Erudition from primary posterior sagittal anorectoplasty for anorectal malformations over two decades.

PURPOSE: Perioperative and early post-operative outcomes of Primary Posterior sagittal anorectoplasty (P-PSARP) were evaluated. METHOD: Retrospective analysis of cases who underwent P-PSARP from 2004 to 2019 was done. Perioperative care, management, complications, voluntary bowel movement, soiling and constipation, graded by Krickenbeck criteria were studied. RESULTS: One hundred fifty six patients (134 girls) underwent P-PSARP at median age of 5 months (3 months to 14 years) in girls and 5(1-10) days in 21 boys. One male cloaca was operated at 5 months age. Of 20 boys, 5, 8, 4, 3 had rectobulbar urethral fistula, rectoprostatic urethral fistula, bladder neck fistula and male cloaca. Girls had vestibular fistula, rectovaginal fistula, vulval anus, anterior ectopic anus, pouch perineal fistulae and posterior anus with H type fistula in 114, 7, 6, 5, 1 and 1. Complications included wound infection, excoriation, oedema, mucosal prolapse, anal stricture, anal retraction and mortality in 6, 4, 5, 4, 4, 1 and 1, respectively. 35/155(12 neonates) required postoperative dilatations for 5(1-12) months. At follow-up, 96/114(84.2%) had voluntary bowel movements. 46/155 (29.7%) and 9/155 had constipation and soiling. 32:14:0 had grade 1:2:3 constipation, treated with diet (grade 1) and laxatives (grade 2) respectively. 4:3:2 had grade 1:2:3 soiling for initial 3 months, treated with bowel management programme. CONCLUSION: P-PSARP is feasible, subject to proper case selection and good perioperative care, once learning curve is achieved.

The cloacal anomalies: Anatomical insights through a complex spectrum.

PURPOSE: To study the detailed anatomy of cloacal anomalies using the multiplanar capabilities of MRI in addition to other available modalities. PATIENTS AND METHODS: The study was conducted on 27 cases of cloaca that were managed at our unit during the last 12 years. Preoperative assessment included conventional investigations and MRI studies. Endoscopic and operative findings were important for confirmation and completion of the whole picture. For better perception of the degree of deviation from the norm, we included another control group of girls who underwent pelvic MRI studies for causes other than anorectal anomalies. RESULTS: For practical reasons, we dissociated the cloacal complex into its two basic components to be described separately namely the anorectal anomaly and persistent urogenital sinus. The anorectal anomaly may be best described regarding two parameters: the position of the rectum and type of rectal communication with the urogenital tract. Persistent urogenital sinus anomalies were stratified based on referring the level of the urogenital confluence to the back of pubic symphysis. Three types could be identified (low, intermediate, and high) in which the mean length of the common channel was 6.4, 14.6, and 24 mm respectively with a significant statistical difference (Kruskal-Wallis chi-squared test). CONCLUSION: Applying MRI in the preoperative assessment of cases of cloaca has the advantage of demonstrating the real anatomy of the anomaly in multiple planes. This can improve our perception of the degree of deviation from the norm in these cases, which may be important during planning for surgical reconstruction. LEVEL OF EVIDENCE: This is a case control study (level III evidence).

Associated anomalies in cases with anorectal anomalies.

