Purpose: We perform single-stage reconstruction (SR) for cloacal exstrophy (CE). SR comprises the following steps: gastrointestinal tract reconstruction with colostomy, bladder and urethral closure, pubic approximation without osteotomy, and abdominal closure. Herein, we describe the procedure for each step of SR and postoperative management, and we review the outcomes of SR for CE.

Methods: The cases of six patients with CE who had undergone SR from 1990 to 2019 were reviewed. SR was performed within 48 hours of birth. Anticholinergic administration and clean intermittent catheterization were started soon after SR. Data were collected regarding surgical complications, bladder volume, and the achievement of urinary continence.

Results: The patients were four males and two females aged 3 to 26 years (mean 11.3 years). Four patients had omphalocele, and all patients had spinal anomaly. The pubis was approximated without osteotomy in all cases. Abdominal compartment syndrome occurred in one patient with a giant omphalocele prolapsing the liver. The urethral catheter was accidentally removed soon after SR in one patient; this patient developed urethral stenosis and required vesicostomy. Regarding late surgical complications, two patients needed surgery to relieve intestinal obstructions, and another patient needed recreation of the colostomy and vagina. Three patients became able to perform clean intermittent catheterization themselves at 7–10 years of age, and two of them achieved urinary continence at 10–15 years of age without undergoing continence procedures.

Conclusions: Our SR and postoperative management enables some patients with CE to achieve urinary continence, thus avoiding the need for multiple surgical procedures such as staged closure with osteotomy or continence procedures.

Purpose: In acute appendicitis with fecalith, the distal end of the cavity of the appendix is considered to be closed. However, the relationship between the fecalith and the bacterial distribution in the appendix has not been adequately clarified. Therefore, we retrospectively examined the correlation between the result of the bacterial culture of smears collected from both the proximal and distal ends of the cavity in the appendix and the existence of fecalith. Then, we discussed the selection of antibacterial drugs in cases with fecalith.

Methods: We examined pediatric patients who underwent immediate laparoscopic appendectomy for acute appendicitis in our department for two years from October 2017 to September 2019. Bacterial culture was performed for each of the swabs inserted into both ends of the appendiceal cavity. The group with a clear fecalith on the image was defined as the Stone Group and the group without it was defined as the Stoneless Group. We analyzed the patient background and the species isolated from colonies obtained by culture. Although most of the detected bacterial species were anaerobic, no significant difference in species was found between the collection sites.

Results: There were 15 patients in the Stone Group and 12 patients in the Stoneless Group. Although the average length of stay was longer in the Stone Group, there was no significant difference in other patient backgrounds. Bacteroides species were isolated more frequently from the distal end than from the proximal end in both the Stone Group and the Stoneless Group. When Bacteroides species were isolated from smears from the distal end in the Stone Group, the stage tended to be gangrenous appendicitis.

Conclusions: Even in cases that have not developed perforation or abscess, Bacteroides species may contribute to the pathogenesis of appendicitis with fecalith. Particularly for perforated appendicitis with fecalith, it may be necessary to select an antibacterial drug that is efficacious against such species.

Purpose: Disimpaction is the first step in managing pediatric chronic constipation. The aim of this study was to assess the clinical features of patients with fecal impaction and the efficacy of the regimen with rectal agents.

Methods: This was a retrospective study of 48 patients who were diagnosed as having fecal impaction among 138 patients with chronic constipation aged one year and above. Data on the characteristics of patients, symptoms and signs, treatment method, and outcome were reviewed. Glycerin enema (GE) or bisacodyl suppositories combined with oral osmotic laxative were used with informed consent on an outpatient basis. The therapeutic effect was judged after two weeks.

Results: The most common ages of first visit and onset of symptoms were two years and around one year, respectively. Ninety percent of the patients had suffered from constipation for more than a year. The most frequent symptom or sign of fecal impaction among those described in the Japanese Guideline for Pediatric Chronic Constipation was “fecal mass in rectum on imaging study” (96%), and 90% of the patients had more than two symptoms that matched those in the guideline. GE was administered in 39 patients and bisacodyl suppositories in five. Forty (90.9%) of the 44 outpatients were disimpacted successfully. Two of the four patients who were unsuccessfully disimpacted were intolerant to GE and one required manual evacuation in addition to GE.

Conclusions: The management with rectal agents, mainly GE and bisacodyl, was effective. Adequate education made most patients and parents accept it, and the success rate reached around 90%.

Purpose: This study was aimed to determine the impact of frenotomy in tongue-tie and/or lip-tie on breastfeeding problems.

