Treatment and follow-up of late onset intra hepatic bile duct stones in congenital biliary dilatation.

PURPOSE: The postoperative course after surgery for congenital biliary dilatation (CBD) has some complications. Intrahepatic bile duct (IHBD) stones were known as a late complication. We report on the treatment and long-term follow-up of postoperative IHBD stones in our department. METHODS: Patients who underwent CBD surgery at age 15 years or younger in our department were identified. Those followed up for 5 years or more were enrolled. Annual blood chemistry tests and abdominal ultrasonography were performed. Each patient's surgical procedure, IHBD stone diagnosis, treatments, and outcomes were retrospectively assessed. RESULTS: Fifty-one patients were analyzed. The median age at the last visit was 24 years (range 7-45 years), and the median age at CBD surgery was 3 years. Eight patients (16%) developed late-onset IHBD stones. The median age at onset was 25 years, and the median duration after surgery was 20 years. The initial treatment was double-balloon enteroscopy (DBE) in 4 cases, which resulted in stone removal in 3 of the 4 patients (75%). CONCLUSION: Since CBD may cause late-onset IHBD stones, continuous follow-up is required even in adulthood. In this study, DBE was effective and minimally invasive, and it is recommended as the initial treatment.

Prevalence of urachal remnants in children according to age and their anatomic variants.

PURPOSE: The aim of this study was to elucidate the prevalence of urachal remnants in children in relation to patient age as well as to identify their anatomic variants, using a laparoscopic view. METHODS: The medical records of 394 pediatric patients who underwent laparoscopic inguinal hernia repair were reviewed. Patients were divided into four groups based on their age at surgery. Using laparoscopic visualization, the presence and anatomic variants of urachal remnants were analyzed. RESULTS: A urachal remnant was confirmed in 140 children (35.5%). Although the prevalence was significantly higher in the group of children aged < 1 year (63.2%) than in any other group, no significant difference in the prevalence was observed between the groups aged ≥ 1 year. In 42 cases (10.7%), the urachal remnant merged into the lateral umbilical ligament. CONCLUSIONS: Our results suggest a recommendation of nonoperative management of asymptomatic urachal remnants, especially in patients less than 1 year of age due to its probable spontaneous resolution. Knowledge of the anatomic variants could improve the accuracy of diagnosis of urachal remnants and the comprehension of its structure and localization for the achievement of accurate and complete excision.

Clinical Usefulness of Blenderized Food for Selenium Deficiency in Pediatric Patients with Severe Motor and Intellectual Disabilities.

INTRODUCTION: This study aimed to assess the preventive or therapeutic effects of blenderized food (BF) on selenium deficiency in pediatric patients with severe motor and intellectual disabilities (SMID). METHODS: The medical records of all 40 consecutive pediatric patients with SMID who underwent nutritional assessment were retrospectively reviewed and compared between two groups: the enteral formula (EF) group and the BF group fed with BF providing more than 10% of total caloric intake. Next, for the selenium-deficient patients who were newly started on blenderized tube feeds after the first nutritional assessment, improvement of selenium deficiency and change of dietary contents were assessed. RESULTS: The BF group patients had a significantly lower prevalence of selenium deficiency and higher serum selenium levels than the EF group patients. In all 7 selenium-deficient patients who started blenderized tube feeds after the first nutritional assessment, serum selenium levels were significantly increased at the second nutritional assessment, even though total selenium intake, selenium intake by EF, and total caloric intake did not differ significantly, and, in fact, caloric intake was significantly decreased by EF. CONCLUSION: Combined feeding of BFs can be useful for prevention and therapy of selenium deficiency in pediatric SMID patients.

Importance of plasma ghrelin levels with special reference to nutritional metabolism and energy expenditure in pediatric patients with severe motor and intellectual disabilities.

