Anatomical alterations following the 'PSARP' procedure: Correlating MRI findings with continence scores.

PURPOSE: To identify anatomical aberrations following PSARP procedure by using MRI, while correlating MRI findings to clinical outcome. PATIENTS AND METHODS: Between January 2014 and December 2017, we conducted our study on male patients with rectourethral fistula who underwent PSARP. Postoperative pelvic MRI studies were performed and correlated to their clinical continence scores (Rintala, and Krickenbeck classification). RESULTS: The study included 31 patients. Fourteen patients were retrieved from the hospital records and accepted to participate in the study; while the remaining 17 were collected from the fecal incontinence clinic. Their age ranged from 40 to 156 months (mean 83) We divided patients in the study into two groups according to their Rintala continence scores: (Group A) 15 patients with low scores (10 or less); and (Group B) 16 patients with higher scores (more than 10). We detected wider pelvic hiatus (hiatus/PC ratio) and more obtuse anorectal angle in group A than B. CONCLUSION: Several anatomical alterations can be detected by MRI following the PSARP procedure that include abnormalities in the striated muscle sphincter (attenuation/deficiency), deviated neorectum, and presence of excessive perirectal fat. A widened pelvic hiatus and/or obtuse anorectal angle may correlate with poor fecal continence in these patients. LEVEL OF EVIDENCE: This is a case control study (level III evidence).

Colonic conduit for esophageal replacement: long-term endoscopic and histopathologic changes in colonic mucosa.

BACKGROUND: Long-term follow-up has substantiated the colon as a durable and highly acceptable esophageal substitute. Exposure of colonic conduit to gastric acid may lead to histopathologic changes in the form of chronic inflammation. MATERIALS/METHODS: Thirty children with esophageal replacement were studied from 2 to 12 years (mean, 5.20 years) postoperatively. All cases underwent upper gastrointestinal tract endoscopy to evaluate the gross appearance of colonic conduit mucosa, and punch biopsies were taken from upper and lower junctions of the conduit. All biopsies were submitted to histopathologic examination. RESULTS: Endoscopic findings were comparable with normal regarding the gross appearance of colonic mucosa in both upper and lower junctions (25 cases/83.3%). Some abnormalities were seen including cervical anastomosis stricture (2 cases/6.7%), redundancy (3 cases/10%), mucosal ulcer in the lower residual esophagus (1 case/3.3%), and hyperemia (3 cases/10%). Pathologic changes were minimal regarding the change in position of the colon to a thoracic organ during follow-up. Most of the cases were normal (22 cases/73.3%). Seven cases (23.4%) showed mild chronic nonspecific inflammation of the colonic mucosa, whereas only 1 case (3.3%) showed mildly active inflammation of colonic mucosa. CONCLUSION: The use of the colon for esophageal replacement showed that no significant pathologic changes affecting its function as a conduit because its mucosa showed no significant change in response to gastric acid reflux in long-term follow-up and can be further protected by an antireflux procedure.

Risk of bleeding and inhibitor development after circumcision of previously untreated or minimally treated severe hemophilia A children.

BACKGROUND: Surgery and intensive factor VIII (FVIII) replacement may be risk factors for development of inhibitors. OBJECTIVE: To evaluate time and rate of inhibitor development postcircumcision over 12-month period, and to assess bleeding of children with severe hemophilia A after low-dose FVIII replacement and local hemostasis. PATIENTS AND METHODS: Sixty-one previously untreated patients (PUPs) or minimally treated patients (MTPs) with severe hemophilia A less than 36 months were enrolled; 25 underwent circumcision during the 18-month enrollment period, and 36 matched patients were not circumcised. All patients were treated on demand with plasma-derived FVIII, and all were inhibitor negative at the time of enrollment. Intron 22 inversion was analyzed. A potent hemostatic agent (gelatin sponge) was applied on the site of surgery, and then dressed with gauze. Two doses of FVIII concentrate (25 U/kg) were given, 1 hour before circumcision and 1 hour before removal of dressing. The inhibitor was determined every 8 exposure days (EDs). RESULTS: None of the patients had bleeding or infection, except one who had minimal transient bleeding 8 days after surgery, and was treated easily by a single dose of FVIII (50 U/kg). After a median of 16 EDs, high-titer inhibitors developed in seven patients: three patients in the circumcised group (12%) in contrast to four patients (11.1%) in the noncircumcised group. CONCLUSION: Two doses factor concentrate and gelatin sponge application were generally enough to prevent bleeding after circumcision of severe hemophilia A. Circumcision and low-dose FVIII protocol were not an additional risk for development of high-titer inhibitor.