emergency surgery was performed owing to the exacerbation of her anemia and inflammatory reaction and the whorled appearance of her uterus on magnetic resonance imaging. Intraoperative examination revealed a congested uterus that was rotated 180 degrees in the clockwise direction. However, the left ovarian tumor was not twisted. We considered that the uterine torsion occurred because the left ovarian tumor pulled on it as it migrated to the right flank. The left ovarian tumor was enucleated, and the uterus was preserved, as evidenced by the restoration of its normal color after the release of the twist. The ovarian tumor was diagnosed on the basis of histopathological findings as a teratoma. Uterine torsion is a very rare condition, defined as uterine rotation of more than 45 degrees around the longitudinal axis. Most cases are seen in pregnant women or in elderly women with myoma of the uterus. This was the second pediatric case reported in Japan and, to the best of our knowledge, the youngest case reported worldwide.

Congenital pouch colon (CPC), which is one of the anorectal malformations with pouch formation of the colon, is rare in Japan. Persistent descending mesocolon (PDM) is a syndrome with the displacement of the left colon with a shortened mesocolon and radial distribution of the inferior mesenteric artery. No case of CPC complicated by PDM has been reported. A boy was born with anal atresia. X-ray examination showed a huge colon gas. After 15 h, laparoscopy showed that the left colon is fixed to the midline, and the sigmoid colon extended like a pouch, which suggested Type-4 CPC with PDM. Colostomy was carried out at the oral edge of the pouch to preserve the normal sigmoid colon, although Type-4 CPC commonly requires the excision of the pouch at the first operation. Contrast enema of the pouch colon showed a colourethral fistula. MRI showed closed spinal dysraphism with myelocystocele. Because of repeated stoma prolapse, we performed laparoscopic pull through after the excision of the pouch and the ligation of the colourethral fistula on the 47th day. The control of defecation is good two years after the last operation. Because CPC has not been well recognized in Japan, an operative strategy based on a precise diagnosis is difficult. However, CPC is accompanied by multiple anatomical malformations in the gastrointestinal, urological, gynecological, skeletal, and neurospinal areas.

The patient was an 11-day-old girl born at 39 weeks and 1 day of gestation, weighing 2,918 g, and without any abnormal findings during fetal growth. She initially showed poor feeding and poor weight gain, followed by bilious vomiting 10 days after birth. She was referred to our department for detailed examination and treatment 11 days after birth. Congenital duodenal stenosis (the initial finding) was diagnosed on the basis of chest and abdominal plain X-ray findings and upper gastrointestinal series, and laparoscopic surgery was performed. A diameter difference of the intestine was observed in the descending part of the duodenum, and a transverse incision was made slightly towards the oral side. However, insertion of an 8-Fr Nelaton catheter (NC) was not possible from either the oral or anal side of the incision. No air flow in the incision was seen when air was introduced through a nasogastric tube. Another transverse incision was made to the enlarged portion of the oral side of the duodenum, and patency to the oral side was confirmed. A 2-cm vertical incision was made in the horizontal part of the duodenum, which was sufficiently mobilized, and patency to the anal side was confirmed by introducing air through an NC placed via the vertical incision. These findings indicated two stenoses. The oral side was reconstructed with a diamond-shaped anastomosis, formed by the quadrilateral resection of the intestinal wall contralateral to the mesentery to connect the two transverse incisions. In this patient, we demonstrated that the presence of multiple stenoses should be examined not only during laparotomy, but also during laparoscopic surgery. Our findings suggest that air flow through the NC, which was used in the present case, is an effective method of examining the presence of any other stenosis.