Clinical Differentiation between a Normal Anus, Anterior Anus, Congenital Anal Stenosis, and Perineal Fistula: Definitions and Consequences—The ARM-Net Consortium Consensus

In the past, an anteriorly located anus was often misdiagnosed and treated as an anorectal malformation (ARM) with a perineal fistula (PF). The paper aims to define the criteria for a normal anus, an anterior anus (AA) as an anatomic variant, and milder types of ARM such as congenital anal stenosis (CAS) and PF. An extensive literature search was performed by a working group of the ARM-Net Consortium concerning the subject “Normal Anus, AA, and mild ARM”. A consensus on definitions, clinical characteristics, diagnostic management, and treatment modalities was established, and a diagnostic algorithm was proposed. The algorithm enables pediatricians, midwives, gynecologists, and surgeons to make a timely correct diagnosis of any abnormally looking anus and initiate further management if needed. Thus, the routine physical inspection of a newborn should include the inspection of the anus and define its position, relation to the external sphincter, and caliber. A correct diagnosis and use of the presented terminology will avoid misclassifications and allow the initiation of correct management. This will provide a reliable comparison of different therapeutic management and outcomes of these patient cohorts in the future

Rectal atresia and rectal stenosis:the ARM-Net Consortium experience

Purpose: To assess the number, characteristics, and functional short-, and midterm outcomes of patients with rectal atresia (RA) and stenosis (RS) in the ARM-Net registry. Methods: Patients with RA/RS were retrieved from the ARM-Net registry. Patient characteristics, associated anomalies, surgical approach, and functional bowel outcomes at 1 and 5-year follow-up were assessed. Results: The ARM-Net registry included 2619 patients, of whom 36 (1.3%) had RA/RS. Median age at follow-up was 7.0 years (IQR 2.3–9.0). Twenty-three patients (63.9%, RA n = 13, RS n = 10) had additional anomalies. PSARP was the most performed reconstructive surgery for both RA (n = 9) and RS (n = 6) patients. At 1-year follow-up, 11/24 patients with known data (45.8%, RA n = 5, RS n = 6) were constipated, of whom 9 required stool softeners and/or laxatives. At 5-year follow-up, 8/9 patients with known data (88.9%, RA n = 4, RS n = 4) were constipated, all requiring laxatives and/or enema. Conclusion: RA and RS are rare types of ARM, representing 1.3% of patients in the ARM-Net registry. Additional anomalies were present in majority of patients. Different surgical approaches were performed as reconstructive treatment, with constipation occurring in 46% and 89% of the patients at 1 and 5-year follow-up. However, accurate evaluation of long-term functional outcomes remains challenging.</p

Appendiceal Intussusception: A Diagnostic Challenge

Introduction Appendiceal intussusception is a rare condition in children characterized by an invagination of the appendix into the cecum to various degrees. The treatment is appendectomy; however since symptoms are not specific, clinical diagnosis is challenging and frequently only intraoperative. We present a series of five patients with appendiceal intussusception and discuss features that may direct the pediatric surgeon to achieve early recognition and provide optimal treatment. Materials and Methods The database of a tertiary medical center was retrospectively reviewed for all patients treated for appendiceal intussusception during the period from January 1995 to January 2016. Data collected by chart review included demographics, clinical characteristics, imaging studies, surgical technique, and outcome. The findings were analyzed by descriptive statistics. Results This series included five patients (two females and three males) with ages ranging between 27 and 42 months (mean: 35.2). Patients presented with intermittent abdominal pain (IAP, all five patients), alternate vomiting (three of five patients), alternate diarrhea (two of five patients), fever (two of five patients), and rectal bleeding (one of five patients). The average length of symptoms was 22.6 days. Eighteen diagnostic studies were performed, including abdominal ultrasound for all patients, barium enema for three patients with secondary ileocolic intussusception, and abdominal computed tomography (CT) for one patient. The average number of studies per patient was 3.6. In surgery, the appendiceal intussusception was found to be complete in four patients, whereas it was partial in the remaining patient. In all patients, appendectomy was performed with resection of a small rim of cecal wall due to marked congestion and edema in an attempt to decrease recurrence. Conclusion The mainstay of clinical presentation is intermittent abdominal pain while patients may be completely asymptomatic between attacks. Appendiceal intussusception may act as a leading point to ileocolic intussusception and is frequently concealed by it. The treatment is appendectomy. Both pediatric surgeons and radiologists should be aware of this occurrence to provide adequate management and avoid complications.</jats:p