Methods: A total of 343 infants with tongue-tie and/or lip-tie operated owing to their breastfeeding problems at Musashino Tokushukai Hospital from July 2015 to June 2019 were studied prospectively. Operation was indicated using the “Tongue-tie Score for Infants,” which the author developed. One month after surgery, improvement of symptoms and mothers’ satisfaction was evaluated on the basis of the mothers’ responses to a questionnaire.

Results: The male-to-female ratio was 1.5:1. Heredity of tongue-tie was observed in 48% of patients. The average age was 3.1 months. Poor latch was seen in 312 infants, nipple pain in 148 mothers, breast problems in 110 mothers, and poor weight gain in 23 infants. Two hundred seventy-one infants underwent both tongue-tie and lip-tie release. Tongue-tie release alone was conducted in 41 infants, and lip-tie release alone was performed in 31 infants. The percentages of tongue-tie types were as follows: 14% for the tongue tip type, 48% for the anterior membranous type, 20% for the tenting type, and 18% for the posterior type. The mean tongue-tie score was 4.5. Poor latch improved in 93% of the infants, nipple pain in 81% of the mothers, and poor weight gain in 62% of the infants one month after surgery. Poor latch and nipple pain improved in 85% of the infants and 81% of the mothers, respectively, even in infants with only lip-tie release. Ninety-seven percent of the mothers answered “satisfied” or “very satisfied” with the results.

Conclusions: Surgical release of tongue tie and/or lip tie resulted in improvement in the majority of the infants with poor latch and mothers with nipple pain at one month after surgery. Feeding problems was also improved by lip-tie release alone in those for whom tongue-tie release was not indicated.

Purpose: Pneumomediastinum is an uncommon disorder and usually benign and self-limiting. However, it sometimes requires invasive workup of suspected esophageal perforation and prophylactic antibiotics to prevent sequential mediastinitis. The purpose of this study is to review our experience and propose a management pathway for this rare condition.

Methods: The cases of all children over one year of age treated at our hospital with pneumomediastinum between September 2005 and November 2018 were retrospectively reviewed.

Result: We identified nine patients: eight males and one female. Their age range was from four to fourteen years (median, seven years). The primary symptoms were chest pain, back pain and sore throat. Asthma was the most common cause (five patients), followed by trauma (two patients). The remaining two patients were considered to have idiopathic pneumomediastinum. All patients were diagnosed on the basis of chest radiography findings. Computed tomography (CT) was performed in six patients and contrast-enhanced CT was performed to exclude gastrointestinal perforation in a patient with trauma. A boy with dysphagia underwent esophagography. Eight of the patients were admitted to our hospital: one patient with trauma and one with severe asthma were initially admitted to the pediatric intensive care unit. The length of hospital stay was three to eight days (median, four days). All patients improved with rest, and four patients were administered prophylactic antibiotics. No mediastinitis occurred and pneumomediastinum did not recur.

Conclusions: Most pediatric patients with pneumomediastinum need only supportive care and treatment for the underlying causes. We propose a management algorithm for pediatric pneumomediastinum based on the vital signs and underlying causes.

An 11-year-old boy with a complaint of two days of vomiting and abdominal pain was transferred to our hospital for small bowel obstruction (SBO). His CT images obtained from a previous hospital showed SBO caused by an intestinal malrotation with internal hernia. Emergency laparotomy was performed. On the basis of the intraoperative findings, he was finally diagnosed as having an intestinal malrotation and intramesenteric hernia (Treves’ field hernia). The hernia sac and its orifice was located at the ventral part of the superior mesenteric vessels. Right paraduodenal hernia is the most common type of internal hernia associated with intestinal malrotation and shows findings similar to those of intramesenteric hernia in diagnostic imaging. Intramesenteric hernia should be discriminated from paraduodenal hernia by careful intraoperative confirmation.

The incidence of intussusception with pathological lead points in children increases with age. We report two cases of childhood intussusception caused by malignant pathological lead points. [Case 1] A 13-year-old boy was admitted with a one-year history of abdominal pain. Computed tomography demonstrated multiple small bowel tumors and ileocolic intussusception, and tumor resection was performed. Histopathological examination revealed the pathological lead point to be myeloid sarcoma. [Case 2] A 13-year-old boy was admitted with a two-month history of abdominal pain. Computed tomography demonstrated ileocolic intussusception and a small bowel tumor, and we performed tumor resection by laparotomy. Histopathological examination revealed the pathological lead point to be Burkitt lymphoma. Intussusception in older children and recurrent intussusception were reported to be associated with lead points. When lead points cannot be detected by ultrasonography or other imaging modalities, exploratory laparotomy should be considered.

