A case of sacrococcygeal teratoma associated with antenatally acquired urethrovaginal fistula and hydrocolpos.

BACKGROUND: Sacrococcygeal teratomas (SCTs) are known to cause urological complications, but urethrovaginal (UV) fistula as a complication of SCT is rare. We herein report a case of SCT with UV fistula and hydrocolpos. CASE PRESENTATION: A 1-day-old female neonate presented to our department with prominent swelling in the sacrococcygeal region. She was born at 37 gestational weeks via spontaneous vaginal delivery from a 39-year-old woman. The weight of the baby was 2965 g, and her Apgar scores were 4/10 (at 1 and 5 min). An MRI examination confirmed an 11 × 11 cm Altman classification typeII SCT associated with hydrocolpos, a dilated urinary bladder, and bilateral hydronephrosis. When she was 5 days, the SCT was excised totally and a coccygectomy was performed. After the operation, as her urinary output appeared unstable, a cystoscopic examination was performed on the third postoperative day. This revealed that the UV fistula was located approximately 1 cm from the urethral opening. In addition, the proximal urethra was unobstructed and connected to the bladder. The cystoscope allowed for the passage of a urinary catheter through the urethra. After 1 month of catheter placement, she was discharged from the hospital at 57 days of age. Follow-up was uneventful, with neither urinary infection nor retention. CONCLUSIONS: SCTs are associated with not only trouble with rectal function and lower extremity movement but also urinary complications. The pathogenesis of this UV fistula is thought to be the rapid growth of the SCT that developed in the fetal period, resulting in obstruction of the urethra by the tumor and the pubic bone, which in turn caused urinary retention and the formation of a fistula as an escape route for the pressure. Because SCTs can cause a variety of complications depending on the course of the disease, careful examination and follow-up are necessary.

Proctoring System of Pediatric Laparoscopic Surgery for Choledochal Cyst.

Background: Endoscopic surgery also has been becoming widespread in the field of pediatric surgery. However, most disease treated by pediatric surgery in a single institution are small number of cases. Besides, the variety of operative procedures that need to be performed in this field is quite wide. For these reasons, pediatric surgeons have limited opportunities to perform endoscopic surgery. Therefore, it is difficult to introduce advanced endoscopic surgery at a single local hospital. To educate pediatric surgeons in local hospitals, for widespread advanced pediatric endoscopic surgery safely, and to eliminate the need for patient centralization, we have introduced a proctoring system. We compared the surgical results of our institution, a center hospital, with other local institutions, to investigate the feasibility of our proctoring system. Methods: The experienced pediatric surgeon of our institution visits local hospitals to provide onsite coaching and supervises pediatric surgeons on the learning curve. All patients who underwent laparoscopic cyst excision and hepaticojejunostomy for choledochal cysts, one of the advanced pediatric endoscopic surgeries was retrospectively reviewed. Results: Thirty-four cases were evaluated (14 cases in our institution, 20 cases in 9 other institutions). The procedures of all 34 cases were performed by surgeons with 0-2 cases of experience in the procedure. There were no open conversion cases. There was no significant difference in the operative date. There was 1 case (6.7%) of postoperative complications during hospitalization at our institution and 3 cases (14.3%) at other institutions (P = .47). Two cases of late complications (13.3%) occurred at our institution, whereas 6 cases (28.6%) occurred at other institutions (P = .28). Conclusion: With the proctoring system, the performance and completion of advanced pediatric endoscopic surgery at local institutions was feasible. This has important implications given the ever-growing demand for pediatric endoscopic surgery and the increasing need for competent pediatric endoscopic surgeons.

Anterior mediastinal tracheostomy for severe tracheal stenosis in a child with severe motor and intellectual disabilities: a case report.