Anorectal anomalies (ARA) are common congenital anomalies. The etiology of ARA is unclear and its pathogenesis is controversial. Cases with ARA often have other non-ARA-associated congenital anomalies. The purpose of this study was to assess the prevalence and the types of these associated anomalies in a defined population. The associated anomalies in cases with ARA were collected in all live births, stillbirths, and terminations of pregnancy during 29 years in 387,067 consecutive births in the area covered by our population-based registry of congenital malformations. Of the 202 cases with ARA, representing a prevalence of 5.21 per 10,000, 100 (49.5%) had associated anomalies. There were 7 (3.3%) cases with chromosomal abnormalities, and 31 (15.3%) nonchromosomal recognized dysmorphic conditions, including 17 cases with Vertebral defects, Anal atresia, Cardiac septal defects, esophageal atresia or TracheoEsophageal fistula, Renal anomalies and radial Limb defects association. Sixty two (30.7%) of the cases had nonsyndromic multiple congenital anomalies (MCA). Anomalies in the urogenital, the musculoskeletal, the cardiovascular, the digestive, and the central nervous systems were the most common other anomalies in the cases with MCA. The anomalies associated with ARA could be classified into a recognizable malformation syndrome or pattern in 38 out of the 100 cases (38%) with associated anomalies. This study included special strengths: each affected child was examined by a geneticist, all elective terminations were ascertained, and the surveillance for anomalies was continued until 2 years of age. In conclusion, the overall prevalence of associated anomalies, which was close to one in two cases, emphasizes the need for a routine screening for other anomalies in cases with ARA.

Varied facets of rectal atresia and rectal stenosis.

Rectal atresia (RA) and rectal stenosis (RS) are rare anomalies with varied treatment options. A thorough literature review was done on reported cases/series of RA and RS. Based on evidence from cases managed over last 15 years, new insights into embryology were hypothesized. A comprehensive review was compiled with updated knowledge on diagnosis and management. RA is classified into five types I: II: III: IV: V as RS: RA with septal defect: RA with a fibrous cord between two atretic ends: RA with a gap: Multiple RA and/or RS. Current definitive surgical repair of these anomalies preserves the anal canal, dentate line, and sphincter complex. Most neonates with RA undergo sigmoid colostomy except few with RS who can rarely decompress adequately. Membranous RS and septal RA may seldom respond to dilatation or be amenable to transanal repair. Posterior sagittal anorectoplasty with an end-to-end/side repair is recommended for RA and most intramural RS. RS may be associated with a presacral mass and colonic/rectal motility disorders. The expected postoperative outcome is good if the normally developed anal sphincter complex is retained undamaged. Early recognition of the type of anomaly is necessary for appropriate management.

The Currarino triad: What pediatric surgeons need to know.

PURPOSE: We report our experience in managing a group of patients with Currarino syndrome, highlighting diagnostic challenges, surgical techniques, in addition to a review of current neurosurgical options. PATIENTS AND METHODS: The study included patients with Currarino syndrome who presented to our pediatric surgery department during the period 2010 through 2016. The 'sacral scimitar' in plain X-ray provided the clue for the diagnosis; while MRI examination was essential to define the nature of the presacral mass and associated spinal anomalies. RESULTS: The study included 17 patients (13 girls and 4 boys). Their age at presentation ranged from 7months to 10years. We used posterior sagittal approach to correct anorectal anomalies, and excise presacral cysts that were subjected to histopathological examination. Two cases presented with a pelvic abscess (infected presacral dermoid cyst), which were initially drained followed by excision. The presacral mass consisted of either lipomyelocele (6), lipomyelomeningocele (3), or a developmental (dermoid) cyst (8). Tethering of the spinal cord was a common association (70%) CONCLUSION: Apart from diagnostic challenges, the management of Currarino syndrome is similar to the usual management of ARM regarding the surgical approach and probably the prognosis that mainly depends on degree of associated sacral dysplasia. LEVEL OF EVIDENCE: This is a case series with no comparison group (level IV).

One or Two Stages Procedure for Repair of Rectovestibular Fistula: Which is Safer? (A Single Institution Experience).

