Ureteral Sextuplication With a Blind-ending Ureter Originating From the Upper Pole in a Boy: A Case Report.

We experienced an extremely rare case of ureteral sextuplication with a blind-ending ureter originating from the upper pole. The patient had 6 separate ureters and 6 renal pelvises with 1 ureteral orifice on the left. The left kidney was hypoplastic and its differential function was 6.5%. The patient underwent definitive surgical treatment to repair the anomaly (ureteroureterostomy and reimplantation of the formed ureter). The surgery was successful and the postoperative course was uneventful. We herein report the first such case in the English-language literature and discuss the etiology of this ureteral anomaly, the method of diagnostic imaging, and the treatment.

Surgical approach for fecal incontinence with a patulous anus after transanal pull-through for Hirschsprung disease.

BACKGROUND: We have performed transanal pull-through (TAPT) for Hirschsprung disease since 1998. Some of our patients after TAPT showed a patulous anus and suffered from severe true fecal incontinence. We performed anal canal plasty for these patients and evaluated its efficacy in restoring anorectal function. METHODS: Thirty-one patients who were ≥5years old were previously operated on for Hirschsprung disease, and seven (22.5%) of these were indicated for this procedure. Anorectal function was evaluated using the Japanese Study Group of Anorectal Anomalies (JSGA) clinical assessment of defecation function score. For surgery, the patients were positioned in the prone jackknife posture. The posterior half of the anal canal was exposed and folded inward until the anal canal lumen was as narrow as the surgeon's index finger. External anal sphincter muscles were repaired, and the wound was closed vertically. RESULTS: The mean preoperative JSGA score was 1.42±0.4. The mean JSGA scores at 2-6months and 2years after this procedure were 5±2.1 and 5.8±2.1, respectively. Postoperatively, the JSGA score significantly improved at both times (p<0.05). CONCLUSIONS: Anal canal plasty may be effective for true fecal incontinence and a patulous anus after TAPT. This surgical approach may be useful for these conditions. LEVEL OF EVIDENCE: Type of study: Treatment study, Level IV.

Cartilage-hair hypoplasia associated with isolated hypoganglionosis: A case report.

Cartilage-hair hypoplasia is a rare metaphyseal chondrodysplasia characterized by diverse clinical manifestations and a high incidence of Hirschsprung disease. We present a male patient with cartilage-hair hypoplasia associated with severe intestinal obstruction. Genetic analysis of ribonuclease mitochondrial RNA-processing complex gene identified compound heterozygous mutations consisted with previously reported mutations: n.-14_3dupGAAGCTGAGGACGTGGT and n.183G > T. First, we considered that intestinal obstruction was due to an extensive type of Hirschsprung disease, but it was later confirmed as isolated hypoganglionosis. Isolated hypoganglionosis is rare and its therapeutic strategies are not well established. In cases of cartilage-hair hypoplasia associated with severe intestinal obstruction, the differential diagnosis of not only Hirschsprung disease, but also isolated hypoganglionosis, should be considered.

[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.