Posterior Sagittal Anorectovaginourethroplasty Research Articles

Cloacal Malformation with Associated Urethral Atresia

Introduction Cloacal malformations comprise a heterogeneous group of anomalies that are considered the most complex anorectal malformations (ARMs) in females. Precise evaluation to identify the unique anatomy prior to reconstruction with collaboration between colorectal surgeons, urologists, and gynecologists is vital. Here, we present a rare anatomical variation in a patient with a cloacal malformation which affected operative and postoperative management. Case description A 6-year-old female with cloaca who underwent colostomy, vaginostomy, and vesicostomy as a newborn presented for reconstruction. Her VACTERL workup was negative except for an atretic right kidney. Her ARM index included the cloaca, a normal spine, and sacrum with a lateral sacral ratio of 0.7, predicting good potential for bowel continence. Cystoscopy through the vesicostomy showed a small bladder with normal ureteral orifices, and a closed bladder neck, with no identifiable urethra. A cloacagram showed an atretic common channel, a single small vagina, and a rectum below the pubococcygeal line. The patient underwent a posterior sagittal anorectovaginourethroplasty, vaginal patch using rectum, rectoplasty, and perineal body reconstruction. The urethra was not amenable to reconstruction, so the vesicostomy was preserved and a future Mitrofanoff was planned. Conclusion Urethral atresia is a rare and challenging finding in cloaca patients, and a vesicostomy is needed to drain urine in the newborn period. Preoperative examination under anesthesia, cystoscopy, vaginoscopy, and cloacagram are crucial to identify the precise anatomy and to plan accordingly.

Experience with the management of anorectal malformations in Ibadan, Nigeria.

Introductionanorectal malformation is a multi-systemic birth defect of the distal gastrointestinal tract, the management of which is challenging to the surgeons, the patients and the parents. The presence of associated congenital malformations may worsen the outcome with consequent psychosocial effects on the patients and the parents. The characteristics of anorectal malformations with the challenges associated with their management and the outcomes are therefore presented here.Methodsall patients managed for anorectal malformations from January 2003 to December 2017 were studied. Patients´ demography, clinical presentations, types of malformations, associated anomalies, procedures performed, post-operative complications and management outcome were obtained and analysed.Resultseighty-eight children with anorectal malformations comprising 61 (69.3%) boys and 27 (30.7%) girls were studied with 76 (86.3%) patients presenting within the first year of life. Low anorectal malformation was observed in 14 (15.9%) patients, 71 (80.7%) patients had intermediate or high malformations and cloacal malformation was present in 3 (3.4%) patients. Associated congenital malformations were observed in 18 (20.5%) patients with 10 (55.6%) patients associated with intermediate or high malformations and urogenital system was the most common system whose anomalies were associated with anorectal malformations in 12 (13.6%) patients. Anoplasty was performed on 14 (15.9%) patients, posterior sagittal anorectoplasty was performed on 67 (76.1%) patients, abdominosacroperineal pull through on 4 (4.6%) patients and posterior sagittal anorectovaginourethroplasty on 3 (3.4%) patients. Six (6.8%) neonates died.Conclusionimmediate post-operative outcome was good; however, good functional outcome can only be assessed in an atmosphere of good follow-up which is still a problem in our environment.

An overview of opioid usage and regional anesthesia for patients undergoing repair of anorectal malformation.

The recent opioid crisis in the USA compelled us to evaluate our practice of opioid use for postoperative pain management and the influence of regional anesthesia on opioid requirement in patients undergoing repair of anorectal malformations. A retrospective chart review was performed evaluating patients who underwent posterior sagittal anorectoplasty (PSARP) and posterior sagittal anorecto-vagino-urethroplasty (PSARVUP), with or without laparotomy, between January 2016 and March 2020. Morphine milligram equivalents per kilogram (MME/kg) were calculated. IRB approval was obtained for this study. A total of 105 surgical patients had either a PSARP (74 without laparotomy, 10 with laparotomy) or PSARVUP (13 without laparotomy, 8 with laparotomy). Regional anesthesia included epidurals, transversus abdominis plane block, caudal block or paravertebral catheters. Of the PSARP patients, 4 without laparotomy and 7 with laparotomy received regional anesthesia. For PSARVUP with laparotomy, 4/8 received regional. 44% of PSARP patients without laparotomy did not require opioids postoperatively. The MME/kg required exponentially increased for patients over the age of 5 who underwent PSARP. Regional anesthesia is a useful modality for pain control for PSARP/PSARVUP with laparotomy, decreasing the opioid usage, but it is unnecessary for the already low opioid requirements, in patients younger than 5years of age, without laparotomy.

