Kimio Asagiri

A case of split notochord syndrome with congenital ileal atresia, the total absence of a colon, and a dorsal enteric cyst communicating to the retroperitoneal isolated ceca with a vesical fistula.

Split notochord syndrome (SNS) is an extremely rare anomaly. This report presents the case of a male infant with SNS associated with congenital ileal atresia and a dorsal enteric cyst communicating to the retroperitoneal isolated ceca with a vesical fistula. Dorsal fistulography and vesicography were useful and essential for the detailed study of the topology in this patient. The embryological mechanism and etiologic theories are discussed with a review of 19 cases reported in the literature.

Mesenchymal Hamartoma of the Liver Accompanied by a Daughter Nodule: Report of a Case

Mesenchymal hamartoma of the liver (MHL) is an uncommon benign tumor found primarily in children younger than 2 years of age. We report a rare case of MHL with a daughter nodule and atypical histological findings in a 14-month-old girl. On admission, computed tomography, magnetic resonance imaging, and angiography showed a solid hypovascular mass with a central cystic area in the liver. Laparotomy revealed a tumor, 8 cm in size, occupying segment 5 and parts of segments 4 and 6 of the liver, and a small nodule, 10 mm in size, in segment 7. Thus, we performed a partial hepatic resection (S4–6) and tumor extirpation (S7). The histological findings of both tumors were the same, but atypical of MHL. Recent studies on the pathogenesis of this tumor have found neoplastic features such as genetic anomalies and malignant transformation. These findings suggest that the conventional approach of completely resecting the tumor whenever possible is the best treatment.

Laparoscopic Nissen fundoplication mainly reduces the volume of acid reflux and potentially improves mucosal integrity up to the middle esophagus in neurologically impaired children detected by esophageal combined pH–multichannel intraluminal impedance measurements

BACKGROUND The aim of this study was to evaluate detailed changes of gastroesophageal reflux disease (GERD) in children before and after laparoscopic Nissen fundoplication (LNF) utilizing esophageal combined pH-multichannel intraluminal impedance (pH/MII) measurements. PATIENTS AND METHODS Thirteen patients with neurological impairment, who received laparoscopic Nissen fundoplication (LNF) owing to refractory pathological GERD, were enrolled in this study. 24h pH/MII was conducted in all patients before and one year after LNF. Each parameter of the pH/MII was evaluated and compared each other. RESULTS Both the mean pH index and bolus exposure index after LNF were significantly lower than those before LNF. The mean numbers of total and nonacid reflux episodes after LNF were significantly lower than those before LNF. The mean numbers of total, acid and nonacid proximal reflux episodes after LNF were significantly lower than those before LNF. The mean baseline impedance values from Z3 to Z6 after LNF were significantly higher than those before LNF. CONCLUSIONS The present study provides new insights into the effects of LNF in pediatric patients, which prevents mainly acid reflux episodes from rising to the proximal esophagus, and is expected to improve the mucosal integrity up to the middle esophagus.

Clinical Efficacy of Herbal Medicine for Pediatric Lymphatic Malformations: A Pilot Study

Lymphatic malformations (LMs) are congenital malformations of the lymphatic system that commonly affect the head and neck region and cause marked cosmetic and functional complications. In this pilot study, we present eight children with LMs treated using an herbal medicine for this indication.

Thoracic Lymphangiomatosis with Massive Chylothorax After a Tumor Biopsy and with Disseminated Intravenous Coagulation-Lymphoscintigraphy, an Alternative Minimally Invasive Imaging Technique : Report of a Case

Thoracic lymphangiomatosis in childhood is a rare disorder that frequently has poor response to medical therapy as well as a poor prognosis. This disease is often misdiagnosed, and a definitive diagnosis is usually delayed because of the rarity and slow course of the disease. No previous reports have so far described the usefulness of lymphoscintigraphy in the diagnosis of lymphangiomatosis, although some authors have reported the efficacy of lymphoscintigraphy for evaluating chylothorax. A 6-year-old boy presented with a diffuse mediastinal mass and received an open mediastinal biopsy for a definitive diagnosis, which led to the occurrence of massive chylothorax postoperatively. A diagnosis of lymphangiomatosis was finally made based on the lymphoscintigraphic findings demonstrating an obstruction of the thoracic duct. This report describes a rare case of thoracic lymphangiomatosis diagnosed at autopsy, and suggests that the minimally invasive technique of lymphoscintigraphy should be employed to obtain a definitive diagnosis at an early stage.

A baseline impedance analysis in neurologically impaired children: A potent parameter for estimating the condition of the esophageal mucosa

The aim of the present study was to investigate whether the baseline impedance (BI) value is a useful parameter to evaluate the condition of the esophageal mucosa in neurologically impaired (NI) children undergoing multichannel intraluminal impedance pH measurements (pH/MII).

