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.

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.

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.

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.

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.

Prenatal ultrasonographic and magnetic resonance imaging findings of congenital cloacal anomalies associated with meconium peritonitis.

BACKGROUND/PURPOSE: Cloacal malformations are rare anomalies, occurring in females, and in which they demonstrate a single perineal orifice for urethra, vagina, and rectum. Prenatal ultrasonograms (US) of cloacal malformations sometimes show ascites, hydrocolpos, and hydronephrosis. We herein describe the characteristic prenatal US and magnetic resonance imaging (MRI) findings of the cloacal malformations associated with meconium peritonitis. METHODS: The pre- and postnatal records of 11 newborn patients with cloacal malformations, treated in our hospital from 1988 to 2004, were reviewed. All fetuses underwent prenatal US by experienced obstetricians, whereas in addition, fetal MRI was performed in 1 patient. RESULTS: The prenatal US and/or MRI findings showed fetal ascites, a multicystic pelvic mass, oligohydramnios, and bilateral hydronephrosis in 5 of 11 patients with cloacal malformations. In these 5 cases, postnatal examinations showed associated hydrocolpos, hydrometrocolpos, and bilateral hydronephrosis; furthermore, 4 of these 5 cases also showed meconium peritonitis at laparotomy. CONCLUSIONS: The prenatal US and MRI findings, showing fetal ascites, multicystic pelvic mass, bilateral hydronephrosis, and oligohydraminios are highly suggestive of the cloacal malformations associated with meconium peritonitis.

A case of an abdominoscrotal hydrocele surgically treated under laparoscopic assistance.

We herein describe a 9-month-old boy who presented with bilateral scrotal hydroceles shortly after birth. The right hydrocele spontaneously resolved during this period, and a nonpalpable testis of the right side was noted. In contrast, the left hydrocele gradually increased in size. Ultrasound and magnetic resonance investigations performed at the age of 7 months could not detect the right testis either in the inguinoscrotal region or the abdominal cavity. These examinations also pointed out a huge hydrocele that extended from the left scrotum to the abdominal cavity. At the age of 9 months, a laparoscopic-assisted operation for both the right undescended testis and the left abdominoscrotal hydrocele (ASH) was performed. During the operation, an atrophic testis secondary to in utero intravaginal torsion was shown on the right inguinal region, and a huge ASH was revealed in the left inguinoscrotal region. The finding of the left ASH was confirmed by a laparoscope inserted through the processus vaginalis of the right side. At first, the processus vaginalis of the left side was highly ligated by the inguinal approach, and then the ASH was opened and its wall was resected. Our findings suggest that both an understanding of the pathogenesis and a corrective operation of ASH can be made via the inguinal approach. In cases associated with cryptochidism owing to an intraabdominal testis or an in utero extravaginal torsion, a laparoscopic-assisted operation may therefore be useful both for making a precise diagnosis and for surgically removing an ASH.

Age-specific effect of phthalate ester on testicular development in rats.

PURPOSE: Phthalate esters have been shown to induce testicular damage in both adult and immature rats; however, there have been, so far, few reports describing the age-specific effects of phthalate esters on testicular function. The aim of this study is therefore to investigate the age-specific effects of mono-n-butyl phthalate (MBP) on the testes in both prepubertal and mature adult rats. MATERIALS AND METHODS: Both prepubertal male rats and adult mature male rats were fed special rat chow containing 1% MBP for 10 days. Control prepubertal and adult rats were fed standard commercial rat chow during the same period as the MBP-treated rats. After 10 days of feeding, all rats were killed, and the testes were removed. The weight of the testis was measured, and histological examination of the testis was performed. In addition, the frequency of an apoptotic cell appearance in the seminiferous tubules was determined in both MBP-treated and control groups. RESULTS: In the prepubertal rats, the mean weight of the testes was significantly lower in the MBP-treated rats than in the control rats. A histological examination of the MBP-treated testes showed a decreased seminiferous tubular diameter and an inhibited maturation of germ cells in comparison to those of the control testes. Furthermore, apoptotic cells appeared more frequently in the MBP-treated testes than in the control testes. Although in adult mature rats, no significant difference was observed in either the testicular weight or the histological findings between the MBP-treated and control rats. CONCLUSIONS: The oral administration of MBP to male rats was observed to produce more pronounced testicular damage in prepubertal rats than in adult mature rats. Immature testes may thus be more sensitive to MBP, which induces the germ cell apoptosis in seminiferous tubules and testicular atrophy in prepubertal young rats.

IA-2beta, but not IA-2, is induced by ghrelin and inhibits glucose-stimulated insulin secretion.

