Intestinal volvulus in utero causing torsion of dilated bowel with ileal atresia: a case report.

BACKGROUND: In utero intestinal volvulus with intestinal atresia is a rare and life-threatening condition that can cause torsion of the dilated bowel. The management and outcomes of this disease remain unclear. CASE PRESENTATION: A 19-year-old woman noticed a decrease in fetal motion at 35 weeks. Fetal ultrasound showed dilated fetal bowel and the whirlpool sign. The patient was referred to our hospital for an emergency cesarean section. The neonate's abdomen was dark and severely distended, and a laparotomy was performed. Necrotic ileum and cord-type intestinal atresia (Type II) were observed in the dilated terminal ileum. The necrotic ileum was resected, and a second-look surgery was performed the following day. Then, we anastomosed the remaining intestine, and the total intestine length was 52 cm. There were no surgical complications, and the patient was discharged without requiring total parenteral nutrition or fluid infusion. The patient's height and weight were within the - 2 standard deviation range of the growth curve at 5 months. CONCLUSIONS: Emergency and appropriate management of intestinal volvulus in utero causing torsion of the dilated bowel resulted in good outcomes in a patient with intestinal atresia. Perinatal physicians should be aware of this emergency condition and plan their treatment approach accordingly.

The risk factors for cholestasis in patients with duodenal atresia in a single institutional cohort.

PURPOSE: The etiology of cholestasis in neonates is associated with several factors including gastrointestinal disease and surgery. We aimed to identify the potential risk factors for perioperative cholestasis in patients with duodenal atresia and determine specific cutoff values for the risk factors. METHODS: This retrospective cohort study included 76 neonates diagnosed with duodenal atresia surgically treated during the neonatal period at our institution between January 1990 and March 2017. The neonates were categorized into two groups: those with and without cholestasis. Univariate and multivariate analyses were performed to identify the possible risk factors for cholestasis. RESULTS: Among the 76 neonates with duodenal atresia, 21 (27%) developed cholestasis. The duration of total parenteral nutrition was identified as a risk factor in univariate analysis; however, it was not an independent risk factor for cholestasis. Gestational age and highest C-reactive protein (CRP) values were independent risk factors, with adjusted odds ratios of 0.53 and 1.25, respectively. To predict the occurrence of cholestasis, the cutoff value for gestational age was 35.0 weeks, and highest CRP value was 2.4 mg/dL. CONCLUSIONS: The occurrence of cholestasis in patients with duodenal atresia was associated with preterm delivery and severity of the inflammatory response during the perioperative period.

Long-term morbidity in adolescents and young adults with surgically treated esophageal atresia.

PURPOSE: To investigate the long-term morbidity of surgically treated esophageal atresia (EA) in adolescents and young adults and establish whether these long-term morbidities are affected by the type of EA. PATIENTS AND METHODS: We reviewed the medical records, including backgrounds and associated conditions, of 69 long-term survivors of EA, aged >15 years. The long-term morbidities included neurodevelopmental abnormality, nutritional impairment (short height <-2SD, low BMI <18.5), subjective symptoms, and musculoskeletal deformities. Comparisons of the results were made between Gross A-type EA (n = 6) and Gross C-type EA (n = 63). RESULTS: All patients underwent esophageal anastomosis without esophageal replacement. Cardiac anomalies and long gap were present in 26 and 18%, respectively. Esophageal dilatation, fundoplication, and aortopexy were performed in 40, 34, and 18%, respectively. The incidence of long gap and esophageal stenosis was higher in Gross A-type EA than in Gross C-type EA. The long-term morbidities included neurodevelopmental abnormality (13%), nutritional impairment (62%: as short height in 34% and as low BMI in 46%), subjective symptoms (14%), and musculoskeletal deformities (59%). There were no differences in the long-term morbidities between Gross A and Gross C. CONCLUSIONS: The incidence of the long-term morbidities is high in adolescents and young adults, regardless of the type of EA. Early detection of morbidity is important to improve the long-term outcomes of EA.

Efficacy of CT-guided localization followed by video-assisted thoracoscopic surgery in children with tiny pulmonary nodules.