BACKGROUND & AIMS: Nutritional metabolism is complex in pediatric patients with severe motor and intellectual disability (SMID), and therefore, appropriate estimation of the energy requirements is difficult. Focusing on ghrelin's role in energy metabolism regulation, we investigated plasma ghrelin levels in pediatric SMID patients and analyzed its nutritional significance as a regulatory marker of energy reserve. METHODS: Fasting plasma total, acyl, and des-acyl ghrelin levels in 40 patients with SMID, including cerebral palsy (CP) (n = 20) and muscular disease (MD) (n = 8), and healthy controls (n = 13) were investigated. The correlations of plasma ghrelin levels with anthropometry, blood nutritional markers, energy intake, and resting energy expenditure (REE) measured with indirect calorimetry were analyzed. A p value < 0.05 was considered significant. RESULTS: SMID patients had significantly higher acyl ghrelin, and lower body mass index (BMI), z-scores of body weight (BW), body height and BMI, and albumin than controls. CP patients had significantly higher total and acyl ghrelin, z-score of the mid-upper arm circumference (MUAC), retinol-binding protein, transthyretin, creatinine, and glucose than MD patients. Total and acyl ghrelin in CP patients and des-acyl ghrelin in MD patients had significant negative correlations with MUAC and upper arm fat area. In CP patients, total and acyl ghrelin had significant positive correlations with REE/BW (kcal/kg), and total ghrelin was predictive of REE/BW (r2 = 0.625, p < 0.0001). CONCLUSIONS: An increase in acyl ghrelin observed in SMID patients possibly indicates energy reserve deficiency. In CP patients, total and acyl ghrelin inversely reflected total body fat mass, resulting in strongly positive correlations with REE/BW. The measurement of plasma ghrelin may be useful to assess nutritional metabolism and energy reserve in pediatric SMID patients, such as CP and MD patients.

Safety and efficacy of laparoscopy-assisted percutaneous endoscopic gastrostomy in infants and small children weighing less than 10 kg: A comparison with larger patients.

INTRODUCTION: Laparoscopy-assisted percutaneous endoscopic gastrostomy (LAPEG) can reduce the risk of percutaneous endoscopic gastrostomy-related complications, such as intra-abdominal organ injury, and determine the optimal position for placement of the gastrostomy tube. We first employed LAPEG 10 years ago but limited its application to elderly patients. Indications for LAPEG have now expanded to small children. This retrospective study aimed to determine the feasibility of LAPEG in children weighing <10 kg. METHODS: Our LAPEG procedure for small children involves three essential techniques: gastric insufflation with CO2 to prevent intestinal dilation, a T-fastener device to overcome the difficulties of gastropexy, and primary placement of a button gastrostomy to create less torque than tube gastrostomy at the insertion site and to prevent early tube dislodgement. The medical records of 48 patients with physical and mental disabilities who underwent LAPEG between 2010 and 2018 were evaluated. The outcomes of LAPEG in patients weighing <10 kg (group A, n=11) and ≥10 kg (group B, n=37) were compared. RESULTS: The LAPEG procedure was completed in all cases without intraoperative complications or open conversion. The median bodyweight of group A was 6.3 kg (range, 3.6-8.2 kg). None of the patients in group A developed postoperative complications such as stomal infection or dislodgement. The operative time was significantly shorter in group A than in group B (P < .05). CONCLUSION: By improving surgical techniques for small children, our LAPEG procedure might be feasible and safe for treating children weighing <10 kg, including those weighing as little as 3.6 kg.

Thoracoscopic resection of congenital esophageal stenosis.

Congenital esophageal stenosis (CES) is rare and usually manifests in infants as dysphagia, failure to thrive, and food impaction. Dilatation is considered to be the first-line therapy for CES, but the incidence of complications (perforation and mediastinitis) is relatively high. We report two cases of CES treated by thoracoscopic resection without prior dilatation. Both infants recovered without recurrent stenosis and were able to eat solid food soon after surgery. One had postoperative gastroesophageal reflux and eventually required fundoplication. Thoracoscopic resection could be a valid option for CES.

A sutureless technique using cyanoacrylate adhesives when creating a stoma for extremely low birth weight infants.