Anorectal malformation with rectobladder neck fistula: A distinct and challenging malformation

© 2016 Elsevier Inc. Background Rectobladder neck fistula is the highest and most complex anorectal malformation in boys and the only one that requires an abdominal approach, open or laparoscopic, for repair. The aim of this study was to describe the unique characteristics of rectobladder neck fistulas that warrant special attention and to describe the associated anatomic variants in the genitourinary tract. Methods The database of a tertiary medical center was retrospectively reviewed for all patients treated for rectobladder neck fistula, by our team in 1980–2011. Data on surgical history, associated and functional defects, treatment and outcome were collected by chart review. Results The study group included 111 patients. The most common anatomic urologic defect was a single kidney in 37 patients (33.3%) and the most common functional urologic defect was vesicoureteral reflux in 40 patients (36%), including 11/37 patients with a single kidney (29.7%). Of the 40 patients who underwent cystoscopy, 16 (40%) had a higher than normal location of the verumontanum. Follow-up ranged from 2 to 290 months (median 59). Urinary continence was achieved in 40 of the 61 patients (65.5%) for whom data were available, and fecal continence was achieved in 9 of the 69 patients (13%) for whom data were available. A sacral ratio of 0.4 or less was associated with lower rates of urinary control (23%) and fecal control (0%), relative to higher ratios. Twenty stomas (18%) were found to be located too distally, limiting the availability of the bowel for a pull through. Conclusions Rectobladder neck fistula carries a poor prognosis for bowel control and is associated with a high rate of urinary malformations that require long-term care. Pediatric surgeons need to be aware of these complications in order to provide proper treatment and parental counseling. Intra-vesical verumontanum is found in a surprisingly high percentage of patients. The combination of a single kidney with vesicoureteral reflux is common and should be closely followed to avoid renal deterioration. Special attention should be given to colostomy construction to avoid complications and unnecessary procedures. A sacral ratio of 0.4 or less is an indicator of poor fecal and urinary control

Dual versus Triple Antibiotics Regimen in Children with Perforated Acute Appendicitis

Introduction Acute appendicitis is the most common emergency condition in pediatric surgery. Historically, a triple-antibiotic therapy consisted of ampicillin, gentamicin, and clindamycin has been used postoperatively for perforated appendicitis. According to recently published trials, dual therapy consists of ceftriaxone and metronidazole only, offers a more efficient and cost-effective antibiotic management compared with triple therapy. Based on these results, our department applied dual antibiotic therapy for children with perforated appendicitis that underwent appendectomy from 2009 and forth. Aim The aim of our study was to compare postoperative outcomes between patients treated with triple therapy (ampicillin, gentamicin, and metronidazole) (group A) versus dual therapy (ceftriaxone and metronidazole) (group B). Methods Clinical and laboratory data were retrospectively collected by review of the medical records for all children who underwent appendectomy for the perforated appendix at the Schneider Children's Medical Center of Israel, a tertiary pediatric care center between 2007 and 2011. Children with perforated appendicitis received antibiotic therapy in accordance with the hospital's guidelines that were valid at the time the surgery took place. In the first period (years 2007–2009) (group A) a triad of ampicillin, gentamicin, and metronidazole and the second period (2009–2011) (group B) dual therapy consists of ceftriaxone and metronidazole. The two groups were compared for outcome and complications, such as wound infections, changing of antibiotic therapy, and length of stay. Results During the study period 1,203 patients underwent an appendectomy. Of these, 175 patients were diagnosed with perforated appendix and were treated with postoperative antibiotic's regimen. Group A and group B consisted of 89 and 86 patients, respectively. The two groups were not different significantly in terms of demographic data, length of stay, or readmission rates. However, more rates of wound infection and changing of antibiotic therapy were seen in group B, although not statistically significant (p = 0.064). Conclusion Dual antibiotic therapy for perforated appendicitis is a cost-effective and efficient mode of therapy compared with triple-antibiotic's regimen. However, prospective studies are required to determine whether this policy is associated with higher rates of wound infections and change in antibiotic therapy.</jats:p