BACKGROUND: Rectovestibular fistula (RVF) is the most common type of anorectal malformations in females. The need for a diverting colostomy during correction of defect has ignited a heated debate. In this study, we reviewed the girls with RVF that had been treated by either one or two stage procedure in the past 10 years in our institution to define whether one stage or two stage procedures is safer and more beneficial for the patients. MATERIALS AND METHODS: Seventy girls with RVF that had been operated from January 2005 to January 2015 were studied retrospectively. Data were obtained from medical hospital records. The cases were divided into two groups. Group A (46 patients): were operated by two stages technique (simultaneous sigmoid colostomy and anterior sagittal anorectoplasty [ASARP]). Group B (24 patients): were operated by one stage (ASARP without covering colostomy). The short-term outcome as regard wound infection, wound dehiscence, anal stenosis, anal retraction, recurrence of fistula as well as complications of colostomy was reported. The long-term outcome as regard soiling, constipation and voluntary bowel movement was evaluated. RESULTS: The age of patients at the time of surgery ranged from 3 months to 2 years (mean; 9.5 months). In Group A, seven patients (15.2%) developed wound infection, two patients developed wound disruption. One patient developed anterior anal retraction and required redo-operation, anal stenosis was noticed in five (10.9%) patients. Complications from colostomy had occurred in nine patients (19.5%). In Group B, wound infection occurred in ten patients (41.7%). Seven patients (29.2%) developed wound disruption. Anal stenosis occurred in eight patients (33.3%). Five patients required redo-operation because of anal retraction in three patients and recurrence of fistula in the other two patients. Constipation recorded in 15 patients (32.6%) of Group A and in ten patients (41.3%) of Group B. Soiling was reported in six girls (13.04%) of Group A and five girls (20.8%) of Group B. CONCLUSION: The avoidance of colostomy is not outweighed achieving sound operation and continent child. Two stages correction of RVF is safer and more beneficial than one stage procedure, especially in our locality and for our paediatric surgeons during their learning curve.

CT-guided transgluteal biopsy for systematic sampling of the prostate in patients without rectal access: a 13-year single-center experience.

OBJECTIVES: The purpose of our study was to examine the safety and diagnostic utility of transgluteal CT-guided prostate biopsy for prostate sampling in patients without rectal access. METHODS: Seventy-three biopsies were performed in 65 patients over a 13-year period (2002-2015). Mean prostate-specific antigen (PSA) at biopsy was 7.8 ng/mL (range 0.37-31.5). Electronic medical records were reviewed for procedural details and complications. Mean PSA and number of cores in malignant and benign cohorts were compared with Student's t test. RESULTS: Technical success rate was 97.3% (71/73; mean cores 8, range 3-28). Of these, 43.6% (31/71) yielded malignancy (mean Gleason score 7, range 6-10) and 56.3% (40/71) yielded benign tissue. The only complication was an asymptomatic periprostatic hematoma (1/73; 1.4%). In 14 patients who underwent surgery, Gleason scores were concordant in 71.4% (10/14) and discordant in 28.6% (4/14; Gleason 6 on biopsy but Gleason 7 on surgical specimen). Mean effective radiation dose was 18.5 mSv (median 15.0, range 4.4-86.2). There was no significant difference in either mean PSA (p = 0.06) or number of core specimens (p = 0.33) between malignant and benign cohorts. CONCLUSION: CT-guided transgluteal prostate biopsy is highly safe and reliable for the detection of prostate cancer in men without rectal access. KEY POINTS: • Prostate cancer detection in men without rectal access is challenging. • CT-guided transgluteal prostate biopsy is safe and effective in these patients. • CT-guided biopsy may be particularly effective in diagnosing high-grade prostate cancer. • Unilateral CT-guided biopsy may be effective in patients with focal lesions. • The radiation exposure with this technique is acceptable.

Low-type anorectal malformations in the male: Extent of deviation from the norm.

PURPOSE: To define the degree of deviation from the norm among boys with minor forms of anorectal malformations (ARM). PATIENTS AND METHODS: Between March 2015 and January 2016, we studied the preoperative MRI of nine boys with low-type ARM. For comparison, we included another nine boys (control group) who underwent MRI pelvis for causes other than ARM (e.g. impalpable testes). RESULTS: In boys with low-type ARM, the rectum descends forwards to touch the back of the prostate (as in the norm), but then goes downwards (with little or no backward deflection) keeping intimately attached to the bulb of the corpus spongiosum and displacing it downwards and forwards. The striated sphincter muscles do not follow the anterior displacement of the bowel termination, but remain orthotopically compacted at the normal predestined site of the anal canal. CONCLUSION: Among boys with low ARM, the minor abnormalities at the external anal orifice are associated with deeper anatomical aberrations in the form of anterior misplacement of the anorectum. These findings may help in understanding the disturbed act of defecation among these patients, and provide guidance to the best way of surgical correction.