Correction of persistent cloaca in children: experience and analysis of treatment results

Introduction. Cloaca is one of the most difficult to be corrected varieties of the pathology of the anorectal zone, occurring with a frequency of 1 case per 40 000–25 000 live births. Purpose: аnalysis of diagnostic methods and surgical correction, treatment outcomes of children with cloacal malformations. Materials and methods . The article analyzed 16 cases of surgical treatment of cloacal anomalies based on the Irkutsk Municipal Pediatric Clinical Hospital in Irkutsk for the period from 2008 to the present. Gender composition: girls – 14 (87,5%), genotype (46XX – 13; 46XX, del (6) – 1), boys – 2 (12,5%). Results. The most common cloacal anomaly was a persistent cloaca – 11 (68,8%), the extrophy of the cloaca was 4 – 25 (25%) and the posterior cloaca – 1 (6,25%) was much less common. Aggravated obstetric history was found in reliable data on the presence of teratogenic factors is associated with a lack of information on obstetric history. In 93,8% of cases, cloacal anomalies were combined with other congenital malformations. In 100% (11) of cases the posterior-sagittal anorectovaginourethroplasty was performed in its various modifications, including LAPassisted – 3 (27,3%). When evaluating long-term results, it was revealed that 3 (27,3%) patients developed chronic recurrent pyelonephritis, 2 (18,2%) had urinary incontinence and first-degree anal incontinence, 1 (9,1%) had sphincter-detrusor insufficiency and anal incontinence of 3 degrees. Conclusion. According to the authors, the issues of surgical strategy for the correction of cloacal anomalies remain unresolved. The morphology of blemish, the spectrum of concomitant pathology are important in the choice. The results of treatment in favorable cases should be considered quite satisfactory. However, in severe forms, poor functional results occur, which often requires repeated reconstructive interventions and long-term rehabilitation of patients.

Early vaginal replacement in cloacal malformation.

We assessed the surgical outcome of cloacal malformation (CM) with emphasis on need and timing of vaginal replacement. An ambispective study of CM was carried out including prospective cases from April 2014 to December 2017 and retrospective cases that came for routine follow-up. Early vaginal replacement was defined as that done at time of bowel pull through. Surgical procedures and associated complications were noted. The current state of urinary continence, faecal continence and renal functions was assessed. 18 patients with CM were studied with median age at presentation of 5days (1day-4years). 18;3;2 babies underwent colostomy; vaginostomy; vesicostomy. All patients underwent posterior sagittal anorectovaginourethroplasty (PSARVUP)/ Pull through at a median age of 13 (4-46) months. Ten patients had long common channel length (> 3cm). Six patients underwent early vaginal replacement at a median age of 14 (7-25) months with ileum; sigmoid colon; vaginal switch; hemirectum in 2;2;1;1. Three with long common channel who underwent only PSARVUP had inadequate introitus at puberty. Complications included anal mucosal prolapse, urethrovaginal fistula, perineal wound dehiscence, pyometrocolpos, bladder injury and pelvic abscess. Persistentvesicoureteric reflux remained in 8. 5;2 patients had urinary; faecal incontinence. 2 patients of uterus didelphys are having menorrhagia. One patient succumbed to sepsis at 7months age. Renal functions remained normal in 16. One patient is undergoing dialysis. Early vaginal replacement in CM is feasible. Patients with inadequate introitus may suffer from menorrhagia. A regular follow-up is mandatory.