PURPOSE: Intestinal perforation and necrotizing enterocolitis (NEC) are neonatal intestinal emergencies that are especially common in premature infants. While prompt surgical intervention, including stoma creation, is often required, the optimal surgical treatment has been controversial because of the substantial risks related to the stoma creation and management. The use of a tissue adhesive may have some advantages over the use of sutures when creating an intestinal stoma in extremely low birth weight (ELBW) infants. The purpose of this report was to present a novel approach for creating a stoma using a tissue adhesive in ELBW infants. METHODS: A total of eight ELBW infants that underwent laparotomy with the creation of intestinal stomas using cyanoacrylate adhesive at our institution between 2009 and 2014 were enrolled. The clinical parameters, including the length of the operation, intra- and postoperative complications and the outcomes were evaluated. RESULTS: The median body weight and gestational age at birth were 630 g and 24.3 weeks, respectively. The median age at referral was 11.5 days. The median length of the procedure was 58.5 min, including the inspection and resection of the intestine. All procedures were completed without any intraoperative complications. There were no postoperative complications associated with the stoma. Two patients died of the associated septic status. CONCLUSIONS: Sutureless enterostomy using cyanoacrylate adhesive is a simple technique which has the potential to reduce the incidence of complications related to the intestinal stoma in ELBW infants.

Surfactant protein-D attenuates the lipopolysaccharide-induced inflammation in human intestinal cells overexpressing toll-like receptor 4.

PURPOSE: Necrotizing enterocolitis (NEC) is a devastating inflammatory disease of preterm infants that may depend on overexpression of toll-like receptor-4 (TLR4) in the immature intestine. Surfactant protein (SP)-D is a member of the collectin family and plays an important role in innate immunity, particularly in the airways. Although SP-D also exists in the intestines, little is known about its function. This study investigated whether SP-D can attenuate the inflammatory response of TLR4-overexpressing embryonal intestinal cells. METHODS: All experimental procedures were performed using the human intestinal cell line INT407 originally derived from human embryonal intestines. Platelet-activating factor (PAF), reported to be elevated in NEC patients, was used to induce TLR4 overexpression in the human embryonal intestinal cell line INT407. TLR4 expression was measured using quantitative real-time PCR. Inflammatory responses to PAF (5 µM), the TLR4 agonist lipopolysaccharide (LPS, 100 ng/ml), PAF + LPS, and PAF + LPS following SP-D pretreatment (20 µg/ml) were assessed by enzyme-linked immunosorbent assay (ELISA) of interleukin-8 (IL-8) release (in pg/ml). RESULTS: Expression of TLR4 mRNA (mean ± SD) was upregulated by PAF (369 % ± 28 %, p < 0.001). Stimulation with PAF + LPS resulted in higher IL-8 release (1959.3 ± 52.3) than control (141.2 ± 12.4), LPS (167.3 ± 65.8), or PAF (1527.2 ± 129.4) treatment (p < 0.05). Release in response to PAF + LPS (1590.1 ± 319.3) was attenuated by SP-D pretreatment (1161.6 ± 131.6; p < 0.05). CONCLUSION: SP-D attenuates LPS-induced IL-8 production in TLR4-overexpressing intestinal cells, suggesting that SP-D may have a protective effect in the development of NEC in preterm infants.

Risk factors for surgical intestinal disorders in VLBW infants: Case-control study.