The utility of a phase angle analysis in patients with severe motor and intellectual disabilities

PURPOSE The purpose of the present study was to evaluate whether evaluating the phase angle (PhA), in a bioelectrical impedance analysis (BIA) is useful for estimating the nutritional status of severe motor and intellectual disabilities (SMID) patients. SUBJECTS AND METHODS This retrospective study included 31 SMID patients (mean age: 33.9±13.5years, median age: 29years (range: 18-58years), male/female: 23/8). First, each of the parameters from the total study population and the male and female SMID patients were compared with those of healthy Asian subjects. Second, correlation analyses were conducted to investigate the correlation between the PhA and the other BIA parameters (appendicular skeletal muscle mass index (ASMI), appendicular muscle mass (AMM), extracellular water (ECW)/total body water (TBW)) as well as subjective global assessment and serum nutritional markers. Finally, all patients were divided into 2 groups according to their albumin (Alb) (<3.5 or ≥3.5) values and PhA of the 2 groups were compared. RESULTS The mean PhA and ASMI were a considerably low, whereas ECW/TBW was considerably high in comparison to the healthy Asian subjects. Significant negative correlations were observed between the PhA and ECW/TBW, whereas there were significant positive correlations between PhA and AMM, ASMI, total protein and albumin levels. Furthermore, PhA of Alb ≥3.5 group was significantly higher than that of Alb <3.5 group. CONCLUSIONS The present study indicated that SMID patients demonstrate the low PhA, which were similar to sarcopenia and a certain proportion of them also potentially have nutritional disturbances.

Analyses of the relationship between a ‘number of reflux episodes’ exceeding 70 and the pH index in neurologically impaired children by evaluating esophageal combined pH-multichannel intraluminal impedance measurements

Abstract Background: The present study aimed to evaluate the characteristics associated with a number of reflux episodes (NoRE) of 70 by comparing the clinical and multichannel intraluminal impedance pH measurements (pH/MII) and pH index (pHI) in neurologically impaired (NI) children. Patients and methods: NI children (1–16 years of age) in whom pH/MII had been measured for GERD study were enrolled in this study. All children were divided into NoRE >70 or ≤70 and pHI >4.0 or ≤4.0, >5.0 or ≤5.0 or >7.0 or ≤7.0. In addition, the NI children with pHI >4.0, >5.0 and >7.0 were subdivided into NoRE >70 and ≤70 groups. The clinical and pH/MII measurements were compared between each of the two groups. The cutoff values of pHI and baseline impedance (BI) (Z6) were calculated to discriminate NoRE >70 and ≤70. Results: A total of 61 NI children were enrolled in this study. There was a significant difference in the acid-related parameters, the NoRE (nonacid) and BI between NoRE >70 and ≤70, acid-related parameters and BI between pHI >4.0 and ≤4.0, >5.0 and ≤5.0 and >7.0 and ≤7.0 groups. Furthermore, a significant difference was still observed in the BI between NoRE >70 and ≤70 groups among patients with pHI >4.0, >5.0 or >7.0. The cutoff values of pHI and BI (Z6) for discriminating NoRE >70 and ≤70 were 9.2 and 1049Ω, respectively. Conclusion: The present study indicates that NoRE 70 corresponds to GERD in which patients suffer severe acid exposure with pH of around 9% and esophageal mucosal damage with low BI value in NI children.

Perineal lipoma with anorectal malformation: Report of two cases and review of the literature

A female newborn presented with a 1 cm perineal mass located in the right labium majus with accessory labioscrotal fold and anovestibular fistula (Fig. S1). Preoperatively, Hegar dilatation and a glycerin enema were used for defecation management via the fistula without a protective colostomy. At 6 months of age, excision of the mass and anterior sagittal anorectoplasty (ASARP) were performed. Although superficial wound dehiscence of the right labium majus occurred, it was treated with local wound care and antibiotics. Histology of the perineal mass indicated adipose tissue covered by normal skin. No complications such as constipation or anal stenosis occurred during 1 year of follow up.

Severe Delayed Gastric Emptying Induces Non-acid Reflux up to Proximal Esophagus in Neurologically Impaired Patients

Background/Aims The aim of this study is to investigate the degree of delayed gastric emptying (DGE) and evaluate how the severity of DGE affects gastroesophageal reflux disease (GERD) in neurologically impaired (NI) patients utilizing 24-hour multichannel intraluminal impedance pH measurements (pH/MII) and 13C-acetate breath test (13C-ABT) analyses. Methods 13C-ABT and pH/MII were conducted in 26 NI patients who were referred to our institution due to suspected GERD. At first, correlation analyses were performed to investigate the correlation between the 13C-ABT parameters and the clinical or pH/MII parameters. Thereafter, all patients were divided into 2 groups (DGE and severe DGE [SDGE] group) according to each cut off half emptying time (t1/2, 90–170 minutes). Each pH/MII parameter was compared between the 2 groups in each set-up cutoff t1/2. Results The mean t1/2 of all patients was 215.5 ± 237.2 minutes and the t1/2 of 24 (92.3%) patients were > 100 minutes. Significant moderate positive correlations were observed between both t1/2 and lag phase time and the non-acid reflux related parameters. Furthermore, the patients in the SDGE group demonstrated higher non-acid reflux related parameters than those of the DGE groups when the cutoff was t1/2 ≥ 140 minutes. Conclusion The present study demonstrated that GE with t1/2 ≥ 140 minutes was related to an increase of non-acid exposure reaching up to the proximal esophagus in NI patients, and indicating that NI patients with SDGE might have a high risk of non-acid GERD.