Malignant Phyllodes Tumor in a Pubertal Girl: A Report of a Case.

BACKGROUND: Malignant phyllodes tumor (MPT) is a rare breast disease that is extremely rare in children. A few cases of pediatric malignant phyllodes tumors have been reported, including some with a poor prognosis. CASE: A 14-year-old girl presented with a growing lump on her right breast. On the basis of imaging tests and a core needle biopsy, MPT was diagnosed, and right mastectomy was performed. The postoperative course was uneventful. SUMMARY AND CONCLUSION: MPT is an infrequent disease in adult females and is extremely rare in pubertal females. It occasionally shows rapid growth, metastasis, and recurrence with a poor prognosis. Early surgical resection is necessary to obtain a cure. When a rapidly growing breast tumor is observed in pubertal females, MPT should be considered.

Low placental weight may be involved in the etiology of congenital cryptorchidism in neonatal boys.

INTRODUCTION: Several factors have been reported to be associated with the etiology of cryptorchidism; however, clear evidence regarding the risk factors for cryptorchidism is elusive. In the present study, we evaluated the clinical characteristics of cryptorchidism using the common neonatal intensive-care unit (NICU) database of the National Hospital Organization and explored one of possible factors associated with the development of cryptorchidism. METHODS: A total of 7882 male neonates were included in this study. We separated them into two groups: those without cryptorchidism (n = 7852) and those with cryptorchidism (n = 30) at the time of discharge from the NICU. Cryptorchidism was defined as a condition in which the testis was located out of the scrotum on the route of descent at the time of NICU discharge. The associations between cryptorchidism and the maternal, placental, and neonatal information were analyzed. Analyses were performed statistically to compare nominal variables between the groups using Fisher's direct establishment calculation method and logistic regression analyses. RESULTS: Univariate analyses showed the placental weight <10% tile (odds ratio 3.31, 95% confidence interval [CI] 1.18-8.64), birth height <-2 standard deviations (SD) (odds ratio 3.65, 95% CI 0.92-10.6), birth weight <-2SD (odds ratio 4.06, 95% CI 1.55-9.51), and small for gestational age (odds ratio 3.82, 95% CI 1.46-8.97) were significantly associated with the development of cryptorchidism. Multivariate analyses showed that placental weight <10th percentile (odds ratio 2.86, 95% CI 1.11-7.44) was significantly associated with the development of cryptorchidism. DISCUSSION: Although, this study population was limited to infants admitted to the ICU, the data indicated a possible association between low placental weight and the development of cryptorchidism in neonatal boys.

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.

Utility of Positional Instillation of Contrast Cystography for Diagnosing Occult Vesicoureteral Reflux in Children: A Report of Two Cases.

Positional instillation of contrast (PIC) cystography is effective for detecting occult vesicoureteral reflux (VUR), which can not be revealed by standard voiding cystourethrography (VCUG). We experienced two cases of young female patients; one had repeated urinary tract infection with a negative VUR on standard VCUG, and the other had findings suggestive of reflux hydronephrosis and intolerance of standard VCUG. They underwent PIC cystography, and occult VUR was detected in both cases. Both were successfully treated with simultaneous endoscopic injection therapy with dextranomer/hyaluronic acid. PIC cystography is useful for detecting occult VUR in children with negative VUR findings on standard VCUG or who are unable to tolerate standard VCUG.

Abnormal placental cord insertion, hypertensive disorders of pregnancy and birth length may be involved in development of hypospadias in male fetuses.