Gynecologic anatomic abnormalities following anorectal malformations repair.

Patients may present with gynecologic concerns after previous posterior sagittal anorectoplasty (PSARP) for repair of an anorectal malformation (ARM). Common findings include an inadequate or shortened perineal body, as well as introital stenosis, retained vaginal septum, and remnant rectovestibular fistula. An inadequate or shortened perineal body may impact fecal continence, sexual function and recommendations regarding obstetrical mode of delivery. We describe our experience with female patients referred to our center for evaluation of their previously repaired ARM, with a specific focus on perineal body anatomy and concomitant gynecologic abnormalities. We outline our collaborative evaluation process and findings as well as subsequent repair and outcomes. A single site retrospective chart review from May 2014 to May 2016 was performed. Female patients with a history of prior ARM repair who required subsequent reoperative surgical repair with perineoplasty were included. The decision for reoperation was made collaboratively after a multidisciplinary evaluation by colorectal surgery, urology, and gynecology which included examination under anesthesia (EUA) with cystoscopy, vaginoscopy, rectal examination, and electrical stimulation of anal sphincters. The type of original malformation, indication for reoperative perineoplasty, findings leading to additional procedures performed at time of perineoplasty, postoperative complications, and the length of follow up were recorded. During the study period 28 patients were referred for evaluation after primary ARM repair elsewhere and 15 patients (60%) met inclusion criteria. Thirteen patients (86.6%) originally had a rectovestibular fistula with prior PSARP and 2 patients (13.4%) originally had a cloacal malformation with prior posterior sagittal anorectovaginourethroplasty. The mean age at the time of the subsequent perineoplasty was 4.6years (0.5-12). Patients had an inadequate perineal body requiring reoperative perineoplasty due to: anterior mislocation of the anus (n=11, 73.3%), prior perineal wound dehiscence with perineal body breakdown (n=2, 13.4%), acquired rectovaginal fistula (n=1, 6.6%), and posterior mislocated introitus with invasion of the perineal body (n=1, 6.6%). During the preoperative evaluation, additional gynecologic abnormalities were identified that required concomitant surgical intervention including: introital stenosis (n=4, 26.6%), retained vaginal septum (n=3, 20%) and remnant recto vestibular fistula (n=2, 13.3%). Patients with a previously repaired ARM may present with gynecologic concerns that require subsequent surgical intervention. The most common finding was an inadequate perineal body, but other findings included introital stenosis, retained vaginal septum and remnant recto vestibular fistula. Multidisciplinary evaluation to assess and identify abnormalities and coordinate timing and surgical approach is crucial to assure optimal patient outcomes. Case series with no comparison group. IV.

Anorectal Malformations in a Tertiary Pediatric Surgery Center from Romania: 20 Years of Experience

Introduction: The main goal of surgery and multidisciplinary behavioral therapy in anorectal malformations (ARMs) is to achieve good anatomical and functional outcomes and a better quality of life. Material and Methods: A retrospective analysis of 146 patients with ARMs was conducted over a 20 years period in our pediatric surgery department. The clinical and demographical data, operative protocols, short and long term outcomes were subject to assessment. Results: The patient series included 85 boys and 61 girls aged between one day and two years, of which 82.19% were diagnosed in the first few days of life by means of clinical examination and current imaging tests. In 26.02% of cases we found associated congenital abnormalities. A total of 142 (97.25%) cases of all our series underwent surgical treatment; four patients died before surgery. For 131 children the definitive treatment involved posterior sagittal anorectoplasty (PSARP) respectively 11 posterior sagittal anorectovaginourethroplasty (PSARVUP). Long-term followup in 115 patients showed good clinical continence in 82 cases (71.30%) but more than half of the teenagers had body dissatisfaction and social difficulties. Conclusions: Through this review of 20 years of experience our findings are consistent with other reports regarding male predominance, low birth weight, clinical and imaging diagnosis. We found however a lower incidence of associated abnormalities. The most frequent surgical intervention was posterior sagittal anorectoplasty (PSARP) or posterior sagittal anorectovaginourethroplasty (PSARVUP), both followed by satisfactory functional outcome reporting nevertheless moderate social insertion. Our experience in ARMs proved the effectiveness of the consecrated surgical strategy; long-term assistance for patients and their families should be improved.

