Laparoscopic surgery for urachal remnants in pubescent children: a case series.

BACKGROUND: Various techniques are applied in laparoscopic surgery for the treatment of urachal remnants, which are less invasive and associated with lower morbidity. We herein report a case series in which we treated urachal remnants and medial umbilical ligaments using a laparoscopic approach. CASE PRESENTATION: From 2015 to 2019, seven patients (male, n = 5; female, n = 2) with a urachal remnant were treated by laparoscopic surgery in our institute. Five boys and two girls with a median age of 11 years (range 10-15 years) were enrolled in this series. The clinical results of laparoscopic treatment, the perioperative records, and the pathologic results were evaluated. The operation was performed with the use of three ports and an EZ access® (Hakko Medical, Nagano, Japan), which is a silicon cap for the wound retractor (Lap Protector®, Hakko Medical, Nagano, Japan). The removal of the urachal remnant and medial umbilical ligaments was completed with a median operative time of 92 min (range 69-128). The median hospital stay after surgery was 4 days (range 2-5). No patients developed intra-postoperative complications or recurrence. CONCLUSIONS: Although our data are preliminary, complete laparoscopic removal of symptomatic urachal remnants and medial umbilical ligaments was a safe and effective minimally invasive approach, with better cosmetic outcomes.

Does clinical score accurately support fecoflowmetry as a means to assess anorectal motor activity in pediatric patients after anorectal surgery?

PURPOSE: We investigated the relationship between Krickenbeck score (KS) and fecoflowmetry (FFM) parameters and assessed the characteristics of this new questionnaire test by comparing Kelly's clinical score (KCS) in pediatric patients with anorectal surgery for anorectal malformation (ARM) and Hirschsprung's disease (HD). METHODS: We enrolled pediatric patients who underwent anorectal surgery for ARM or HD. Bowel function was assessed with KS and KCS thereafter, FFM and anorectal manometry (AM) were conducted. Patients were divided into subgroups according to each parameter of the scoring system and each FFM parameter was compared among the KCS or KS subgroups, respectively. Moreover, correlation analyses were conducted between FFM and AM parameters. RESULTS: The comparison of FFM parameters among the subgroups of KCS showed that Fmax in the KCS staining 2 group was significantly higher than that in KCS staining 1 group and the Fmax in KCS sphincter squeeze 1 group was significantly higher than that in KCS sphincter squeeze 0 group. Moreover, Fmax in the KCS "good" group was significantly higher than that in the KCS "fair" group. The comparison of FFM parameters among the subgroups of KS parameters showed that TR in the no soiling group was significantly higher than that in the KS grade 2 soiling group. FFM and AM parameters showed a significant positive correlation between Fmax and voluntary squeezing anal pressure. CONCLUSION: FFM clarified the different characteristics of two scoring systems, namely, KCS reflects the anal sphincter performance, whereas the KS soiling score might reflect the tolerance and evacuation ability.

[Usefulness of Palliative Gastrojejunal Bypass Surgery].

OBJECTIVES: The aim of this study was to evaluate the usefulness of gastrojejunal bypass surgery performed in patients presenting with upper gastrointestinal tract obstruction due to unresectable advanced cancer. SUBJECTS AND METHODS: The subjects were 21 patients who underwent gastrojejunal bypass surgery at our division between 2010 and 2014 for symptom palliation. We retrospectively evaluated the operative outcomes, whether chemotherapy was administered, the oral ingestion period, and survival time. RESULTS: The median postoperative day of starting oral ingestion was 6 (range: 2-42), and the median period from decreased oral ingestion to death was 4 (range: 0-26) days. Twelve patients (57%) were discharged. Postoperative chemotherapy was prescribed to all the 9 patients who desired treatment. The median duration of oral digestion time was 61 days, and the median overall survival time was 92 days. CONCLUSION: Gastrojejunal bypass surgery is found to have the potential to not only make relatively long-term oral ingestion possible, but also broaden available treatment options, such as home care or chemotherapy, thereby contributing to improved quality of life.