OBJECTIVES: Hypospadias is a congenital disease characterized by morphological abnormalities of the penis, including abnormal urethral opening and penile flexion, which cause urination disorders and/or sexual intercourse difficulty. Various factors have been suggested to cause this anomaly, but evidence concerning risk factors causing this anomaly is insufficient. We evaluated the etiology of hypospadias in Japan using the Common Database of the National Hospitals' Neonatal study group. STUDY DESIGN: We retrospectively evaluated 7,865 male neonates registered in the NICU Common Database of the National Hospitals' Neonatal study group. The subjects were divided into two groups by the presence (n = 43) or absence (n = 7,822) of hypospadias. Statistical analyses were performed to compare nominal variables between the groups by Fisher's direct establishment calculation method and logistic regression analyses. RESULTS: A univariate analysis showed significant between-group differences in hypertensive disorders in pregnancy (odds ratio [OR]: 4.02, 95% confidence interval [CI]: 1.95-7.90), placental weight <-1.28 standard deviation (SD; OR: 5.06, 95% CI: 2.45-10.32), abnormal placental cord insertion (OR: 4.7, 95% CI: 2.62-9.76), birth length <-2SD (OR: 10.56, 95% CI: 5.00-21.1) and birth weight <-2SD (OR: 8.17, 95% CI: 4.17-15.68). A multivariate analysis showed a significant between-group difference in hypertensive disorders of pregnancy (adjusted OR [AOR]: 2.30, 95% CI: 1.09-4.85), abnormal placental cord insertion (AOR: 3.69, 95% CI: 1.83-7.44) and birth length <-2SD (AOR: 3.44, 95% CI: 1.26-9.42). CONCLUSION: Abnormal placental cord insertion, hypertensive disorders of pregnancy and birth length may be involved in hypospadias development in male neonates in conjunction with placental dysfunction in early pregnancy.

Late Occurrence of the Tubo-Ovarian Abscess after Appendectomy for Perforated Appendicitis in a Virginal Adolescent Girl.

BACKGROUND: Tubo-ovarian abscess (TOA) is generally seen in sexually active women. It is rarely reported in virginal adolescent girls. CASE: A 12-year-old virginal girl was referred to us for repeated fever and right lower abdominal pain. She had undergone an appendectomy for a perforated appendix with abscess 5 years previously. Laparoscopic surgery revealed pelvic adhesions associated with TOA in the right pelvis. At 2 months after laparoscopic drainage, she underwent resection of the affected tube with wedge resection of the ipsilateral ovary due to the recurrence of TOA. SUMMARY AND CONCLUSION: Late occurrence of TOA should be considered in the differential diagnosis of repeated abdominal pain and fever in virginal adolescent girls with a history of appendectomy for complicated appendicitis, even if the history is remote.

Clinical Utility of the Meal Tolerance Test in the Care of Patients with Type 2 Diabetes Mellitus.

Objective The measurement of C-peptide immunoreactivity (CPR) is essential for evaluating the pancreatic ß-cell function and selecting appropriate therapeutic agents in patients with diabetes mellitus. The meal tolerance test (MTT) is simple to administer physiological insulin-stimulating test. Previous studies have reported that several CPR-related indices are useful markers for predicting insulin requirement in type 2 diabetes. In the present study, we investigated the serum CPR response during the MTT in hospitalized patients with type 2 diabetes mellitus in order to clarify the clinical utility of the MTT. Methods We performed the MTT using a test meal with timed measurements of the serum CPR level based on the oral glucose tolerance test over 180 minutes and tested the correlation of various CPR-related indices and clinical factors in patients with type 2 diabetes mellitus. Patients The subjects were patients with type 2 diabetes mellitus who had been admitted to our hospital for diabetes management and education. The final study population consisted of 68 patients. Results The fasting CPR level was correlated with the 24-hour urinary CPR excretion and body mass index. The serum CPR level at 120 minutes in the MTT was strongly correlated with the area under the curve of CPR during the MTT. The patients who needed insulin therapy at 6 months after hospitalization showed a significant lower incremental CPR value from 0 to 120 minutes in the MTT than those who did not need insulin therapy. Conclusion The plasma C-peptide levels at 0 and 120 minutes in the MTT provide essential information for the clinical management of patients with type 2 diabetes mellitus.

Laparoscopic Abdominoscrortal Hydrocele: A Case Series.