Early reported rectal sensation predicts continence in anorectal anomalies.

BACKGROUND: Straining at stool is an automatic reflex in babies and implies the presence of rectal sensation. We hypothesised that early reported rectal sensation would predict future continence in children with anorectal anomalies. AIM OF THE STUDY: The aim of this study is to determine if early straining at stool was a useful predictor of future continence in infants born with high anorectal malformations. METHODS: A retrospective case note review of prospectively collected clinical information was performed with institutional review board approval. All patients with intermediate/high anorectal malformation operated on by a single surgeon from 1984 to 2010 were included. After stoma closure, parents were asked: The responses were noted within the first year of stoma closure and then all patients were followed up until they were at least 3 ½years old and continence could be assessed using the Krickenbeck outcome classification. Data were compared using Fisher's exact test and sensitivity, specificity and positive predictive value (PPV) were calculated. MAIN RESULTS: Forty-eight patients were included in the study. Sixteen (33%) were female (12 cloacal malformation, 3 rectovaginal fistula, 1 rectal atresia) and 32 (66%) were male (6 rectovesical fistulae, 22 rectourethral fistulae, 4 no fistula). Median follow-up was 9.7years (range 3.5-17.9). Twenty-one children were noted by their parents to exhibit early straining at stool after stoma closure. Twenty of them achieved long term continence. The sensitivity of early straining as a predictor for long term continence was 77%, specificity 95% and positive predictive value 95%. CONCLUSION: The presence of early rectal sensation reported by parents is a good predictor of long term continence. This allows more informed discussion with families in the early years of life.

Effects of reconstructive surgery on bladder function in patients with anorectal malformations.

PURPOSE: Bladder dysfunction is common in patients with anorectal malformations and can be congenital or acquired as a consequence of surgery. We investigated the effects of surgical correction of anorectal malformations on bladder function. MATERIALS AND METHODS: The charts of all 341 patients who underwent surgery at our center between 1990 and 2010 were retrospectively analyzed for preoperative and postoperative videourodynamics. A total of 52 patients were eligible for study inclusion. Each assessment was scored according to International Children's Continence Society standards. RESULTS: Urodynamic study indicated normal bladder function preoperatively in 36 patients (69%) and postoperatively in 37 (71%). Median bladder emptying efficiency and relative bladder capacity changed significantly after posterior sagittal anorectoplasty. Bladder function according to International Children's Continence Society standards did not change postoperatively in 43 patients (83%). In 1 of 4 patients with deterioration of bladder function the deterioration could be attributed solely to surgery. Clinical outcome was available in 38 patients and showed complete urinary continence with spontaneous voiding in 24 (63%). Seven of 25 patients (28%) with preoperative videourodynamics indicating normal bladder function demonstrated dysfunctional voiding at latest followup. CONCLUSIONS: Urodynamic and clinical outcomes after anorectal malformation repair are good, with 63% of patients being continent of urine. Urodynamic studies are of limited value in preoperative settings in these patients. Current techniques of reconstructive surgery for anorectal malformations seem to preserve bladder function in the majority of patients.

Ectopic anus with barrel gun perineum rare type of anorectal anomaly.

Perineal ectopic anus in female infants is not a very uncommon congenital anorectal anomaly with opening into the low vaginal or vulvar region. However, ectopic anus with barrel gun perineum is a less common variety. Patients generally present with frequent history of constipation, but may seek medical help for just aesthetic reasons. We present here one such case of an asymptomatic seven years old female with the rare form of anterior ectopic anus with barrel gun perineum without any fistulous communications with an innovative method of demonstration of the anomaly by using ultrasound gel as rectal contrast in MRI pelvis.