A case of a choledochal cyst associated with a lymphatic infiltration of a hyperplastic gallbladder epithelium.

A 4-year-old girl with a congenital choledochal cyst (Todani IV-A, Komi type A) underwent a resection of the dilated common bile duct and gallbladder. Histologic studies of the gallbladder showed a general hyperplastic change associated with cribriform proliferation at the gland base of the gallbladder. In this region, clusters of cribriform glands were found within the lymphatic vessels, compatible with lymphatic infiltration of tumor cells. However, careful histologic studies did not reveal any apparent neoplastic changes in the gallbladder and common bile duct, so a final diagnosis of epithelial atypism with reactive hypertrophy was made. The displacements observed in the lymphatics are just an incidental finding in a proliferative process of the hyperplastic gallbladder epithelium. In the follow-up observation for 3 years, the patient is doing well without evidence of tumor recurrence. These results suggest that a mere lymphatic infiltration of hyperplastic gallbladder epithelium should not be directly considered as evidence of carcinogenesis.

Relationship between the results of in vitro receptor binding assay to human estrogen receptor alpha and in vivo uterotrophic assay: comparative study with 65 selected chemicals.

For screening chemicals possessing endocrine disrupting potencies, the uterotrophic assay has been placed in a higher level in the OECD testing framework than the ER binding assay to detect ER-mediated activities. However, there are no studies that can demonstrate a clear relationship between these assays. In order to clarify the relationship between the in vitro ER binding and in vivo uterotrophic assays and to determine meaningful binding potency from the ER binding assay, we compared the results from these assays for 65 chemicals spanning a variety of chemicals classes. Under the quantitative comparison between logRBAs (relative binding affinities) and logLEDs (lowest effective doses), the log RBA was well correlated with both logLEDs of estrogenic and anti-estrogenic compounds at r(2)=0.67 (n=28) and 0.79 (n=23), respectively. The RBA of 0.00233% was found to be the lowest ER binding potency to elicit estrogenic or anti-estrogenic activities in the uterotrophic assay, accordingly this value is considered as the detection limit of estrogenic or anti-estrogenic activities in the uterotrophic assay. The usage of this value as cutoff provided the best concordance rate (82%). These findings are useful in a tiered approach for identifying chemicals that have potential to induce ER-mediated effects in vivo.

Sacral parasite with histopathological features of an unequally conjoined twin.

A sacral parasite is a rare congenital anomaly classified etiologically as duplicitas asymmetros, where the parasitic mass is regarded as the tissue remnants of an unequally conjoined twin. We, herein, report a case of a sacral parasitic mass found in a female neonate. A huge sacral mass was found at 30 weeks of gestation by fetal ultrasonography. The fetus was delivered by a Caesarean operation at 37 weeks and 6 days of gestation. The alpha-fetoprotein (AFP) level was 174,640 ng/ml. The mass separated by an operation on the day after her birth, contained structures similar to imperfect limbs and backbone. A variety of tissue and organs having derived from three germ layers were identified within the mass and showed a tendency to be highly differentiated. Even though a large proportion of the central nervous system tissue showed immature and dysplastic features, there were no evidences suggesting the presence of neoplasm. The infant has shown normal growth and is doing well without a recurrence of the sacral mass or any elevation in the serum AFP level during the12-month follow-up. We therefore believe that the present case should be considered a sacral parasite, rather than a sacrococcygeal teratoma. Although it should be recognized that such sacral parasite is very rare, this case provides further information that can be useful for diagnosis.

Treatment of a case with right-sided diaphragmatic hernia associated with an abnormal vessel communication between a herniated liver and the right lung.