OBJECTIVE: To evaluate the effect of laparoscopic percutaneous extraperitoneal closure (LPEC) of the internal inguinal ring for the treatment in pediatric abdominoscrotal hydrocele (ASH) and to assess the feasibility and safety of the procedures. PATIENTS AND METHODS: Data were collected from the charts of patients with ASH who underwent surgery in Kokura Medical Center from April 2014 to December 2019. The patients' characteristics, preoperative diagnosis, forms of abdominal components, presence of patent processus vaginalis (PPV), associated pathologies, and postoperative results were evaluated. RESULTS: The study population included 10 patients (4.3% of all 230 hydroceles). The mean age of 10 patients was 3.5 years (range, 7 months to 7 years). A preoperative diagnosis of ASH was made in 3 patients. In the other 7 patients, ASH was detected during laparoscopic repair of the scrotal hydrocele. The abdominal forms of hydrocele were monolocular cysts (n = 6) and multilocular cysts (n = 4). PPV was detected by laparoscopy in all cases. Six patients had contralateral pathologies, including PPV (n = 4), inguinal hernia (n = 1), and scrotal hydrocele (n = 1). One patient had ipsilateral undescended testis. Preoperative ultrasonography showed some degree of testicular dysmorphism on the affected side in 4 cases. In all cases, treatment was accomplished by closing the PPV at the internal inguinal ring by LPEC procedures. No patients had postoperative complications, including recurrent ASH or hydrocele after ASH repair (mean follow-up, 2.6 years). CONCLUSION: LPEC may be an adequate and minimally invasive method for the treatment of the pediatric ASH.

Congenital hyperinsulinism associated with Hirschsprung's disease-a report of an extremely rare case.

BACKGROUND: Congenital hyperinsulinism (CH) is a rare disease, characterized by severe hypoglycemia induced by inappropriate insulin secretion from pancreatic beta-cells in neonate and infant. Hirschsprung's disease (HD) is also a rare disease in which infants show severe bowel movement disorder. We herein report an extremely rare case of combined CH and HD. CASE PRESENTATION: The patient was a full-term male infant who showed poor feeding, vomiting, and hypotonia with lethargy on the day of birth. He was transferred to tertiary hospital after a laboratory analysis revealed hyperinsulinemic hypoglycemia. The patient showed remarkable abdominal distension without meconium defecation. An abdominal X-ray showed marked dilatation of the large bowel. He was diagnosed with CH (nesidioblastosis) associated with suspected HD. He was initially treated with an intravenous infusion of high-dose glucose with the intermittent injection of glucagon. This was successfully followed by treatment with diazoxide and octreotide (a somatostatin analog). At 8 months of age, HD was confirmed by the acetylcholinesterase staining of a rectal mucosal biopsy specimen, and a transanal pull-through operation was performed to treat HD. At 14 months of age, subtotal pancreatectomy was performed for the treatment of focal CH located in the pancreatic body. His postoperative course over the past 12 years has been uneventful without any neurologic or bowel movement disorders. CONCLUSIONS: Although it is extremely rare for CH to be associated with HD, associated HD should be considered when a patient with CH presents severe constipation.

Emergent laparoscopic dome resection and omental suturing to the splenic parenchymal edge for a spontaneously ruptured non-parasitic large splenic cyst in a pediatric patient: a case report.

BACKGROUND: Spontaneously ruptured large splenic cyst is a rare in children, and traditionally total or partial splenectomy has been performed for treating an emergent case. We herein present a first case with spontaneously ruptured pediatric splenic cyst treated with emergent laparoscopic dome resection with omental suturing to the parenchymal edge of the spleen. CASE PRESENTATION: A 12-year-old girl with a spontaneously ruptured large non-parasitic splenic cyst (SC) was successfully treated by emergent laparoscopic dome resection with omental suturing to the edge of the splenic parenchyma. The patient presented with acute abdominal pain and was diagnosed with a ruptured non-parasitic SC and peritonitis by contrast-enhanced computed tomography (CT). Emergent laparoscopic dome resection of the SC and omental suturing to the splenic parenchymal edge were then performed. The protruding part of the cyst wall was completely resected using an ultrasonically activated device (USAD), and the greater omentum was then sutured to the anterior edge of the splenic parenchyma under a laparoscopic view. No complications were observed during the operation. A histological examination revealed a congenital splenic cyst lined by epithelial cells. The postoperative course was uneventful, and an ultrasound scan showed no evidence of cyst recurrence at 3 years after the operation. CONCLUSIONS: This minimally invasive laparoscopic procedure was feasible and effective for treating a ruptured large splenic cyst in an emergent pediatric patient.

