Risk factors for complications in patients with Hirschsprung disease while awaiting surgery: Beware of bowel perforation

Determining the Correct Resection Level in Patients with Hirschsprung Disease Using Contrast Enema and Full Thickness Biopsies: Can the Diagnostic Accuracy be Improved by Examining Submucosal Nerve Fiber Thickness?

“…We found that a contrast enema is not sufficient in patients with a long-segment disease and TCA, which is in line with results of other studies [32]. Next, our findings emphasize the importance of creating new diagnostic tools to preoperatively estimate the correct length of disease for these patients [5]. Ultra-high frequency ultrasound can be the future solution for this problem, which is recently tested for intraoperative ex vivo determination of the resection level [33].…”

“…In case there were inconsistencies, the complete record was checked by the other author not involved in the initial extraction of data. The following patient characteristics were extracted: sex (male/female), contrast enema (yes/no), visible caliber change on contrast enema (yes/no), location of caliber change on contrast enema (recto-sigmoid (defined as proximal to lineadentate), sigmoid, sigmoid-descendens, colon descendens, flexura lienalis, colon transversum, flexura hepatica, colon ascendens, cecum, or ileum), age at time of biopsy (weeks), type of surgery (single-stage, two-stage), total number of FTBs, pathological results of FTBs (ganglionated, aganglionated, or non-assessable), length of resected bowel specimen measured by the pathologist (cm), length of diseased segment (short-segment was defined as aganglionosis extending to the rectosigmoid, long-segment as aganglionosis extending to the proximal colon or TCA) [5], location of TZ (recto-sigmoid (defined as proximal to linea-dentata), sigmoid, sigmoid-descendens, colon descendens, flexura lienalis, colon transversum, flexura hepatica, colon ascendance, cecum, or ileum) and TZPT (yes/no). In patients in which redo surgery was performed, the following procedure characteristics were collected: intraoperative FTB (yes/no), number of intraoperative FTB, pathological result of FTB (ganglionated, aganglionated, or non-assessable) and length of resected segment measured by pathologist (cm) and TZPT (yes/no).…”

“…After the diagnosis of HD, surgery is planned, aimed at resecting the diseased bowel section. Surgery can take place in single-stage or in two-stages, with the latter being preferred in patients with a long diseased bowel (diseased bowel extending from the rectosigmoid) [5].…”

Diagnosing Hirschsprung Disease in Children Younger than 6 Months of Age: Insights in Incidence of Complications of Rectal Suction Biopsy and Other Final Diagnoses

“…Such a low threshold approach is expected to prevent complications caused by delayed or inadequate treatment and the need to use FTB to confirm a diagnosis of HD at an older age, which requires general anesthesia and is associated with a higher complication rate compared with RSB. 14,31 The second aim of our study was to investigate the final diagnosis of patients referred for RSB. After HD, the most frequent final diagnosis assigned was functional constipation.…”

“…12,13 Also, inadequate bowel decompression can lead to possibly life-threatening complications, underlining the importance of early recognition of HD. 14 However, recognition based on clinical findings remains troublesome. As a consequence, the threshold to take RSB in patients with potential HD is low.…”