BACKGROUND: Owing to recurrent aspiration pneumonia and airway stenosis secondary to thoracic deformities, tracheostomy or laryngotracheal separation are often necessary in children with severe motor and intellectual disabilities. However, these procedures are associated with the risks of tracheal stenosis due to tracheal granulation and tracheoinnominate artery fistula formation. We report a case of a child with severe motor and intellectual disabilities treated with an anterior mediastinal tracheostomy. CASE PRESENTATION: The patient was a 15-year-old boy with severe motor and intellectual disabilities due to intractable epilepsy. Due to thoracic deformity and tracheomalacia, the patient had a flattened and narrowed trachea. Accordingly, laryngotracheal separation was performed 4 months before admission to avoid aspiration pneumonia. Due to a common cold, the patient required frequent sputum suctioning, which exacerbated the tracheal stenosis. Bronchoscopy revealed tracheal stenosis 4-5 cm caudal to the tracheostomy site, tracheal mucosal ulcers, and pulsation of the innominate artery on the anterior wall of the trachea. We performed an anterior mediastinum tracheostomy to release the tracheal stenosis and prevent tracheoinnominate artery fistula formation. CONCLUSIONS: Anterior mediastinal tracheostomy has several advantages. Including sufficient release of bony compression, release of tracheal hyperextension, and relief of tracheal and innominate artery contact ensures a cannula-free tracheostomy, and there is no need to dissect the brachiocephalic artery. It is the procedure of choice in cases of head and neck malignancies requiring extensive tracheal resection and could be a good surgical option for severe tracheal stenosis and tracheoinnominate artery fistula in children with severe motor and intellectual disabilities.

Feasibility of Laparoscopic Fundoplication Without Removing the Preceding Gastrostomy in Severely Neurologically Impaired Patients: A Multicenter Evaluation of the Traction Technique.

Purpose: Severely neurologically impaired patients sometimes require anti-reflux surgery with preceding gastrostomy. We apply a traction technique for laparoscopic fundoplication (LF) without gastrostomy takedown (GTD) in such cases. We conducted a multicenter review to assess the feasibility of our approach. Materials and Methods: In brief, the traction technique involves left-lateral-traction of the stomach body, right-lateral-traction of the round ligament of the liver, and elevation of the left liver lobe to create a sufficient field for manipulating the forceps. Patients who underwent LF with Nissen's procedures in 2010-2022 were retrospectively reviewed. Data were analyzed by a one-way analysis of variance. Results: The operative approaches included the traction technique (n = 16; Group 1), GTD and reconstruction (n = 5; Group 2), and LF followed by gastrostomy (n = 92; Group 3). In comparison with Group 1, significant differences were only found in pneumoperitoneum time (Group 1 versus Group 2 versus Group 3: 174.4 minutes versus 250.4 minutes versus 179.5 minutes; P = .0179). Operating time (222.7 minutes versus 303.0 minutes versus 239.7 minutes; P = .0743), duration to full-strength enteral nutrition (10.4 days versus 17.2 days versus 11.0 days; P = .0806), and length of hospital stay (17.2 days versus 31.0 days versus 18.5 days; P = .3247) were equivalent. No re-fundoplication was required in Group 1. Conclusion: The traction technique secures the operative quality and outcome of LF without GTD.

Thoracoscopic intraoperative esophageal close technique for long-gap esophageal atresia.

Long-gap esophageal atresia (EA) remains a challenging operative procedure. Several techniques have been reported to resolve the problem of distance between the proximal and distal esophagus. We report a thoracoscopic intraoperative esophageal close technique for long-gap EA. A female neonate was born at 39 weeks of gestation with long-gap EA (five vertebrae). The patient underwent thoracoscopic esophageal anastomosis after esophageal elongation. Careful dissection of the proximal and distal esophagus was performed; however, the distance between them was still long. Extra-thoracic traction sutures were placed at the upper esophagus. Then, a Neraton catheter was inserted trans-orally and the upper esophagus was pushed and opened. An internal traction suture was placed between the Neraton catheter and lower esophagus. The gap between the proximal and distal esophagus could be approximated by pulling the Neraton catheter. Esophageal anastomosis was performed successfully. Our thoracoscopic intraoperative esophageal close technique was simple and useful.

Laparoscopic-assisted Stamm-gastrostomy: technical modifications to ease suturing inside the minimal trocar site.

Laparoscopic gastrostomy tube placement has been increasingly adopted by pediatric surgeons. We herein report our experience with the performance of a laparoscopic-assisted Stamm-operation inside the minimal trocar site without the extension of the trocar site incision or mini-laparotomy. We present some technical modifications that facilitate suturing inside the minimal trocar site. METHODS: A retrospective chart review was conducted of cases involving patients who underwent laparoscopic-assisted gastrostomy, using a simple extracorporeal method inside the trocar site from April 1998 to March 2018. RESULTS: One hundred five gastrostomy tubes were placed in a laparoscopic-assisted operation. All but two of the cases involved patients with neurological impairment. The mean age was 12.5 years; 28 cases were > 16 years of age. Seventy-five cases underwent gastrostomy during laparoscopic fundoplication. All gastrostomy procedures were completed without intraoperative difficulties; however, 8 cases, which involved a thick abdominal wall, required extension of the trocar site. No cases required conversion to open gastrostomy. No major complications were observed. Two patients developed continuous peristomal cellulitis after surgery, due to the mismatch of the site position and an unsuitable button device size. CONCLUSION: We demonstrated that laparoscopic gastrostomy with fully extracorporeal suturing within the trocar site is feasible and beneficial, especially for the most neurologically impaired pediatric cases. Technical modification, changing the order of the process, and suturing technique in the minimal space, made it easier to perform the procedures inside the minimal trocar hole.