The Safety and Efficacy of Laparoscopic Percutaneous Extraperitoneal Closure for Inguinal Hernia in Neonates and Infants Younger Than 1 Year of Age in Comparison to Older Patients.

PURPOSE: Laparoscopic percutaneous extraperitoneal closure (LPEC) has been performed in Japan for the repair of the pediatric inguinal hernias for over a decade. However, the safety and efficacy of LPEC in neonates and infants under 1 year of age remain unknown. The aim of the present study is to elucidate the safety and efficacy of LPEC in the treatment of inguinal hernia in patients who are younger than 1 year of age. PATIENTS AND METHODS: The medical records of the patients who underwent LPEC at Saga-Ken Medical Center Koseikan (Saga, Japan) between August 2007 and November 2012 were collected. The intraoperative findings and postoperative outcomes were retrospectively investigated. The data of the patients who were younger than 1 year of age (Group A) were compared with the data of patients who were older than 1 year of age (Group B). RESULTS: During the study period, 150 LPEC procedures were performed in 112 Group A patients, whereas 607 LPEC procedures were performed in 456 Group B patients. There were no serious complications in either group. After a mean follow-up period of 50.4 ± 15.6 months (range, 28-91 months), there were no significant differences between the two groups in the operating time or the incidence of intraoperative or postoperative complications. Postoperative testicular ascent and recurrence were observed in some cases of each group. CONCLUSIONS: LPEC is a safe and effective procedure for the repair of an inguinal hernia, even in neonatal and infant patients who are younger than 1 year of age.

Changes in uncuffed endotracheal tube leak during laparoscopic inguinal herniorrhaphy in children.

The present study was conducted to investigate changes in uncuffed endotracheal tube (ETT) leak during laparoscopic surgery. The study included 31 patients aged between 1 and 6 years scheduled for elective laparoscopic inguinal herniorrhaphy. Inspiratory and expiratory tidal volumes (TVi and TVe) were measured during mechanical ventilation, and ETT leak was calculated using the formula-ETT leak = (TVi - TVe)/TVi × 100 (%), assessed at the following time-points-5 min after the start of mechanical ventilation (T1, baseline), just before the start of surgery (T2), 5 min after the induction of pneumoperitoneum with 15° Trendelenburg tilt (T3), and at the end of surgery (T4). Additionally, leak pressure was assessed after successful tracheal intubation (T0, baseline) at T2, T3 and T4. Uncuffed ETT leak significantly decreased at T3 compared with T1 (baseline). Leak pressure significantly increased at T3 and T4 compared with T0 (baseline). Further studies are needed in order to determine whether the results are universal and associated with clinically significant outcomes.

Testicular ascent after laparoscopic percutaneous extraperitoneal closure for inguinal hernias.