BACKGROUND: Very low-birthweight (VLBW) infants (VLBWI) are at increased risk for surgical intestinal disorders including necrotizing enterocolitis (NEC), focal intestinal perforation (FIP) and meconium-related ileus (MRI). The aim of this study was to identify disease-specific risk factors for surgical intestinal disorders in VLBWI. METHODS: A retrospective multicenter case-control study was conducted at 11 institutes. We reviewed VLBWI who underwent laparotomy for intestinal disorders including perforation and intractable bowel obstruction. The surgical disorders were classified into four categories (NEC, FIP, MRI, others) based on the macroscopic findings at operation. In order to identify risk factors, two matched controls for each subject were chosen based on gestational age and birthweight. OR and 95%CI were calculated using a conditional logistic regression model and a multivariate model. RESULTS: A total of 150 cases (NEC, n = 44; FIP, n = 47; MRI, n = 42; others, n = 17) and 293 controls were identified. The cases and controls were similar in terms of gestational age and birthweight (cases/controls, 26.7 ± 2.5/26.5 ± 2.6 weeks; 790 ± 256/795 ± 257 g). On multivariate modeling, disease-specific risk factors were as follows: female (OR, 0.23; 95%CI: 0.06-0.89), respiratory distress syndrome (OR, 35.7; 95%CI: 2.48-514) and patent ductus arteriosus (OR, 10.9; 95%CI: 1.51-79.3) for NEC; outborn delivery (OR, 5.47; 95%CI: 1.48-20.2) for FIP; and twin pregnancy (OR, 4.25; 95%CI: 1.06-17.1), PROM (OR, 6.85; 95%CI: 1.33-35.4) and maternal steroid (OR, 0.23; 95%CI: 0.07-0.79) for MRI. CONCLUSIONS: Different risk factors were identified for NEC, FIP and MRI, suggesting that each disease has a different etiology, and that different strategies are required to prevent these diseases.

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.

Treatment and ovarian preservation in children with ovarian tumors.

BACKGROUND/PURPOSE: Ovarian preservation is desirable in children with ovarian tumors. However, the diagnostic and treatment strategies are heterogeneous. The aim of this study was to investigate the management and preservation of ovarian tissue in order to identify the factors associated with ovarian preservation. METHODS: Thirty-seven patients (41 ovaries) were surgically treated for ovarian tumors. Four cases were bilateral. The data on the patient symptoms at presentation, imaging, treatment, outcome, pathology, and status of ovarian preservation were retrospectively analyzed for each patient. RESULTS: Histological examinations revealed 25 mature teratomas, 5 immature teratomas, 5 cystadenomas, 2 dysgerminomas, and other tumors. Ovarian torsion occurred in 16 ovaries (39%). Ovary-sparing surgery was performed in 22 ovaries (53.7%). Successful ovarian preservation was significantly associated with a smaller tumor size, benign pathology, and a lower degree of torsion (P<0.01). CONCLUSIONS: Because the prognosis was favorable in most cases, the preservation of fertility and gonadal function should be a goal in the surgical treatment of ovarian tumors. We recommend ovary-sparing surgery as the first-line treatment for all pediatric ovarian tumors other than those that are preoperatively diagnosed as being malignant and those in which emergent surgical intervention is indicated owing to the suspicion of ovarian torsion.

Chronic ileocolic intussusception due to transmural infiltration of diffuse large B cell lymphoma in a 14-year-old boy: a case report.

Chronic intussusception, defined as intussusception continuing over 14 days, is rare in children. We herein report a case of chronic ileocolic intussusception caused by the transmural infiltration of diffuse large B cell lymphoma in a 14-year-old boy. The patient had been suffering from anorexia and intermittent abdominal pain for 5 weeks, during which his body weight decreased by around 7 kg. Upon admission to our hospital, ultrasonography and enhanced computed tomography (CT) of the abdomen showed ileocolic intussusception. A retrospective examination of abdominal CT led us to suspect that the intussusception had initially appeared 5 weeks before admission, presumably coinciding with the beginning of the patient's abdominal symptoms. Since hydrostatic reduction was unsuccessful, laparotomy was performed, which showed unreducible ileocolic intussusception with a marked edematous ileum and mesentery. Ileocecal resection without lymph node dissection was carried out, and a histological examination of the resected specimen revealed the transmural infiltration of diffuse large B-cell lymphoma of the terminal ileum. The patient's postoperative course was uneventful, and adjuvant chemotherapy was administered. This case illustrates the diagnostic challenges of confirming 'chronic' intussusception in older children.

Immunolocalization of surfactant protein D in the liver from infants with cholestatic liver disease.