We herein report a rare case of a newborn girl with a right-sided congenital diaphragmatic hernia where a herniated liver had an abnormal vessel communication with the right lung. A surgical repair was initially attempted through a thoracic approach at 4 days of age where only a plication of the hernia capsule was performed without a prominent improvement in the respiratory condition. At 1 year of age, an angiographic examination was performed, which revealed pulmonary hypertension and abnormal vessel communications where the right pulmonary flow returned to the herniated liver and the right lung also received an arterial supply from the liver. During the second surgical repair performed at 1 year and 1 month of age, an abdominal approach through a right subcostal incision was selected. The aberrant vessels between the lung and the liver were carefully identified and ligated. Because the right lobe of the liver was completely herniated, a hepatic segmentectomy of S6 and S7 was performed. The patient has been doing well for 21 months without any mechanical ventilation since 2 months after undergoing the radical second operation. When performing surgery on a right-sided diaphragmatic hernia, the potential presence of such a vessel anomaly should be carefully taken into consideration.

An 11-year-old male patient demonstrating cholangiocarcinoma associated with congenital biliary dilatation.

We herein report an 11-year-old male patient demonstrating advanced cholangiocarcinoma associated with congenital biliary dilatation (CBD). This Japanese boy presented with abdominal pain lasting a few days, and a diagnosis of type IV-A CBD was made based on the findings of imaging studies using ultrasonography, computed tomography, and magnetic resonance imaging. These studies also revealed a wide flat lesion associated with a few small round masses in the dilated choledochus. Intraoperative cholangiography revealed the presence of pancreaticobiliary maljunction of CP type. Malignant cells were found in biopsy specimens from both the flat and polypoid lesions. Because the distal stump of choledochus at anomalous confluent to the pancreatic duct also showed malignant cells, a pyloric preserved pancreaticoduodenectomy with lymph node dissection was thus performed. Lymph node metastasis was found in one of the mesenteric lymph nodes, and vascular invasion was also found in the main tumor lesions of the dilated bile duct. The postoperative course was uneventful, and the patient has been followed with chemotherapy as an outpatient without any evidence of recurrence. To our knowledge, this report is the youngest case of cholangiocarcinoma associated with CBD.

A disturbance of the gastric myoelectric activity in post-operative patients with biliary atresia.

BACKGROUND: Abnormalities in gastrointestinal motility have been reported in adult patients with advanced liver disease. However, there have so far been no reports on the gastric myoelectric activity in post-operative patients with biliary atresia (BA). AIM: The purpose of this study was to evaluate the gastric myoelectric activity in post-operative patients with BA in relation to liver fibrosis. PATIENTS AND METHODS: Twenty-one post-operative patients with BA, consisting of 6 boys and 15 girls with a mean age of 8.0 years and 6 healthy children (control group) were included in the study. The gastric myoelectric activity was measured by electrogastrography (EGG). The patients with BA were divided into two groups according to the serum hyaluronic acid (HA) level as a marker of liver fibrosis: the fibrotic group (FG, n=11), HA>50 ng/ml and the non-fibrotic group (NF, n=10), HA <==50 ng/ml. All recorded data were spectrally analyzed and any parameters related to changes in the dominant peak frequency (DPF) and its power were investigated. Furthermore, the gastrointestinal symptom scores (GSS) were calculated in patients with dyspeptic symptoms according to the degree of advanced liver fibrosis. RESULTS: The results showed that 1) the postprandial DPF in the FG tended to be higher than that in the NFG ( p=0.051), 2) the postprandial variability index of the DPF in the FG and NFG were significantly higher than those in the controls ( p<0.05), and 3) the preprandial percentage of normal waves (PNW) in the FG tended to be lower than that in the controls ( p=0.089). The postprandial PNWs in the FG and NFG were significantly lower than those in the controls ( p<0.05). Especially, the postprandial PNW in the FG was significantly lower than that in the NFG ( p<0.05). 4) The power ratio in the FG and NFG were significantly lower than those in the controls ( p<0.05), and 5) the GSSs in the FG were significantly higher than those in the NFG ( p<0.05). CONCLUSIONS: The gastric myoelectric activity appeared to be disturbed in BA patients associated with portal hypertension and neurohormonal changes due to liver fibrosis.