Postoperative complications after reconstructive surgery for cloacal malformations: a systematic review.

The repair of cloacal malformations is most often performed using a posterior sagittal anorecto-vagino-urethroplasty (PSARVUP) or total urogenital mobilization (TUM) with or without laparotomy. The aim of this study was to systematically review the frequency and type of postoperative complication seen after cloacal repair as reported in the literature. A systematic literature search was conducted according to preferred reporting items for systematic reviews and meta-analyses guidelines (PRISMA). Eight records were eligible for this study which were qualitatively analyzed according to the Rangel score. Overall complication rates reported in included studies ranged from 0 to 57 %. After meta-analysis of data, postoperative complications were seen in 99 of 327 patients (30 %). The most common reported complications were recurrent or persistent fistula (n = 29, 10 %) and rectal prolapse (n = 27, 10 %). In the PSARVUP group, the complication rate was 40 % and in the TUM group 30 % (p = 0.205). This systematic review shows that postoperative complications after cloacal repair are seen in 30 % of the patients. The complication rates after PSARVUP and TUM were not significantly different. Standardization in reporting of surgical complications would inform further development of surgical approaches. Other techniques aiming to lower postoperative complication rates may also deserve consideration.

Urogenital function after cloacal reconstruction, two techniques evaluated

ObjectiveCurrent surgical techniques for cloacal reconstruction are posterior sagittal anorecto vagino urethroplasty (PSARVUP) and posterior sagittal anorectoplasty (PSARP) with total urogenital mobilization (TUM). The aim of this study was to explore the results of reconstructive cloaca surgery in the Netherlands and evaluate urogenital function after PSARVUP and TUM. Patients and methodsMedical records from five pediatric surgical departments in the Netherlands were studied for patients with cloacal malformations treated between 1985 and 2009. Forty-two patients were eligible, and patients with short common channels were categorized into PSARVUP and TUM groups. Groups were compared using Fisher's exact test. ResultsMedian age at time of surgery was 9 months (range 1–121). In 24 patients (57%) a PSARVUP was done, in 18 patients (43%) TUM. Median follow-up was 142 months (range 15–289). At follow-up spontaneous voiding was seen in 29 patients (69%). Clean intermittent catheterization (CIC) was needed in 14 patients (33%); a urinary diversion was created in 10 patients (24%). In total 32 patients (76%) were dry with no involuntary loss of urine per urethra. Recurrent urinary tract infections were seen in 23 patients (55%). When comparing PSARVUP and TUM groups in our series of patients with short common channels, there were no differences in urological outcome. Normal menstruation was present in 11 of the 20 patients who reached puberty (55%). ConclusionUrogenital functional outcome after reconstructive surgery for cloacal malformations was similar in PSARVUP and TUM groups in patients with short common channels. A thorough urological follow-up is needed to establish the long-term bladder function and urinary incontinence results to prevent long-term risks of recurrent UTI. Albeit without differences between PSARVUP and TUM groups, 45% of the patients present with abnormal or absent menstruations. Gynecological follow-up is mandatory in all patients with cloacal malformations 6 months after the first sign of puberty.

Surgical reconstruction of cloacal malformation: A case report

It is a surgical challenge to repair a cloacal malformation. We are reporting a case of cloacal malformation in which we performed a reconstructive surgery. We performed a reconstructive surgery in a 5 yr old patient who had got a colostomy done in the neonatal period. The surgical management included purely a posterior sagittal anorectovaginourethroplasty without an abdominal approach.

Clinical experience with persistent cloaca.

