Abstract
Background
Cloacal anomalies are the severest and most complex of all anorectal malformations (ARMs). They require careful evaluation and meticulous surgery tailored to suit each variant. We present our experience in a series of nine cases.
Methods
This includes a retrospective review of 9 cases of cloaca managed at a tertiary care centre between 2015 and 2019.
Results
Associated anomalies were seen in 44% cases. The definitive surgery was performed at a mean age of 15.2 months (10 months-19 months), the definitive surgery being rectal separation with total urogenital mobilisation. The common channel as measured during panendoscopy was up to 3 cm in 7 patients (78%), and only 2 patients had a common channel of more than 3 cm (22%). Of the 34 procedures that these nine patients underwent, there were four complications (12%). The median follow-up period after stoma closure was 18 months (5-32 months), and the mean age at last follow-up was 38 months (22-48 months). Five children (63%) had spontaneous voiding and remained dry in the intervening period. Three patients (37%) had poor urinary stream with dribbling and high postvoid residue requiring clean intermittent catheterisation. Six patients had faecal soiling (66%); four had daily soiling; and two had occasional soiling. Four patients had constipation (44%). Seven patients (77%) required daily enemas for bowel evacuation and to remain dry.
Conclusion
Cloacal anomalies are rare and complex ARMs. Satisfactory urinary and bowel continence rates can be achieved even in these complex anomalies.
Keywords: Cloaca, Total urogenital mobilisation, Stooling outcomes, Urinary continence, Associated anomalies
Introduction
Anorectal malformations (ARM) vary significantly in severity ranging from perineal fistulas to the most severe varieties of cloaca. Cloaca with an incidence of 1:50,000 live births literally means sewer and refers to a common opening into which the urinary, genital and gastrointestinal tract open.1 The presentation is a newborn female with a single perineal opening and small external genitalia (Fig. 1). The management of a patient with cloaca presents a formidable challenge as the presentations are myriad requiring a meticulous approach both during planning and surgery. The goals of the management are separation of urine and fecal stream and functional correction along with the preservation of the internal genitalia. Here, we present our institutes experience in the management of nine cases of cloaca over the last four years and aim to highlight the aspects of presentation, type, length of the common channel (CC), surgical correction along with the short-term urinary and stooling outcomes.
Fig. 1.
A single perineal opening with imperforate anus.
Material and methods
In this retrospective observational study, we reviewed our hospital records and operative data for all patients of cloaca managed at our centre between 2015 and 2019. We looked into the antenatal ultrasound finding, presentation in the newborn period, initial surgery, associated anomalies, age at definitive surgery, type of definitive surgery, length of CC, complications, urinary and stooling outcomes during follow-up period. For the urinary outcomes, we assessed the patient's urinary stream, history of spontaneous voiding, need for clean intermittent catheterisation, history of urinary dribbling and incontinence. For the stooling outcomes, because the age of our patients at the time of study was less than four years, we studied the history of constipation, soiling and the need for laxatives, enemas or dietary management. Follow-up data were acquired by detailed history and physical examination during the postoperative follow-up visits. The clinical photographs were acquired after proper parental consent.
Results
During the five-year study period, nine patients of cloaca were managed at this centre. The mean gestational age at birth was 36 weeks (32–40 weeks). The mean birth weight was 2400 gm (1640-2820 gm). All patients (100%) had undergone an antenatal scan of which three cases (33%) had abnormalities detected. One had a distended bladder with cystic lesion in the pelvis (hydrocolpos), second case showed a solitary kidney and the third had bilateral hydroureteronephrosis. Eight of the nine cases (89%) were born at this centre who after diagnosis of cloaca underwent right transverse colostomy in the newborn period. One patient born in a different centre had undergone sigmoid colostomy and presented to this hospital for definitive surgery at the age of four months. One patient had associated oesophageal atresia with trachea oesophageal fistula (EA-TEF) and underwent primary repair for oesophageal atresia along with transverse colostomy in the newborn period. The patient with antenatal scan of severe hydrocolpos had a grossly distended bladder at birth and underwent vesicostomy, tube vaginostomy and colostomy in the same sitting. Associated anomalies were found in four cases (44%): Atrial septal defect, bilateral vesicoureteric reflux (VUR), solitary kidney with VUR and one patient had EA-TEF, vertebral anomalies and ventricular septal defect (VACTERL association) (Table 1). The patient with the solitary kidney with high grade reflux underwent right Sober's ureterostomy at four months of age for recurrent urinary tract infection, urosepsis and failure to thrive.