Laparoscopic fundoplication for a case of esophageal hiatal hernia after gastroschisis repair.

BACKGROUND: Esophageal hiatal hernia and gastroesophageal reflux have been recognized as inevitable complications after the definitive gastroschisis operation. Patients with refractory gastroesophageal reflux require anti-reflux surgery; however, the surgical adhesions may complicate subsequent surgical therapy, especially in the cases treated by staged repair. CASE PRESENTATION: A male infant who showed a severe gastroesophageal reflux due to hiatal hernia after staged abdominal fascial closure of gastroschisis. In spite of continuous conservative management, frequent vomiting and hematemesis had become progressively worse at the age of 8 months. Laparoscopic Nissen fundoplication was attempted and completed with no adverse events. CONCLUSIONS: Laparoscopic fundoplication may be applied, as a first-line approach, for the treatment of gastroesophageal reflux in this difficult group of patients, after the repair of congenital abdominal wall defect.

Portal venous gas in intestinal malrotation with mild midgut volvulus.

BACKGROUND: Portal venous gas has been considered as a radiological sign requiring urgent operative intervention; however, the reports concerning portal venous gas associated with favorable outcome are recently increasing. CASE PRESENTATION: We describe a 9-month-old boy with acute onset high fever and vomiting. The ultrasonography demonstrated micro-gas bubbles continuously floating in the intrahepatic portal vein. Contrast-enhanced CT, performed 1 h later from echography, revealed a whirlpool sign suggesting an intestinal malrotation with midgut volvulus, but with no signs of residual intrahepatic gas. Operative findings showed a mild volvulus with neither congestion nor ischemic change of the twisted bowel. Detorsion and Ladd's procedure were completed laparoscopically. CONCLUSIONS: Transient portal venous gas bubbles may be generated even in the mild intestinal volvulus with no bowel ischemia. Ultrasonography can be a sensitive detector to visualize such small amounts of gas.

Blowhole tangential cecostomy and transanal tube insertion for neonatal cecal perforation in a patient with Hirschsprung's disease in the earlier definitive operation era.

BACKGROUND: Although uncommon and seldom experienced, intestinal perforation is a well-known complication of Hirschsprung's disease (HD). A literature review revealed that the cecum, including the appendiceal base, is a site of perforation. The cecum is not suitable for making an ordinary loop colostomy, and the optimal operative strategy remains to be established. CASE PRESENTATION: We present a combination technique composed of tangential cecostomy at the perforated portion and postoperative care with a transanal indwelling tube, which was used in the treatment of a 3-day-old boy with cecal perforation with long-segment Hirschsprung's disease. A temporary simple blowhole stoma and continuous decompression with daily irrigation via a transanal indwelling tube in the distal colon achieved a secure recovery and was followed by a definitive operation in the early period. The combination of tangential cecostomy and transanal indwelling catheter management led to the preservation of the ileocecal valve. CONCLUSIONS: We review the Japanese literature and emphasize the usefulness of this combination technique by blowhole tangential cecostomy and transanal tube insertion for neonatal cecal perforation in patients with HD in today's early definitive operation era.

Long-term outcome of peroral endoscopic myotomy for achalasia treatment in a 9-year-old female patient.

Achalasia is a primary motility disorder with incomplete lower esophageal sphincter relaxation; it has an annual incidence of 0.11 cases per 100 000 children. Peroral endoscopic myotomy (POEM) is a new endoscopic treatment method for achalasia. Reports about POEM in pediatric patients are rare. We herein report the case of a 9-year-old female patient with achalasia who underwent POEM. The patient underwent endoscopic balloon dilatation because medication was not effective at a previous hospital; however, endoscopic balloon dilatation was not effective either. She then underwent successful POEM upon admission at our hospital. The patient was symptom-free at 2 years postoperatively with no signs of esophagitis in the absence of proton-pump inhibitor therapy.

Using an indwelled test tube as a good "navigator" during enterostomy closure.