PURPOSE: Surfactant protein D (SP-D) is one of specific surfactant proteins constituting pulmonary surfactant. Recent studies have revealed that SP-D is detected in various non-pulmonary tissues and is involved in the host defense and immunomodulation. However, the relationship between SP-D and liver diseases has not yet been investigated. The aim of this study was to detect the immunolocalization of SP-D in the livers of infants with cholestatic liver disease. METHODS: The expression of immunoreactive SP-D was assessed in infants with cholestasis, including biliary atresia (BA, n=7), neonatal hepatitis (NH, n=2), and paucity of the intrahepatic bile duct (PIBD, n=4). Immunoreactive SP-D was also assessed in six infants who died of non-liver disease as controls. Tissue samples were obtained at liver biopsy, or by post-mortem sampling. The tissue sections were incubated with anti-SP-D polyclonal antibodies and were counterstained with hematoxylin. RESULTS: In the normal livers, SP-D was detected in the intrahepatic bile ducts, but was not detected in hepatocytes. In contrast, intense SP-D staining was noted in the hepatocytes from infants with BA, NH, and PIBD. Although SP-D was detected in the intrahepatic bile ducts in the infants with NH, negative or weak staining was seen in the intrahepatic bile ducts in infants with BA. CONCLUSION: Our data showed that SP-D is present in the bile ducts of the normal infant liver, and it was found to accumulate in the hepatocytes of cholestatic livers. These results suggest that SP-D is produced in hepatocytes and is secreted into the bile ducts.

Laparoscopic treatment of pediatric hydrocele and the evaluation of the internal inguinal ring.

BACKGROUND: There are few reports of the laparoscopic findings of the internal inguinal ring (IIR) in patients with hydrocele. The purpose of this study was to assess the safety and efficacy of laparoscopic percutaneous extraperitoneal closure (LPEC) for hydrocele in comparison with that of open repair (OR) and compare the findings of the IIR between cases of hydrocele and inguinal hernia (IH). MATERIALS AND METHODS: We analyzed 69 consecutive patients with hydrocele who underwent surgery at our institution between April 2009 and February 2014. The patients were divided into two groups (LPEC and OR) according to the procedure. Age, length of operation/anesthesia, and complications were compared. Concerning the findings of the IIR, we classified the features into three categories, as follows: Type 1, flat; Type 2, narrow patent processus vaginalis (PPV) with a peritoneal veil; and Type 3, widely opened PPV. We then compared these findings between the cases of hydrocele and IH treated with LPEC during the study period. RESULTS: Among a total of 69 patients, 40 underwent LPEC, and 29 underwent OR. There were no significant differences in the length of operation/anesthesia and complications. No recurrences were observed in either group. The findings of the IIR were mostly classified as Type 2 (59.1%) among the cases of hydrocele and Type 3 (92%) among the cases of IH. CONCLUSIONS: LPEC is a safe and effective procedure for treating hydrocele. The findings of the IIR differ between cases of hydrocele and IH.

Safety and efficacy of laparoscopic percutaneous extraperitoneal closure for inguinal hernias and hydroceles in children: a comparison with traditional open repair.

INTRODUCTION: Laparoscopic procedures for inguinal hernias and hydroceles in children have become widespread in the past few decades. The purpose of this study was to perform a retrospective analysis of our experience in order to assess the safety and efficacy of laparoscopic percutaneous extraperitoneal closure (LPEC) and to compare the findings with those of conventional open repair (OR). SUBJECTS AND METHODS: We analyzed the medical records of 488 patients who underwent LPEC or OR for inguinal hernia or hydrocele at our institute between April 2008 and December 2012. The indications for the operation, length of the operation, complications, day surgery, contralateral patent processus vaginalis, and incidence of metachronous contralateral hernia were investigated. The chi-squared test, unpaired t test, and Steel-Dwass test were used to analyze the significance of the data. RESULTS: Among a total of 488 patients, 326 patients underwent LPEC (125 males and 201 females), and 162 underwent OR (140 males and 22 females). There was no significant difference in the incidence of recurrence (three in the LPEC and none in the OR group, P=.55) or in the success rates of day surgery (97.8% in LPEC versus 97.6% in OR). The incidence of metachronous contralateral hernias in the LPEC group was lower than that in the OR group (LPEC 0%; OR 2.2%, P=.03). Seventeen subjects with hydroceles were treated by LPEC without any complications. CONCLUSIONS: LPEC is safe and effective for inguinal hernias and hydroceles in children, regardless of age, sex, and incarceration and could reduce the incidence of metachronous contralateral hernias.