Ghrelin is a newly discovered peptide and an endogenous ligand for growth hormone (GH) secretagogue (GHS) receptor. It has been shown to possess various central and peripheral effects, including GH secretion, food intake, and gastric and cardiac effects. Ghrelin and the GHS receptor are expressed also in pancreatic islets. We have identified several ghrelin-induced genes by PCR-select subtraction methods, among which is a beta-cell autoantigen for type 1 diabetes, IA-2beta. Administration of ghrelin increased IA-2beta mRNA in mouse brain, pancreas, and insulinoma cell lines (MIN6 and betaTC3). However, the expression of IA-2, another structurally related beta-cell autoantigen, was not induced by ghrelin. Administration of ghrelin or overexpression of IA-2beta, but not overexpression of IA-2, inhibited glucose-stimulated insulin secretion in MIN6 insulinoma cells and, moreover, inhibition of IA-2beta expression by the RNA interference technique ameliorated ghrelin's inhibitory effects on glucose-stimulated insulin secretion. These findings strongly suggest that inhibitory effects of ghrelin on glucose-stimulated insulin secretion are at least partly due to increased expression of IA-2beta induced by ghrelin. Our data demonstrate the link among ghrelin, IA-2beta, and glucose-stimulated insulin secretion.

In utero exposure to mono-n-butyl phthalate impairs insulin-like factor 3 gene expression and the transabdominal phase of testicular descent in fetal rats.

PURPOSE: Phthalate esters have been shown to cause undescended testes (UT) in rats. It has been proposed that Leydig insulin-like factor 3 (INSL3) may play an important role in testicular descent. The aim of this study was to investigate the effect of mono-n-butyl phthalate (MBP) on both the INSL3 gene expression and the transabdominal testicular descent in rats. METHODS: On the 19th gestational day, the male fetuses that had been exposed to MBP on the 15th to 18th gestational days were dissected and the degree of testicular ascent was examined. Next, quantitative reverse transcriptase polymerase chain reaction was performed to analyze the testicular INSL3 gene expression. At 60 days of age, the testicular position was recorded in the rest of MBP-treated male offspring. RESULTS: On the 19th gestational day, INSL3 messenger RNA expression was significantly decreased in the MBP-treated testes, and the associated degree of testicular ascent was significantly higher than in the controls. At 60 days of age, 12 (54.5%) of 22 rats had either unilateral or bilateral UT. CONCLUSIONS: Prenatal MBP may inhibit the INSL3 gene expression associated with the transabdominal testicular ascent in fetal rats, thereby causing UT in postnatal offspring.

Long-term effects of intrauterine exposure to mono-n-butyl phthalate on the reproductive function of postnatal rats.

PURPOSE: Although it is well known that phthalate esters induce testicular dysfunction in both adult and immature rats, there have been few reports on the long-term effect of phthalate esters on the testicular function of male rats exposed to phthalate esters in utero. This study was designed to assess the long-term effects of the mono-n-butyl phthalate (MBP) ester on the testicular function of neonatal and adult rat offspring from pregnant dams exposed to phthalate esters during gestation. METHODS: Pregnant rats were administered MBP [0.5 g/(kg body weight/.d); 4 days] by gavage from the 15th to the 18th gestational day. Rats administered solvent only were used as control subjects. After the rats' puberty, using male pups whose testes descended normally, the authors examined their fertility while also measuring their testicular weights, mean seminiferous tubular diameter, and the developmental grade of the germ cells (Johnsen score) in their testes. Next, in neonatal rats, the authors measured the testicular concentration of the Mullerian inhibiting substance (MIS) protein using enzyme-linked immunoassay and the expression level of the MIS messenger RNA using the quantitative polymerase chain reaction method as a marker of the Sertoli cells' function. Next the concentration of testosterone protein using a radioimmunoassay as a marker of the Leydig cells' function was measured. RESULTS: The pregnancy rate of the female rats coupled with MBP-treated male rats decreased significantly in comparison with that of the female rats coupled with control male rats (P < .01). Both the testicular weight and the Johnsen score in the MBP-treated group were decreased significantly more than those of the control group (P < .05). Neither the concentration of the MIS protein nor the expression level of the MIS messenger RNA in the MBP-treated neonatal testes differed from those of the control testes, whereas the concentration of testosterone protein in the neonate testes decreased significantly in the MBP-treated group in comparison with that of the control group (P < .01). CONCLUSIONS: A prenatal short-time exposure to MBP induces a long-term effect on postnatal rats and impairs reproductive function in male offspring probably by inhibiting the Leydig cells' rather than Sertoli cells' function in the fetal period.