Naoko Komatsuzaki

Intestinal neuronal dysplasia-like histopathology in infancy

The present patient was delivered at a gestational age of 27 weeks. She had abdominal bloating with symptoms of respiratory distress. We suspected Hirschsprung disease (HD) or small intestinal stricture, but examinations were not definitive. Exploratory laparotomy was performed at 97 days of age. Intraoperative findings showed no evidence of small intestinal stricture or changes in intestinal caliber. A transanal drainage tube was inserted, and decompression therapy and intestinal lavage were started. Rectal mucosal biopsy was performed at 184 days of age, and HE and acetylcholinesterase staining showed intestinal neuronal dysplasia (IND)‐like pathological findings. At 15 months, giant ganglia were no longer present on follow‐up rectal mucosal biopsy, and the pathological diagnosis was normoganglionosis. It should be recognized that while the enteric nervous system is developing, IND‐like pathological findings may be seen as a pathological condition during the maturation process.

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.

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.

Obstruction of Small Bowel Caused by Unconscious Trichotillomania: Rapunzel Syndrome

Rapunzel syndrome is a relatively rare condition in which swallowed hair masses extend to a point distal to the pylorus and cause intestinal problems. Although trichotillomania and trichophagia are secondary to psychiatric illnesses, these conditions tend to be concealed by caregivers in most pediatric cases. Rapunzel syndrome sometimes manifests as acute abdominal symptoms of unknown origin and is difficult to manage appropriately. A 14-year-old female patient was referred to our hospital due to a small bowel obstruction. Although her medical history of trichotillomania was hidden at presentation, a definitive diagnosis of Rapunzel syndrome was made based on the findings from contrast-enhanced computed tomography (CECT). The patient underwent exploratory laparotomy, and hairballs were removed via an incision of the gastric and intestinal walls. The patient recovered uneventfully and was discharged on the 11th post-operative day. Periodical psychiatric counseling is ongoing in order to prevent recurrence. Pediatric trichotillomania often progresses unconsciously, and appropriate care is necessary to prevent the development of a potentially fatal condition like Rapunzel syndrome. CECT is useful for making a definitive diagnosis of Rapunzel syndrome and determining the surgical approach. Radical laparotomy is considered to be reasonable and feasible in cases of Rapunzel syndrome, which manifests as acute abdominal symptoms.

Near-infrared fluorescence cholangiography with indocyanine green for biliary atresia. Real-time imaging during the Kasai procedure: a pilot study

IntroductionHepatoportoenterostomy (HPE) with the Kasai procedure is the treatment of choice for biliary atresia (BA) as the initial surgery. However, the appropriate level of dissection level of the fibrous cone (FC) of the porta hepatis (PH) is frequently unclear, and the procedure sometimes results in unsuccessful outcomes. Recently, indocyanine green near-infrared fluorescence imaging (ICG-FCG) has been developed as a form of real-time cholangiography.MethodsWe applied this technique in five patients with BA to visualize the biliary flow at the PH intraoperatively. ICG was injected intravenously the day before surgery as the liver function test, and the liver was observed with a near-infrared camera system during the operation while the patient’s feces was also observed.ResultsIn all patients, the whole liver fluoresced diffusely with ICG-containing stagnant bile, whereas no extrahepatic structures fluoresced. The findings of the ICG fluorescence pattern of the PH after dissection of the FC were classified into three types: spotty fluorescence, one patient; diffuse weak fluorescence, three patients; and diffuse strong fluorescence, one patient. In all five patients, the feces evacuated after HPE showed distinct fluorescent spots, although that obtained before surgery showed no fluorescence. One patient with diffuse strong fluorescence who did not achieve JF underwent living related liver transplantation six months after the initial HPE procedure. Four patients, including three cases involving diffuse weak fluorescence and one case involving spotty fluorescence showed weak fluorescence compared to that of the surrounding liver surface.ConclusionWe were able to detect the presence of bile excretion at the time of HPE intraoperatively and successfully evaluated the extent of bile excretion using this new technique. Furthermore, the ICG-FCG findings may provide information leading to a new classification and potentially function as an indicator predicting the clinical outcomes after HPE.

Infantile neuroblastoma of the urinary bladder detected by hematuria

Malignant tumors of the urinary bladder in infants are extremely rare. Rhabdomyosarcoma is the most likely tumor in this site, whereas neuroblastoma of the urinary bladder is exceedingly uncommon and is not listed as a differential diagnosis for tumors of this site. We present a case of neuroblastoma arising from the dome of the bladder wall, detected by hematuria. Only six cases of neuroblastoma originating from the bladder, including the present case have been reported. Of the cases, five arose from the dome of the bladder wall. In this report, the differential diagnosis of bladder tumors in children is discussed. A diagnosis of neuroblastoma should be taken into consideration, especially in the case of tumors arising from the dome of the bladder wall despite an uncommon location.

Infantile juvenile xanthogranuloma of the chest wall mimicking mesenchymal hamartoma: report of a case

Juvenile xanthogranuloma (JXG) is essentially a benign neoplasm arising from any site on the body; however, there has so far been only one report of JXG located on the chest wall involving a rib. This report presents a rare case finally diagnosed as JXG based on histopathological and immunohistochemical examinations.