CT-guided localization followed by video-assisted thoracoscopic surgery was performed in three children with pulmonary nodules less than 5 mm in diameter. The patients' respective primary diagnoses were Wilms tumor, hepatoblastoma, and osteosarcoma of the femur. The pulmonary nodules were marked preoperatively by a percutaneously placed hook-wire or dye under CT guidance. Although none of the nodules was grossly detected during the operation, they were correctly resected under the guidance of the hook-wire wound or dye. A histological assessment revealed viable metastatic lesions in the case of hepatoblastoma, completely necrotic lesions in the case of Wilms tumor, and inflammatory nodules in the case of osteosarcoma. CT-guided localization followed by video-assisted thoracoscopic surgery appears to be a beneficial procedure in children with tiny pulmonary nodules.

Prenatal and Postnatal Clinical Course of an Urachus Identified as an Allantoic Cyst in the Umbilical Cord.

PURPOSE: The aim of this study was to clarify the prenatal and postnatal clinical courses of an urachus identified as an allantoic cyst in the umbilical cord. METHODS: Allantoic cysts in the umbilical cord were identified in five fetuses over the past 12 years at our hospital. The prenatal and postnatal clinical courses of these patients were retrospectively reviewed. RESULTS: The presence of allantoic cysts in the umbilical cord was first detected at 15 to 27 weeks of gestation. The cysts subsequently became enlarged, reaching a maximum diameter of 34 to 61 mm at 17 to 32 weeks of gestation. The cysts then suddenly disappeared due to spontaneous rupture at 26 to 35 weeks of gestation. After being born at 38 (35-39) weeks of gestation, four patients were diagnosed with a patent urachus requiring surgery in the infantile period and one was diagnosed with an urachal cyst, which is currently being observed without surgery. CONCLUSION: The presence of an urachus identified as an allantoic cyst in the umbilical cord is frequently associated with spontaneous rupture during the prenatal period, resulting in a patent urachus after birth that requires surgical intervention.

Fluoroscopy-guided Percutaneous Endoscopic Gastrostomy in Children: A Simple and Safe Technique.

Percutaneous endoscopic gastrostomy (PEG) is a widely used minimally invasive procedure. However, PEG is difficult in neurologically impaired patients due to scoliosis with a high-riding stomach and intestinal dilatation. Fluoroscopy-guided PEG (F-PEG) is expected to avoid PEG-related complications such as intestinal injury and malpositioned tube placement. The aim of this retrospective study is to evaluate the feasibility and safety of F-PEG in children. Twenty-one handicapped pediatric patients underwent F-PEG from January 2012 to December 2014 at our hospital. Among them, 6 had scoliosis, 7 had a high-riding stomach, and 3 had gastric volvulus. F-PEG was performed in all 21 patients without any major complications. The median procedure duration was 22 minutes. In 1 patient with severe scoliosis (Cobb angle, 118 degrees) and a high-riding stomach, F-PEG was safely performed. In conclusion, F-PEG is a simple and feasible technique, and can reduce the risk of PEG-related complications in handicapped pediatric patients.

Long-term outcomes of four patients with tracheal agenesis who underwent airway and esophageal reconstruction.

PURPOSE: The aim of this study was to evaluate the long-term outcomes of four patients with tracheal agenesis who underwent airway and esophageal/alimentary reconstruction. MATERIALS AND METHODS: We reviewed the medical records of four long-term survivors of tracheal agenesis and collected the following data: age, sex, type of tracheal agenesis, method of reconstruction, nutritional management, and physical and neurological development. RESULTS: The patients consisted of three boys and one girl, who ranged in age from 77 to 109months. The severity of their condition was classified as Floyd's type I (n=2), II (n=1), or III (n=1). Mechanical respiratory support was not necessary in any of the cases. Esophageal/alimentary reconstruction was performed using the small intestine (n=2), a gastric tube (n=1), and the esophagus (n=1). The age at esophageal reconstruction ranged from 41 to 55months. All of the cases required enteral nutrition via gastrostomy. Three of the patients were able to swallow a small amount of liquid and one was able to take pureed food orally. The physical development of the subjects was moderately delayed-borderline in childhood. Neurological development was normal in two cases and slightly delayed in two cases. CONCLUSIONS: None of the long-term survivors of tracheal agenesis required the use of an artificial respirator, and their development was close to normal. Future studies should aim to elucidate the optimal method for performing esophageal reconstruction to allow tracheal agenesis patients to achieve their full oral intake.