Laparoscopic-assisted percutaneous endoscopic gastrostomy: a simple and efficient technique for disabled elderly patients.

BACKGROUND/PURPOSE: Percutaneous endoscopic gastrostomy (PEG) is a simplified catheter placement procedure for alimentation. Although the endoscopic approach to gastrostomy tube placement is a safe and well-tolerated procedure in most patients, the PEG procedure is difficult in elderly patients disabled since childhood who have severe scoliosis and malpositioning of the stomach. We describe a simple and effective laparoscopic-assisted PEG (LAPEG) technique that can be used for catheter placement in severely disabled patients. METHODS: Thirteen severely disabled patients aged 14-57 years underwent gastrostomy tube placement with the LAPEG technique. After general anesthesia was achieved, an endoscope was placed into the stomach. Then, a 5-mm camera port was inserted at the umbilicus, and a 3-mm working port was inserted to identify and lift the optimal site for gastrostomy tube placement. After the 4-point fixation of the stomach, the 20-Fr gastrostomy tube was placed under endoscopic and laparoscopic observation. RESULTS: All patients tolerated the procedure well, and there were no major complications. The procedure was successful, and all patients could feed via the tube. CONCLUSIONS: Elderly disabled patients who have been bedridden since childhood often have severe scoliosis and malpositioning of the stomach. Our LAPEG procedure is effective, well tolerated, and safe for gastrostomy tube placement in such elderly patients.

Successful treatment of a 14-year-old patient with intestinal malrotation with laparoscopic Ladd procedure: case report and literature review.

Midgut malrotation is an anomaly of intestinal rotation that occurs during fetal development and usually presents in the neonatal period. We present a rare case of malrotation in a 14-year-old patient who presented with cramping, generalized right abdominal pain, and vomiting for a duration of one day. A computed tomography abdominal scan and upper gastrointestinal contrast studies showed malrotation of the small bowel without volvulus. Laparoscopy revealed typical Ladd's bands and a distended flabby third and fourth duodenal portion extrinsically obstructing the misplaced duodeno-jejunal junction. The Ladd procedure, including widening of the mesenteric base and appendectomy, was performed. Symptoms completely resolved in a half-year follow up period. Patients with midgut malrotation may present with vague abdominal pain, intestinal obstruction, or intestinal ischemia. The laparoscopic Ladd procedure is feasible and safe, and it appears to be as effective as the standard open Ladd procedure in the diagnosis and treatment of teenage or adult patients with intestinal malrotation.

Torsion of Meckel's Diverticulum in a Child.

Meckel's diverticulum (MD) is a common congenital anomaly of the gastrointestinal tract, the majority of cases of which are clinically silent. Patients with asymptomatic MD can unexpectedly develop acute abdominal pain. Making a diagnosis of MD is often difficult due to the lack of specific symptoms caused by this condition. Diagnostic laparotomy can be useful for making an accurate and prompt diagnosis of complicated MD. We herein describe a pediatric case of torsion of a MD in whom we performed laparoscopic-assisted emergency surgery. The patient was an 11-year-old male who developed sudden severe right lower abdominal pain. Clinical and laboratory findings were suggestive of appendicitis, however computed tomography scans showed a large cystic mass in the pelvis. Exploratory laparoscopy led to a diagnosis of torsion of a MD, and wedge resection of the gangrenous MD was performed through an umbilical port incision. The patient's postoperative course was uneventful. We conclude that diagnostic laparoscopy followed by laparoscopy-assisted Meckel's diverticulectomy via an umbilical incision is useful in the treatment of acute abdomen caused by MD.