We report two cases of congenital perineal lipoma diagnosed prenatally in female neonates. A female neonate weighing 3,442 g was referred to us for the evaluation of congenital perineal tumor. A fetal perineal mass was detected by fetal ultrasonography at 19 weeks of gestation. She was delivered at 40 weeks and 5 days of gestation. A perineal tumor with pedicule attached to the left side of the anal margin was observed. Another female neonate weighing 3,462 g was referred to us for the evaluation of congenital perineal tumor. A fetal perineal mass was detected by ultrasonography at 30 weeks of gestation. She was delivered at 41 weeks and 3 days of gestation. The perineal tumor extended to the labium majus. She presented with a middle anorectal malformation.

In both patients, the perineal mas was excised 1 month after birth. In the second patient, anorectal malformation repair was performed at 6 months after birth. The histopathological findings showed a lipoma in both patients. Part of the lipoma tended to have vascular tissues and nerves. In the second patient, the lipoma was also observed to have cartilage tissue. In both patients, good progress was obtained by surgical removal. The second patient is doing well and has been defecating spontaneously during four years of follow-up.

Very few reports are available on liver resection with systematic extrahepatic Glissonean pedicle isolation in pediatric surgery. Here, we report the case of a patient with ruptured hepatoblastoma treated by resection of the left lobe with systematic extrahepatic Glissonean pedicle isolation. Case: A 4-year-old male presented to our hospital with lower abdominal pain and frequent vomiting. CT and MRI showed a large tumor that occupied the entire left lobe of the liver. We made a diagnosis of ruptured hepatoblastoma PRETEXT II. Prior to preoperative systematic chemotherapy, the only abnormal finding was mild anemia. However, on the second day of chemotherapy, hepatoblastoma rupture occurred, and emergency left hepatectomy was performed, with systematic extrahepatic Glissonean pedicle isolation safety undertaken. He was discharged from the hospital on the 5th postoperative month with no complications, and there was no recurrence 3 years postoperatively.

Multilocular cystic nephroma (MLCN) has been described as a benign renal tumor. However, imaging modalities, such as CT, MRI, and US, cannot differentiate MLCN from a subset of cystic renal cell carcinomas. Therefore, the majority of MLCN cases are treated by radical nephrectomy. We report a case of MLCN surgically treated. A 13-month-old girl presented with hematuria, which was diagnosed as MLCN with ureterocele on the basis of abdominal CT findings, and her serum CA19-9 level was high. We performed total nephrectomy. Histopathological analysis showed no malignancy. However, the CA19-9 level in the cyst was substantially high.

Sternal cleft is a rare congenital deformity caused by birth defects in the sternum. We report two cases of closure of a simple sternal cleft. Case 1: A 2-year-old girl was referred to our department because of neck protrusion during breathing. Upper sternal cleft was diagnosed, and surgery was performed. Although the perichondrium of the sternal comb was peeled off, the tension was so strong that part of the costal cartilage was cut off. The inferior sternal fusion was excised in a wedge shape. She was discharged eight days after surgery. Case 2: A two-month-old girl was found to have a depression in the suprasternal fossa after birth. She was diagnosed as having complete sternal cleft and was operated on. After detaching and closing the perichondrium, the lower part showed almost no adhesion and was completely transected. A part of the costal cartilage was cut off, and the sternum was closed. Blood pressure decreased at the time of the initial closure, and she was administered catecholamine. She was discharged on the 9th postoperative day. Early surgical stabilization of the thorax is preferred immediately after diagnosis because the thoracic and mediastinal tissues are pliable at the early stage of development. However, the surgical procedure and timing of surgery require careful consideration in neonates and infants in whom respiration and circulation are difficult to manage during the perioperative period.

Tension gastrothorax (TGT), which develops when the stomach herniates owing to congenital diaphragmatic hernia (CDH) into the thorax, can cause massive distension of the stomach caused by trapped air and/or fluid, resulting in mediastinal displacement. Here, we present the cases of three children with TGT due to CDH. Case 1 was a 2-month-old boy with dyspnea. His chest X-ray was misread as tension pneumothorax, and thoracentesis revealed aspiration of stomach content. He was referred to us, and emergent laparotomy with CDH repair was successfully carried out. Case 2 was a 3-year-old boy with abdominal pain and vomiting three days before. He was diagnosed as having gastroenteritis and admitted to a previous hospital. His condition deteriorated, and CT revealed TGT with free air in the thorax. Insertion of a nasograstic (NG) tube failed. During emergency transportation to our institute, he suffered cardiopulmonary arrest (CPA), and resuscitation was started immediately after he reached us. The second CT showed perforation of the stomach and widespread cerebral ischemia, and he died 12 days after the CPA episode. Case 3 was a 4-month-old boy who presented with progressive vomiting. He showed remarkable tachypnea and a preshock state on the next day. CT revealed TGT, and decompression using an NG tube was successful. He was referred to us, and he underwent thoracoscopic repair for CDH. TGT can be misinterpreted as tension pneumothorax or gastroenteritis, and delayed diagnosis may lead to life-threating conditions. Urgent decompression of the stomach using an NG tube is essential for saving life, and if this fails, thoracentesis should be attempted without hesitation.