Clinical application of indocyanine green (ICG) fluorescent imaging of hepatoblastoma.

BACKGROUND/PURPOSE: Although the usefulness of intraoperative indocyanine green (ICG) fluorescent imaging for the resection of hepatocellular carcinoma has been reported, its usefulness for the resection of hepatoblastoma remains unclear. This study clarifies the feasibility of intraoperative ICG fluorescent imaging for the resection of hepatoblastoma. METHODS: In three hepatoblastoma patients, a primary tumor, recurrent tumor, and lung metastatic lesions were intraoperatively examined using a near-infrared fluorescence imaging system after the preoperative administration of ICG. RESULTS: ICG fluorescent imaging was useful for the surgical navigation in hepatoblastoma patients. In the first case, the primary hepatoblastoma exhibited intense fluorescence during right hepatectomy, but no fluorescence was detected in the residual liver. In the second case, a recurrent tumor exhibited fluorescence between the residual liver and diaphragm. A complete resection of the residual liver, with a partial resection of the diaphragm, followed by liver transplantation was performed. In the third case with multiple lung metastases, each metastatic lesion showed positive fluorescence, and all were completely resected. These fluorescence-positive lesions were pathologically proven to be viable hepatoblastoma cells. CONCLUSION: Intraoperative ICG fluorescence imaging for patients with hepatoblastoma was feasible and useful for identifying small viable lesions and confirming that no remnant tumor remained after resection.

Perioperative management after high-dose chemotherapy with autologous or allogeneic hematopoietic stem cell transplantation for pediatric solid tumors.

INTRODUCTION: An increasing number of children with advanced malignancies have recently received high-dose chemotherapy (HDC) with hematopoietic stem cell transplantation (HSCT), followed by surgery. In this study, we reviewed our experience with surgery after HDC and autologous (auto) or allogeneic (allo) HSCT to elucidate the problems associated with this treatment and establish the optimum surgical management strategy. PATIENTS AND METHODS: We retrospectively reviewed the cases of 24 children with advanced malignancy treated with HDC and HSCT before tumor resection at our institution. The tumors included 18 neuroblastomas, 5 soft tissue sarcomas, 2 hepatoblastomas, and 1 Wilms tumor. The source of hematopoietic stem cells was auto-HSCT in 19 patients and allo-HSCT in 5 patients. To be able to undergo surgery, it was necessary that the patient's general condition, including hemostasis, should be fairly good and that the results of hematological examinations should include a white blood cell (WBC) count of>1,000/µL, hemoglobin level of>10 g/dL and platelet count of>5 × 10(4)/µL. RESULTS: The mean duration before WBC recovery after HSCT was 14.5 ± 1.4 days after auto-HSCT and 23.8 ± 1.2 days after allo-HSCT, respectively (p<0.01). The mean duration before platelet recovery after HSCT was 46.5 ± 5.2 days for auto-HSCT and 48.6 ± 5.5 days for allo-HSCT (not significant [n.s.]). The mean interval between allo-HSCT and surgery was significantly longer (92.8 ± 6.2 days) than that between auto-HSCT and surgery (57.0 ± 3.9 days) (p<0.01), likely because of the use of steroids and immunosuppressants after HSCT. The tumors were completely resected in all cases without severe complications. All the patients treated with allo-HSCT had an acute graft versus host (aGVH) reaction at 2 to 3 weeks after HSCT, and specifically required the administration of steroids and immunosuppressants to prevent aGVH. The postoperative complications included paralytic ileus in two cases and a tacrolimus-associated encephalopathy in one case involving allo-HSCT. In half of the patients, the WBC count was not elevated after surgery, whereas the postoperative serum C-reactive protein (CRP) level was elevated in all cases. CONCLUSIONS: Our data indicate that surgical treatment can be safely performed even after HDC with HSCT if attention is paid to myelosuppression and the adverse effects of both chemotherapeutic agents and immunosuppressants.