Surgeons often have difficulty in identifying a suitable incision line to enter the peritoneal cavity for stoma mobilization during enterostomy closure. A mini-size test tube that is preoperatively placed into the stoma can act as an efficient guide in finding a free area to enter the peritoneal cavity, by supplying efficient counter traction and a palpable marker of the intestinal wall.

Hemi-hepatectomy in pediatric patients using two-surgeon technique and a liver hanging maneuver.

AIM: To evaluate the efficacy of the two-surgeon technique with the liver hanging maneuver (LHM) for hepatectomies in pediatric patients with hepatoblastoma. METHODS: Three pediatric patients with hepatoblastoma were enrolled in this study. Two underwent right hemi-hepatectomies and one underwent a left hemi-hepatectomy using the two-surgeon technique by means of saline-linked electric cautery (SLC) and the Cavitron Ultrasonic Surgical Aspirator (CUSA; Valleylab, Boulder, CO) and the LHM. RESULTS: The mean operative time during the parenchymal transections was 50 min and the mean blood loss was 235 g. There was no bile leakage from the cut surface after surgery. No macroscopic or microscopic-positive margins were observed in the hepatic transections. CONCLUSION: The two-surgeon technique using SLC and CUSA with the LHM is applicable to even pediatric patients with hepatoblastoma.

Skill qualifications in pediatric minimally invasive surgery.

PURPOSE: In 2006, The Japanese Society of Pediatric Endoscopic Surgeons devised a plan to develop a pediatric endoscopic surgical skill qualification (ESSQ) system. This system is controlled by The Japan Society for Endoscopic Surgery. The standard requirement for skills qualification is the ability of each applicant to complete common types of laparoscopic surgery. The main goal of the system is to decrease complications of laparoscopic surgery by evaluating the surgical skills of each applicant and subsequently certify surgeons with adequate skills to perform laparoscopic operations safely. METHODS: A committee of pediatric ESSQ created a checklist to assess the applicant's laparoscopic surgical skills. Skills are assessed in a double-blinded fashion by evaluating an unedited video recording of a fundoplication for pediatric gastroesophageal reflux disease. RESULTS: The initial pediatric ESSQ system was started in 2008. In 2008 and 2009, respectively, 9 out of 17 (53%) and 6 out of 12 (50%) applicants were certified as expert pediatric laparoscopic surgeons. CONCLUSIONS: Our ultimate goal is to provide safe and appropriate pediatric minimally invasive procedures and to avoid severe complications. To prove the predictive validity of this system, a survey of the outcomes of operations performed by certified pediatric surgeons is required.

Association of lymphocyte crossmatch and the outcome of intestinal transplantation in swine.

BACKGROUND: The effect of preformed antidonor antibodies have been demonstrated in various types of solid organ transplantation. However, the significance of anti-donor antibodies in intestinal transplantation remains unclear. The aim of this study is to evaluate the impact that the extent of T cell crossmatch has on the outcome of swine intestinal transplantation. MATERIALS AND METHODS: All studies were performed on outbred domestic male pigs weighing from 15 to 20 kg. Intestinal transplantation was performed orthotopically with an exchange of grafts between white and black pigs. FK506 was administered intravenously (0.1 mg/kg per day, POD 0-7) for immunosuppression. A lymphocyte crossmatch test was performed using the direct CDC crossmatch. The results were considered positive when more than 10% of the donor lymphocytes were killed by the recipient's serum. In addition, 0-10, 11-20, 21-30, 31-80 and 81-100% of the killed lymphocytes were classified as grade 1, 2, 4, 6 and 8, respectively. RESULT: A total of 34 intestinal transplantations were performed. All but one case had positive donor specific T cell crossmatches. The number of grade 2, 4, 6 and 8 cases was 11, 14, 6 and 2, respectively. Although there was a tendency towards a decreased survival according to the grade, the survival rate was not statistically different among each different grade. Moreover, the rates of acute cellular rejection and vascular complications were not significantly different among the four grades. CONCLUSION: These results suggest that the extent of positive T cell crossmatch is not associated with the outcome of swine intestinal transplantation.

Giant cystic meconium peritonitis associated with a cloacal anomaly: case report.