PurposePersistent cloaca is one of the most severe types of anorectal malformation. Appropriate initial drainage is difficult due to their various malformations and hydrocolpos or dilated urinary bladder. Corrective surgery also differs among individual patients. We describe our experiences with the surgical management of children with persistent cloaca.MethodsWe retrospectively reviewed 16 children diagnosed with persistent cloaca at Asan Medical Center.ResultsSixteen patients were managed in their neonatal period. Twelve patients had enlarged bladder or vagina at birth. Three patients, who did not undergo cystostomy or vaginostomy at first operation, had earlier complications after surgery or required drainage tube insertion. One patient who did not undergo hydrocolpos drainage died of sepsis and complications. Nine patients underwent corrective surgery; posterior sagittal anorectovaginourethroplasty using the Pena method. Three patients required additional operations due to complications after surgery.ConclusionPatients found to have anatomical malformations before colostomy, as well as hydrocolpos and bladder enlargement, require a vaginostomy with or without a cystostomy to reduce complications. Follow-up is required in patients with hydrocolpos and bladder enlargement to determine whether vaginal drainage improves dilated bladder. Continuous long-term follow-up examination is required to determine the long-term results of corrective surgery.

Management of anorectal malformation: Changing trend over two decades in Zaria, Nigeria

Anorectal malformation is a common congenital defect and its management has evolved over the years. This is a review of the trend in the management of this condition in a major paediatric surgical centre in Nigeria over two decades. A retrospective analysis of 295 patients with anorectal malformations managed from January 1988 to December 2007 was carried out. There were 188 boys and 107 girls aged 1 day-9 years (median 8 years) at presentation. There were 73 (54.5%) and 106 (65.8%) emergency operations in groups A and B, respectively. There were 61 (45.5%) and 55 (34.2%) elective operations in groups A and B, respectively. Regarding treatment, in group A, patients requiring colostomy had transverse loop colostomy, while in group B, sigmoid (usually divided) colostomy was preferred. The definitive surgery done during the two periods were: group A: cutback anoplasty 29 (47.5%), anal transplant 5 (8.2%), sacroabdominoperineal pull through (Stephen's operation) 6 (9.5%) and others 21 (34.4%). In group B, posterior sagittal anorectoplasty (PSARP) 46 (83.7%), anal transplant 1 (1.8%), posterior sagittal anorectovaginourethroplasty (PSARVUP) 2 (3.6%) and anal dilatation 6 (10.9%) were done. Early colostomy-related complication rates were similar in the two groups (P > 0.05). The overall late complication rate was 65.5% in group A and 16.4% in group B (P < 0.05). The mortality was 25 (18.6%) in group A compared to 17 (10.6%) in group B (P < 0.05). There have been significant changes in the management of anorectal malformations in this centre in the last two decades, resulting in improved outcomes.

Rare association of female pseudohermaphroditism, phallic urethra, and posterior cloaca

We describe a child with the rare clinical entity of female pseudohermaphroditism, accessory phallic urethra, and posterior cloaca who was successfully treated with posterior sagittal anorectovaginourethroplasty. Masculinization was limited to the external genitalia, and no chromosomal, metabolic, or adrenal abnormalities were detected. Associated pathology included bilateral vesicoureteric reflux, a non functioning dysplastic kidney, and bicornuate uterus. The investigation and surgical management of this particularly challenging combination of anomalies is detailed.

Surgical treatment of cloacal anomalies.

From 1989-1998 14 patients were treated with cloacal anomalies: 5 typical cloacas (PC), 5 posterior cloacas, and 4 cloacal exstrophies (CE); 12 underwent surgery. Four typical cloacas were resolved with posterior sagittal anorectovagino-urethroplasty (PSARVUP), whereas in the 5th total urogenital mobilization (TUM) was used. Three PCs were managed with transanorectal TUM and 2 with anterior TUM without opening the anal canal and rectum (without a protective colostomy). Two CEs were treated with atypical procedures. Two patients with CE died without surgery and 2 died after surgery due to complex associated anomalies. During postoperative follow-up of 1-8 years, 5 children had voluntary bowel movements and no soiling while the others had soiling with or without enemas; 1 had stress incontinence; 3 were on clean intermittent catheterization due to neurogenic bladder and were dry. PSARVUP provides a satisfactory result if there is no sacral anomaly. TUM makes this operation easier to perform. In patients with a PC it is sometimes possible using TUM to separate the urinary from the genital tract and remove the accessory urethra without opening the anus and rectum.