A boy born after 41 weeks of gestation weighing 2,990 g with anocutaneous fistula underwent anoplasty on day 0. During the operation, the urethra and fistula could not be separated sufficiently, resulting in the anus forming anteriorly. To improve the postoperative constipation due to the malposition of the anus, anterior sagittal anorectoplasty (ASARP) was performed at the age of 3 years, and the anus was reformed through the center of the external anal sphincter. Despite those operations, however, he did not achieve sufficient defecation. At that time, plain X-ray findings showed an elongated sigmoid colon and megarectum. We considered the peristaltic disorders in the elongated and dilated colon as the cause of the severe constipation, and we performed sigmoidectomy with tapering of the megarectum at the age of 5 years. The postoperative course was uneventful, and he was discharged on the 12th postoperative day. During the three-year period after the operation, he defecated almost daily, resulting in a marked improvement of his quality of life (QOL). Resection of the elongated sigmoid colon and megarectum might be very effective in patients with severe constipation and an elongated colon, and in whom other causes were ruled out.

The patient was a 60-day-old female infant. She presented with pale-colored stools and jaundice from 40 days after birth. She was admitted to our hospital because of suspicion of biliary atresia (BA) by her former doctor. She had jaundice and hepatomegaly as physical examination findings, and her laboratory data showed hyperbilirubinemia and liver dysfunction. Abdominal ultrasonography showed an atrophic gall bladder and the triangular cord sign, and biliary scintigraphy revealed no tracer exacerbation in the intestine. Because BA was strongly suspected, a trial laparotomy was performed at 67 days of age. Because she was diagnosed as having this disease (III-b1-ν) on the basis of intraoperative findings of an atrophic gallbladder, a cordlike extrahepatic bile duct, and a connective tissue of the porta hepatis, the Kasai procedure was performed. Heterotopia cartilages were found around the cholangiole in the resected connective tissue of the porta hepatis by histopathological analysis. Case reports of heterotopia cartilage tissue in BA are occasionally found, and it is speculated that developmental abnormalities and cartilage metaplasia due to inflammation may be the pathogenetic factors. However, the mechanisms underlying these abnormalities are as yet unclarified. We summarized the case reports of this disorder with heterotopia cartilage and reviewed the literature.

The coexistence of wandering spleen and gastric volvulus is well known; however, it is unclear whether these conditions can be treated only by splenopexy. We experienced treating two cases of wandering spleen requiring additional gastropexy after splenopexy by the retroperitoneal pouch method. [Case 1] A 3-year-old girl showed agenesis of the splenocolonic ligament and splenorenal ligament along with stretching of the gastrosplenic ligament. The organoaxial gastric volvulus was released, and splenopexy was performed. Anorexia remained, and abdominal X-ray showed a dilated gastric bubble, so gastropexy was additionally performed on post-operative day 43. [Case 2] A 9-year-old boy showed agenesis of all four ligaments and his spleen was fixed by splenopexy. Hiccups and abdominal pain remained; assuming that insufficient gastric volvulus and spontaneous reduction recurred, gastropexy was additionally performed 337 days after the initial operation. In the two cases, performing only splenopexy of the wandering spleen did not resolve the complaints of the patients because the loose organoaxial gastric volvulus remained. Abnormality of the gastrosplenic ligament is recognized as a key substrate of gastric volvulus after the splenopexy. In the treatment of wandering spleen, simultaneous gastropexy seems desirable.