This report describes a case of giant cystic meconium peritonitis (GCMP) associated with a cloacal anomaly. Antenatal ultrasonography and magnetic resonance imaging demonstrated persistent fetal ascites, bilateral hydronephrosis, and 3 pelvic cystic structures. The baby girl showed duplicated hydrocolpos and a single orifice of the cloaca with a long common channel inducing a urinary outflow obstruction. After constructing a diversion colostomy, a cutaneous vesicostomy was necessary to prevent recurrent urinary tract infections. These findings are consistent with a prenatal diagnosis of cloacal anomalies, thus suggesting an association with severe obstruction of lower urinary tract and meconium peritonitis. Most of reported cases of meconium peritonitis associated with the cloaca show fibroadhesive types with scattered intraperitoneal calcifications and adhesions. However, the present case showed a rare GCMP suggesting continuous urinary influx via the fallopian tubes until the later stage of intrauterine life.

The influence of rejection on graft motility after intestinal transplantation in swine: the possibility of using this method for the real-time monitoring of acute cellular rejection.

BACKGROUND: We have previously reported that rejected allografts show dysmotility, which can be detected by real-time monitoring in swine. We examined the correlation between the motility and the mucosal histology to detect rejection at an early stage by real-time monitoring. METHODS: Intestinal transplantation was performed orthotopically using FK506. The distal segment of the allograft measuring about 20 cm was isolated and exteriorized as "Thiry-Vella" stoma for biopsies. Strain-gage force transducers were attached on a graft for the real-time monitoring of graft motility. The pigs without intestinal transplantation were used as controls (C). The rejection was classified into 4 groups based on the histologic findings: nonrejection, mild rejection, moderate rejection, and severe rejection. Migrating motor complex (MMC) phase 3 was estimated by the following parameters: duration, amplitude, interval, motility index, velocity, and frequency of the propagation. RESULTS: In the nonrejection group, all parameters were almost the same as in C group. In contrast, in the moderate rejection and severe rejection groups, most of the parameters were significantly lower than those in the C group. In the mild rejection group, the contractility of the MMC was not significantly altered, but the frequency of the propagation decreased significantly. CONCLUSIONS: The graft motility detected by the real-time strain-gage method correlated closely to the grade of mucosal histology. This method is therefore considered to be useful for detecting rejection at an early stage by examining the frequency of MMC propagation.

A new method of transanal catheter fixation for neonates with long segment-type Hirschsprung's disease.

We managed long-segment aganglionosis in two neonates by performing colonic irrigation through an indwelling transanal catheter for 65 and 30 days, respectively, until a laparoscopy-assisted primary pull-through operation could be performed. The catheter was fixed by tying it to the tubes, criss-crossed at the anus, and securing the opposite ends of the tubes away from the diaper. This form of management with our devised method of transanal catheter fixation and simple frequent tube washouts improved the preoperative quality of life of both the babies and their parents remarkably.

Massive bloody stools in two neonates caused by cow's milk allergy.

Two neonatal cases of cow's milk allergy showing bilous vomiting and massive bloody stool are presented. In both cases, laboratory data on admission showed no eosinophilia. The immediate upper gastrointestinal series of both cases demonstrated no evidence of intestinal malrotation, but revealed a narrowing of the duodenum and upper jejunal loops and marked stasis of barium in one case, suggesting an edema with inflammation. Rectal mucosal punch biopsy of the other case showed eosinophilic infiltration in the lamina propria. These findings suggested an allergic origin, useful information for avoiding confusion with some diseases requiring emergency surgery. In both cases, bleeding resolved soon after elimination of cow's milk from the diet. Thereafter both cases showed a moderate to marked increase in peripheral eosinophilia, while serum radioallergosorbent tests for cow's milk protein were negative. Definitive diagnosis of allergic enterocolitis is difficult because there is neither specific laboratory data nor tests. Allergic enterocolitis can occur in the early neonatal period and should be considered in differential diagnosis of any newborn developing gastrointestinal bleeding.

Z-shaped anastomosis for the treatment of a benign rectosigmoid stricture.

A girl with malrotation underwent a Ladd's operation at 35 days of age and later had an adhesiotomy at 115 days of age. After the adhesiotomy, she suffered from sepsis and subsequent disseminated intravascular coagulation (DIC). At 178 days of age, she developed an intestinal obstruction because of a rectosigmoid stricture probably caused by DIC-associated ischemia. As a result, an ileostomy was performed. At one year, 4 months of age, she underwent a resection of the proximal part of the rectosigmoid stricture and a reconstruction by means of a Z-shaped anastomosis. Based on our experience, Z-shaped anastomosis appears to be an excellent treatment not only for Hirschsprung's disease but also for benign rectosigmoid stricture.