Problems during the long-term follow-up after surgery for pediatric solid malignancies.

INTRODUCTION: With the recent improvements in the prognosis of pediatric malignancies, the number of patients surviving long-term after surgery has been increasing. Therefore, the late effects of cancer treatments are important issues. In this study, we analyzed the problems associated with the treatment of pediatric patients during the long-term follow-up after surgery. PATIENTS AND METHODS: A total of 64 patients with pediatric malignancies who underwent surgical treatment and were followed up for more than 5 years and who were older than 13 years of age were included in this study. The average age was 20.8 (13-33) years, and the follow-up ranged from 5 to 31 years (mean, 17.7 years). Twenty-one patients (32.3%) received high-dose chemotherapy (HDC) and nine (14.1%) received radiotherapy. RESULTS: In this study, 46 patients (71.9%) developed at least one problem during the follow-up period. With regard to the surgical problems, 14 patients underwent nephrectomy, and 1 of them developed renal failure. One patient received cystectomy with urinary tract reconstruction. One patient received a partial vaginectomy. Two cases with ovarian tumors received oophorectomy, one of whom also received partial hysterectomy. Other complications such as ileus, scoliosis, and leg length discrepancies were seen in some patients. In terms of the medical problems, 15 patients showed growth retardation and 2 were treated with growth hormone therapy. Gonadal dysfunction was observed in 23 patients, and 8 of them were treated with hormone replacement therapy. Six patients developed hypothyroidism, two of whom were treated with thyroid hormone replacement therapy. Other medial issues, such as hearing impairment, low bone mineral density, and hepatitis, were seen in some patients. The rate of growth retardation, gonadal dysfunction, and hypothyroidism were significantly higher in the patients who received HDC (p<0.05). There was one case of second malignancy of the parotid gland. CONCLUSION: Various treatment-related complications may occur even many years after treatment, especially in patients who receive HDC. Medical problems, especially endocrine disorders, appear to be more serious than surgery-related problems. Lifetime medical surveillance and continuous follow-up by not only pediatric surgeons but also by various specialists, such as pediatric oncologists, pediatric endocrinologists, urologists, and gynecologists, are necessary.

A case of pediatric live-donor liver transplantation with a left lateral segment reduction by a linear stapler after reperfusion.

In pediatric LDLT, graft reduction is sometimes required because of the graft size mismatch. Dividing the portal triad and hepatic veins with a linear stapler is a rapid and safe method of reduction. We herein present a case with a left lateral segment reduction achieved using a linear stapler after reperfusion in pediatric LDLT. The patient was a male who had previously undergone Kasai procedure for biliary atresia. We performed the LDLT with his father's lateral segment. According to the pre-operative volumetry, the GV/SLV ratio was 102.5%. As the patient's PV was narrow, sclerotic and thick, we decided to put an interposition with the IMV graft of the donor between the confluence and the graft PV. The graft PV was anastomosed to the IMV graft. The warm ischemic time was 34 min, and the cold ischemic time was 82 min. The ratio of the graft size to the recipient weight (G/R ratio) was 4.2%. After reperfusion, we found that the graft had poor perfusion and decided to reduce the graft size. We noted good perfusion in the residual area after the lateral edge was clamped with an intestinal clamp. The liver tissue was sufficiently fractured with an intestinal clamp and then was divided with a linear stapler. The final G/R ratio was 3.6%. The total length of the operation was 12 h and 20 min. The amount of blood lost was 430 mL. No surgical complications, including post-operative hemorrhage and bile leakage, were encountered. We believe that using the linear stapler decreased the duration of the operation and was an acceptable technique for reducing the graft after reperfusion.

Increased expression of survivin in hepatoblastoma after chemotherapy.