A boy of 4 years and 3 months of age was found to have a tumor in the upper abdominal wall. Further studies revealed that the tumor (4×2 cm) was in the muscle layer and had an unclear boundary. Open biopsy of the tumor led to the diagnosis of desmoid fibromatosis. The tumor was resected with a resection margin of over 1 cm, while preserving the rib bones. Histopathological examination of the resected specimen revealed desmoid fibromatosis with a positive margin. Four months after the first surgery, a local recurrent tumor was found and was resected widely again. Histopathological examination of the resected specimen revealed desmoid fibromatosis with a positive margin again. Therefore, he received adjuvant chemotherapy, low-dose vinblastine and methotrexate. Five months later, local recurrence was found again. Tumor excision, chemotherapy (vincristine, actinomycin D, cyclophosphamide: VAC), and radiotherapy (60 Gy) were performed. The multidisciplinary treatment was completed without major complications, and the residual tumor gradually decreased in size. Seven years later, the tumor had grown to a size of 2 cm. The tumor was resected and radiotherapy (60 Gy) was administered again. However, recurrence was observed outside the irradiation field and additional radiotherapy (50 Gy) was performed.

Mesenteric lipomas are relatively rare, benign tumors in children. We present a case of mesenteric lipoma in a child with symptoms of partial bowel obstruction, diagnosed preoperatively via abdominal ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI). A 7-year-old boy with a history of recurrent abdominal pain and failure to thrive presented with symptoms of vomiting over the last four days. Physical examination revealed slight abdominal distention without a palpable mass. Plain abdominal radiography revealed small bowel loop distention, with a flat, dilated junction segment, indicating mechanical intestinal obstruction. Ultrasonography revealed a heterogenous echogenic mass lesion within the pelvic cavity. CT revealed a well-encapsulated nonenhancing mass with nodular organization and negative attenuation values. T1- and T2-weighted MRI sequences revealed that the mass showed hypersignal intensities and was mobile, which are indicative of mesenteric lipoma. Laparotomy revealed a smooth, round, soft, yellow mass measuring approximately 8.5×8.0×3.0 cm, arising from the mesentery, 80 cm proximal to the ileocecal valve. Resection of the affected segment of the ileum and end-to-end ileal anastomosis were performed. Histopathological analysis showed that the tumor was composed of mature adipocytes without atypia, confirming the diagnosis of mesenteric lipoma. The findings were negative for malignancy. No recurrence was observed during the two-year postoperative follow-up. The clinical presentation of the case was indicative of partial obstruction due to compression with spontaneous detorsion. Mesenteric lipoma should be considered a possible differential diagnosis in children with recurrent abdominal pain and failure to thrive.

Amyand’s hernia is a rare inguinal hernia containing the appendix vermiformis, which is difficult to diagnose preoperatively. We report a case of Amyand’s hernia, which is difficult to distinguish from epididymitis, diagnosed preoperatively by Computed Tomography. A 1-year-3-month-old boy was brought to our hospital presenting with distension of the right groin, redness, and swelling of the right scrotum. We diagnosed him as having epididymitis and started conservative treatment with oral antibiotics, but there was no improvement in the patient’s condition. On the 6th day of illness, to investigate the funicular structure that we found in the inguinal canal by ultrasonography, we carried out CT and diagnosed him as having Amyand’s hernia with appendicitis. We performed appendectomy and inguinal hernia repair by the inguinal approach, and we successfully kept the intraperitoneal space free from infection. Although a review of the echography images obtained earlier showed an incarcerated appendix vermiformis in the inguinal canal as a narrow funicular structure, we considered that echography was insufficient for the diagnosis of Amyand’s hernia at the first medical examination and CT was needed for preoperative diagnosis. The inguinal approach was effective in preventing the spread of infection inside the abdomen.

A 30-day-old female infant was admitted to our hospital with symptoms including persistent jaundice and heart failure caused by patent ductus arteriosus (PDA). She was delivered at a gestational age of 37 weeks and 4 days with a birth weight of 2,160 g. No abnormalities were detected on fetal ultrasonography at the gestational age of 28 weeks. Physical examination showed jaundice and blood examination showed elevated levels of biliary enzymes. Abdominal ultrasonography revealed a cystically dilated common bile duct without dilatation of the intrahepatic bile duct. In addition, a large protein plug 18 mm in diameter was found in the cyst. Congenital biliary dilatation (Todani type Ia) with a protein plug was diagnosed. Nine days after admission, the patient underwent PDA ligation; after which, her jaundice worsened. To treat the jaundice and dissolve the protein plug, ursodeoxycholic acid and bromhexine hydrochloride were administered. A radical operation was performed at 75 days of age (weight: 3,300 g). Intraoperative cholangiography showed pancreaticobiliary maljunction and that the common duct measured 12 mm. There was no protein plug in the cyst. These findings suggest that bromhexine hydrochloride is effective for preoperative management and dissolving protein plugs in neonatal cases with congenital biliary dilatation.