Posterior sagittal approach for reconstruction of an unusual type of cloacal anomaly

Posterior sagittal anorectoplasty is a popular procedure for treatment of high anorectal malformations. A female infant underwent posterior sagittal anorectovaginourethroplasty for treatment of an unusual type of cloacal anomaly associated with three fistulas: a high and a low rectovaginal fistula, and a high urethrovaginal fistula. A satisfactory anatomic and functional result was obtained.

The surgical management of persistent cloaca: Results in 54 patients treated with a posterior sagittal approach

This report describes the author's personal experience in the surgical treatment of 54 patients. The approach is called posterior sagittal ano recto vagino urethroplasty (PSARVUP). Forty patients underwent a primary procedure, and 14 a secondary operation. The anatomic variations found were multiple, integrating a wide spectrum of defects. The posterior sagittal approach proved to be a good initial approach, and permitted complete repair of the defect in 47 patients. Seven patients required a laparotomy in addition. The length of the common channel varied from 0.5 to 7 cm. Common channels longer than 3 cm usually required some technical alternative to replace the vagina. In at least 34 cases, the vagina was reconstructed primarily without any additional technical manoeuvres. Different degrees of vaginal and uterine septation were found in 25 of 50 cases. Hydrocolpos was an associated defect in 14 of 49 patients. Sixty-eight percent of the patients had an important associated urological defect. Twenty-six patients were clinically evaluated without medical management, twenty-one of whom had voluntary bowel movements by the age of 3 years, but most of them had minor episodes of soiling. Nineteen patients had a normal sacrum, and five had urinary incontinence that was successfully managed by intermittent catheterization. Seven patients had a very abnormal sacrum, and five of them had urinary incontinence. Twenty patients underwent a late postoperative vaginoscopy, 14 of whom showed an adequate introitus and vagina, whereas five had different degrees of narrowing of the introitus. Six patients had a urethrovaginal fistula. One ureter was accidentally divided and one vagina had complete ischaemic necrosis.

Anorectal anomaly: a review of 82 cases seen at JUTH, Nigeria

The management of anorectal anomaly remains a challenge to Surgeons. This study was carried out to determine the pattern and outcome of management of anorectal malformations (ARM) in a Nigerian tertiary hospital. The clinical and operative records of consecutive children with anorectal anomaly managed between October 1990 and September 2000 at the Jos University Teaching Hospital, Jos, were reviewed. There were 82 patients, (57.3%) males and 35 (42.3%) females (m:f=1.3:1). There were 20 (24.4%) cases each of the high and intermediate types, 40 (48.8%) low variety and 2 (2.4%) cases of persistent cloaca. The high type was proportionately commoner in the males. Less than one-third presented within 24 hours. Eighty three percent of patients (mainly males) presented in acute intestinal obstruction. Passage of stools from abnormal sites and "imperforate" anus were complaints in 60 (73.1%) patients each. A total of 63 patients had definitive corrective procedure. Four patients with stenotic anus were treated by serial anal dilatations while 29 with low anomaly had anoplasty during the neonatal period. Twenty eight patients with intermediate or high anomalies or persistent cloaca had definitive repair or pull-through operations carried out 6-12 months after an initial colostomy. The definitive pull-through operations included abdominoperineal pull-through in 11 patients, PSARP in 15, while 2 girls with persistent cloaca had posterior sagittal anorectovaginoure-throplasty (PSARVUP). Twenty nine children were fully continent of stools after surgery; three patients developed occasional faecal soiling; while six patients had faecal incontinence. Anorectal anomaly is common, but presentation is late in our environment. Although mortality rate was high (26%), early results of definitive operation among survivors were generally good after a mean follow-up period of 19 months.