BACKGROUND: Survivin, an inhibitor of apoptosis, has been reported to be associated with a worse prognosis in some malignancies. However, its expression in hepatoblastoma (HB) remains to be elucidated. We assessed the survivin expression in HB specimens collected before and after chemotherapy to elucidate the impact of survivin on the outcome of HB therapy. METHODS: HB specimens were collected before and after 2 to 4 cycles of cisplatin-based chemotherapy from 16 patients. The survivin expression level was assessed by immunohistochemical staining and real-time polymerase chain reaction. RESULTS: Out of 16, 12 HB sections collected before chemotherapy were positive for survivin as determined by immunohistochemical staining. The intensity of survivin expression was found to significantly increase after chemotherapy. Surprisingly, all of the HB specimens obtained after chemotherapy were positive for survivin. The expression of survivin messenger ribonucleic acid from a human HB cell line, Huh-6 was significantly higher when the cells were cultured with cis-diamminedichloroplatinum(II) than when the cells were cultured without the drug. CONCLUSION: Our results indicate that most of the primary HB tissue specimens express survivin, and its expression increased after chemotherapy, thus suggesting that survivin may concern with the survival of tumor cells, therefore be a candidate for the target of the treatment of HB.

Repair of congenital diaphragmatic hernias through umbilical skin incisions.

The use of thoracoscopy and laparoscopy in the treatment of congenital diaphragmatic hernias (CDHs) has been recently reported; however, the use of these procedures still remains controversial due to CO2 insufflation and limited working space. In addition, because of difficult techniques, it has not been widely accepted. Among CDH patients, mild cases whose lung is not hypoplastic, and not associated with persistent pulmonary hypertension of the neonate (PPHN) often develop small defects in the diaphragm that can be repaired using "direct closure." Because direct closure does not require as wide an operative field as that needed for patch closure, we repaired CDH through umbilical skin windows in two neonates with mild CDH to minimize the wounds. With the creation of additional radical small incisions, the surgeries were successfully performed without any intra- or postoperative complications, and the wounds were cosmetically pleasing. The repair of CDHs through umbilical skin windows is a feasible and useful approach in neonates with mild CDH.

Psychosocial and cognitive consequences of major neonatal surgery.

PURPOSE: To evaluate the long-term quality of life (QOL) of patients who had undergone major neonatal surgery, the psychosocial and cognitive consequences of neonatal surgical stress were assessed when the patients reached school age. MATERIALS AND METHODS: Seventy-two patients who had undergone major neonatal surgery were enrolled in this study. Their primary diseases were anorectal malformation (ARM) in 27 cases, esophageal atresia (EA) in 23, and congenital diaphragmatic hernia (CDH) in 22. Intelligence tests using Wechsler Intelligence Scale for Children III (WISC-III) or a developmental test and the Child Behavior Checklist were conducted through questionnaires and interviews with clinical psychologists. RESULTS: Mental retardation (MR) was apparent in 25% of EA, 20% of ARM, and 18% of CDH, significantly higher than the 2% to 3% commonly found in the general population. The clinical range (CR) of the Child Behavior Checklist was seen in 35% of EA, 59% of ARM, and 38% of CDH, which is also significantly higher than the 25% typically seen in the general population. No significant differences in MR and CR were seen among the primary diseases. The most important factors influencing MR and CR remain to be identified. CONCLUSIONS: To ensure true quality of life after neonatal surgical stress, pediatric surgeons must consider not only physical assessments but also cognitive, emotional, and psychosocial assessments.

A case of childhood dermatofibrosarcoma protuberans of the face.

We report an extremely rare case of dermatofibrosarcoma protuberans located on the face of an 8-year-old boy. Although dermatofibrosarcoma protuberans typically has intermediate malignancy, the tumor in the present case was highly invasive and required a multistaged operation, including wide resection and full-thickness skin grafting. These operations were successfully performed, and the patient is alive without recurrence for 7 years.

Meconium-related ileus in extremely low-birthweight neonates: etiological considerations from histology and radiology.

