Anal duplication: is surgery indicated? A report of three cases and review of the literature.

AIM OF THE STUDY: Anal canal duplications (ACDs) are extremely rare with only approximately 90 cases described in the literature. We report on three additional patients. METHODS: Cases were analyzed to evaluate presenting symptoms, physical exam and MRI findings. A comprehensive literature review was performed to compare our patients to previously described cases. IRB approval was obtained for this study (19-0394). MAIN RESULTS: The first female patient presented with an asymptomatic ACD at 2 years old. The second patient was a 13-year-old female with perianal drainage that was initially mistaken for a fistula-in-ano and ultimately found to have an ACD associated with a dermoid cyst. Both posterior midline duplications shared a common wall with the rectum, but did not communicate with it. The ACDs and dermoid cyst were successfully excised through a posterior sagittal approach with no postoperative complications. Histology demonstrated the presence of both squamous epithelium and transitional anal epithelium in each case. The third patient was 8 months old and had a tethered cord, hemisacrum, presacral mass, and anal duplication that was initially undiagnosed. These results corroborate patterns identified in other reports of ACDs with over 90% being female and in the posterior midline. The majority are asymptomatic, but may present with symptoms of local or even systemic infection. CONCLUSION: An opening in the midline posterior to the anus should raise clinical suspicion for anal canal duplication. An associated presacral mass must be ruled out. Complete excision through a posterior sagittal approach is recommended upon diagnosis to avoid symptomatic presentations. The key part of the operation is the separation of the ACD from the posterior rectal wall.

Development of a postoperative care pathway for children with renal tumors.

PURPOSE: To identify the factors associated with a shorter postoperative stay, as an initial step to develop a care pathway for children undergoing extirpative kidney surgery. STUDY DESIGN: This study retrospectively reviewed patients managed with upfront open radical nephrectomy for renal tumors between 2005 and 2016 at a pediatric tertiary care facility. Univariate and multivariate logistic regression were performed to identify factors associated with early discharge (by postoperative day 4). RESULTS: A total of 84 patients met inclusion criteria. Median age was 28.1 months (range 1.8-193.1). Thirty-four (40.5%) patients had a nasogastric tube postoperatively. The patients were advanced to a clear liquid diet on a median postoperative day 2 (range 0-7) and regular diet on a median postoperative day 3 (range 1-8). Median time from surgery to discharge was 5 days (range 2-12), with 38 (45.2%) discharged early. Univariate and multivariate logistic regression analyses showed that earlier resumption of regular diet (OR 0.523, P = 0.028) was positively associated with early discharge. Other analyzed factors were not significant (see Table). DISCUSSION: Timely initiation of adjuvant therapy is a specific requirement of Children's Oncology Group (COG) protocols. Chemotherapy and radiation therapy are ideally initiated simultaneously, as early as possible, within 2 weeks of surgery. Thus, factors that can facilitate early discharge from the hospital can maximize protocol adherence with respect to timing of adjuvant therapy initiation and optimize patient outcome. This study shed light on several postoperative factors and how these relate to postoperative stay and recovery. Specifically, tumor size, pre-operative bowel preparation, extent of lymph node sampling, stage, operative time, estimated blood loss, surgical service, postoperative nasogastric tube use, transfusion, and chemotherapy prior to discharge were not associated with discharge timing. Early re-feeding was associated with early discharge. Thus, it seems reasonable that, when developing a postoperative care pathway for these patients, these factors be considered and specifically encourage early re-feeding. In pediatrics, data on early recovery after surgery protocols are limited, and high-quality studies are unavailable. Within pediatric urology, early recovery after surgery protocols in children undergoing major urologic reconstruction have been shown to reduce hospital stay and can decrease complication rates. It seems reasonable that a similar pathway can be applied to children undergoing radical nephrectomy for suspected malignancy. CONCLUSIONS: For children with renal tumors who underwent radical nephrectomy, early re-feeding was associated with a shorter time to discharge. Use of bowel preparation and nasogastric tube did not appear to shorten time to discharge. These data are important for developing postoperative care pathways for these patients.

The role of interleukin-converting enzyme in Fas-mediated apoptosis in HIV-1 infection.

Apoptosis of CD4+ lymphocytes is partially responsible for the depletion of these cells in HIV-infected individuals. CD4+ lymphocytes from HIV-1-infected patients express higher membrane levels of the Fas receptor, and are particularly susceptible to apoptosis after Fas triggering. IL-1beta- converting enzyme (ICE) is a key enzyme of the apoptotic machinery involved in Fas-mediated apoptosis of normal lymphocytes. The role of ICE in mediating the increased susceptibility of CD4+ lymphocytes from HIV-1-infected patients to apoptosis has not been examined. In this study, we found that ICE mRNA was present in T cells from both HIV-1-infected patients and controls. Active ICE proteins, p10 and p20, were demonstrated by immunoblot in lymphocytes from HIV-1-infected patients and in normal lymphocytes after treatment with Fas agonist, CH11 mAb. Cocultivation of lymphocytes from HIV-1-infected persons with Fas antagonist, antibody ZB4, resulted in decreased expression of ICE protein in lymphocytes from HIV-infected patients, and decreased apoptosis. Similar effects were obtained when cells were treated with synthetic ICE inhibitors, which blocked apoptosis in response to Fas triggering. When CD4+ and CD8+ cells were sorted by flow cytometry and analyzed by reverse transcriptase PCR, ICE mRNA was present in both CD8+ and CD4+ cells. However, flow cytometric analysis of lymphocytes with intracellular staining for ICE demonstrated ICE protein in the CD4+ but not the CD8+ cell fraction derived from blood of HIV-1-infected patients. These data suggest that activation of ICE occurs in vivo in CD4+ lymphocytes from HIV-1-infected individuals, and may account for the increased susceptibility of CD4+ cells to apoptosis.