AIM: Laparoscopic percutaneous extraperitoneal closure (LPEC) has been widely performed for the repair of pediatric inguinal hernias in Japan. This study aimed to evaluate the testicular ascent and orchiopexy after LPEC in males with inguinal hernias. METHODS: The medical records of male patients who underwent LPEC procedures for the repair of an inguinal hernia from January 2010 to December 2013 at our institution were reviewed. The patients who underwent orchiopexy after the LPEC procedure were investigated, the characteristics studied were the birth weight of the patients, the age when they underwent LPEC, the mean time from LPEC to orchiopexy, and the location of the affected testes. The LPEC procedure was performed by extraperitoneal circuit suturing around the internal inguinal ring with a long straight special needle (Lapaherclosure; Hakko Medical Co., Tokyo, Japan). RESULTS: During the 4-year period of this study, 438 LPECs were performed on 367 male patients. Orchiopexy was performed on 14 testes (3.2%) in 10 patients who had previously undergone LPEC. Five of the 10 patients were extremely low-birth-weight infants. The mean time from LPEC to orchiopexy was 13.2 months. In 7 of these 10 patients, both testes were initially identified in the scrotum at 3 months after LPECs, but they later showed ascending or retractile testes. In another three patients, the ipsilateral testes were elevated early after LPECs, and they were thought to be missed congenital undescended testes. At orchiopexy, 10 of the 14 testes were located in the inguinal region, and the other four testes were retractile. During the orchiopexy, the remaining processus vaginalis was found to adhere to the spermatic cord in all of the patients with ascending testes. CONCLUSION: The postoperative testicular ascent should be carefully examined after the LPEC procedure in patients with pediatric inguinal hernias, especially in extremely low-birth-weight infants.

Short-time exposure to mono-n-butyl phthalate (MBP)-induced oxidative stress associated with DNA damage and the atrophy of the testis in pubertal rats.

Phthalates are widely used as plasticizer in various consumer domestic products and are known to disturb the male reproductive function in rodents. This study investigated the involvement of oxidative stress and the atrophy of the testes in pubertal rats exposed to mono-n-butyl phthalate (MBP). Four-week-old pubertal male rats were separated into three groups. In group I, 21 rats were fed rat chow containing 2 % MBP for 3 days. In group II, 21 rats were fed rat chow containing 2 % MBP for 3 days and antioxidant vitamins C (250 mg/kg/day) and E (50 mg/kg/day) were injected daily. In group III, 21 rats were fed standard rat chow and used as controls. After 3 days, each testis was weighed and the germ cell development was evaluated using the Johnsen score. The urinary 8-hydroxy-2'-deoxyguanosine (8-OHdG) levels were measured as a biological marker of oxidative DNA damage. The mean testis weight was significantly lower for group I than groups II or III (p < 0.05). The mean Johnsen score was significantly lower for group I than for groups II or III (p < 0.05). Urinary 8-OHdG concentrations were higher in group I than in groups II or III. Short-time exposure to MBP may therefore induce oxidative DNA damage in rat testes, while antioxidant vitamins administered during exposure may protect against this stress.

Extrarenal Wilms tumor in children with unfavorable histology: a case report.

Extrarenal Wilms tumor is extremely rare, and only 25 cases in children have been reported to date in Japan. A 2-year-old girl presented with a large left lower quadrant abdominal mass. Abdominal computed tomography revealed a retroperitoneal tumor located below the left kidney. At laparotomy, the tumor was encapsulated without evidence of metastasis to other abdominal organs. Pathologic diagnosis of the tumor was extrarenal Wilms tumor with diffuse anaplasia. After complete tumor resection, chemotherapy was administered according to the treatment protocol (Regimen I) of the Japan Wilms Tumor Study Group. Cyclophosphamide and etoposide were administered in combination with vincristine and doxorubicin. Two years after treatment, the patient has had no evidence of recurrence.

Recurrent acute pancreatitis caused by a gastric duplication cyst communicating with an aberrant pancreatic duct.

A 38-year-old woman was hospitalized in August 2007. This visit was her fifth episode of acute pancreatitis. Computed tomography revealed a cystic structure located near the antrum. Communication between this structure and the pancreatic duct was revealed by endoscopic retrograde cholangiopancreatography. Ultrasonography revealed that the cyst wall had a layered structure. Thus, we regarded it as a gastric duplication cyst. We thought that the gastric duplication cyst communicating with an aberrant pancreatic duct was responsible for the recurrent acute pancreatitis. In August 2008, a cyst gastrostomy was performed between the gastric duplication cyst and the stomach. No recurrence of acute pancreatitis has since occurred.