BACKGROUND: A nationwide survey on neonatal surgery conducted by the Japanese Society of Pediatric Surgeons has demonstrated that the mortality of neonatal intestinal perforation has risen over the past 15 years. The incidence of intestinal perforation in extremely low-birthweight (ELBW) neonates has been increasing as more ELBW neonates survive and as the live-birth rate of ELBW has increased. In contrast to necrotizing enterocolitis (NEC) and focal intestinal perforation (FIP), the pathogenesis of meconium-related ileus, defined as functional bowel obstruction characterized by delayed meconium excretion and microcolon, remains unclarified. METHODS: The histology of 13 ELBW neonates with intestinal perforation secondary to meconium-related ileus was reviewed, and the radiology of 33 cases of meconium-related ileus diagnosed on contrast enema was reviewed. Specimens obtained from 16 ELBW neonates without gastrointestinal disease served as age-matched controls for histological assessment. RESULTS: The size of the ganglion cell nucleus in meconium-related ileus and in control subjects was 47.3 ± 22.0 µm(2) and 37.8 ± 11.6 µm(2), respectively, which was not significantly different. In all cases of meconium-related ileus, contrast enema demonstrated a microcolon or small-sized colon, with a gradual caliber change in the ileum and filling defects due to meconium in the ileum or colon, showing not-identical locations of caliber changes and filling defects. CONCLUSION: Morphological immaturity of ganglia was not suggested to be the pathogenesis of meconium-related ileus. Impaction of inspissated meconium is not the cause of obstruction, but the result of excessive water absorption in the hypoperistaltic bowel before birth, although the underlying mechanism responsible for the fetal hypoperistalsis remains unclear.

Acquired undescended testes in boys with hypospadias.

PURPOSE: We determined the incidence of acquired undescended testes in boys with hypospadias. MATERIALS AND METHODS: We retrospectively reviewed the records of 566 boys with hypospadias who were referred to our outpatient clinic between January 2000 and September 2009. Acquired undescended testes were defined as testes that were documented at the bottom of the scrotum at least once after birth by the pediatric urologist at our institution but were subsequently documented to have moved from a satisfactory scrotal position by the same pediatric urologist or an equally experienced pediatric urologist. However, this definition did not include undescended testes after inguinoscrotal surgery. We excluded boys with gender development disorders with testicular dysgenesis, those who underwent bilateral inguinoscrotal surgery and those without congenital cryptorchidism who were followed less than 3 months. RESULTS: Of the 566 boys with hypospadias 100 met study exclusion criteria. Of the 466 boys included in analysis 29 (6.2%) had congenital cryptorchidism and 15 (3.2%) had acquired undescended testes. Urethroplasty was performed in 413 boys, including 91 with distal, 132 with mid and 181 with proximal hypospadias. The incidence of congenital cryptorchidism and acquired undescended testes in boys with proximal hypospadias was significantly higher than that in boys with other types of hypospadias (p = 0.03 and 0.001, respectively). CONCLUSIONS: Boys with proximal hypospadias are at a higher risk for acquired undescended testes than those with other mild types of hypospadias. Thus, testicular location should be monitored regularly until after puberty.

Long-term outcome of ovotesticular disorder of sex development: a single center experience.

OBJECTIVES: To describe the clinical features of children with ovotesticular disorder of sex development (DSD) and to review cases of ovotesticular DSD in Japan. METHODS: Medical records of eight children diagnosed with ovotesticular DSD at our institute during the past 17 years were retrospectively evaluated. A review of 165 reported cases of ovotesticular DSD from Japanese institutions was carried out. RESULTS: Mean follow up was 8.2 years for six children, with two children lost to follow up. Mean age at first presentation was 2.4 months. All children were Japanese. The most common initial manifestation was ambiguous genitalia. The female:male ratio as the sex of rearing was 1:1. Gender reassignment, from male to female, was carried out in one child at 4-months-old. Genital surgery was always carried out in early childhood as per family desire. Appropriate gonadal tissue was preserved except for one child. No gonadal tumors were detected during follow up. Spontaneous pubertal development occurred in one boy. In reviewing Japanese data, the frequency of testes was higher than in other ethnicities and this was related to the higher incidence of 46,XY. CONCLUSIONS: According to our experience, most families in Japan desire early genital surgery in the case of ovotesticular DSD. Chromosomal and gonadal distributions in patients with ovotesticular DSD differ between Japanese and other ethnic groups. Treatment for these patients needs to be provided after considering the cultural and social backgrounds of DSD in Japan.