Table 1.
Operative details and associated anomalies.
| Sl No | Initial surgery | Associated anomalies | Age at definitive surgery | Type of surgery | Common channel |
|---|---|---|---|---|---|
| 1 | TC | Nil | 16 m | RS + TUM | 1 cm |
| 2 | TC | ASD | 14 m | RS + TUM | 1.5 cm |
| 3 | TC | Nil | 10 m & 18 m | RS,TUM | 3 cm |
| 4 | TC + V + TVG | Nil | 15 m | RS + TUM | 2 cm |
| 5 | TC | SK,VUR | 10 m | RS + TUM | 4 cm |
| 6 | SC | Nil | 12 m & 19 m | RS,TUM + FORTUNOFF FLAP | 4 cm |
| 7 | EAR + TC | EA + VA VSD |
14 m | RS + TUM | 3 cm |
| 8 | TC | Bilateral VUR | 18 m | RS + TUM | 1 cm |
| 9 | TC | Nil | 13 m | RS + TUM | 2.5 cm |
TC, transverse colostomy; SC, sigmoid colostomy; EAR, oesophageal atresia repair; V, vesicostomy; TVG, tube vaginostomy; ASD, atrial septal defect; SK, solitary kidney; VUR, vesicoureteric reflux; VA, vertebral anomaly; EA, oesophageal atresia; VSD, ventricular septal defect; RS, rectal separation; TUM, total urogenital mobilization.
Before definitive surgery, all the patients underwent panendoscopy to look for the length of CC, anatomical arrangement of the urethra, vagina and the rectum. The definitive surgery was performed at a mean age of 15.2 months (10 months–19 months). Two cases had definitive surgery in two stages, wherein rectal separation through a posterior sagittal approach was carried out in the first stage and total urogenital mobilisation (TUM) was carried out in the second stage. One of these patients required a Fortunoff flap during TUM for a narrow vaginal opening (Table 1). All other cases underwent rectal separation and TUM as a single-stage procedure (Fig. 2 and Fig. 3). The CC as measured during panendoscopy was up to 3 cm in 7 patients (78%) and only 2 patients had a CC of more than 3 cm (22%). In the patients with CC more than 3 cm, the channel was split and used to create a mucosa-lined vestibule (Table 2). The final step in the management was stoma closure. Stoma was closed only after adequate size of anal opening was achieved with suitable duration of calibration with Hegars dilator. At the time of stoma closure, all patients underwent cystoscopy and vaginoscopy to ascertain proper patency of both the channels that was created. The mean duration between definitive surgery and stoma closure was 2.7 months (2–5 months). The patient who had undergone vesicostomy and vaginostomy in the newborn period underwent closure of vesicostomy during stoma closure. The vaginostomy tube was removed as an outpatient procedure. Of the 34 procedures that these nine patients underwent, there were four complications (12%). Two patients had surgical site infection after stoma closure which was managed conservatively, one patient developed dehiscence of perineal body after definitive surgery that required resuturing and one patient developed subacute intestinal obstruction after stoma closure which improved with conservative management. The median follow-up period in our study after stoma closure was 18 months (5–32 months) and the mean age of our patients at last follow-up was 38 months (22–48 months).
Fig. 2.
Operative photograph depicting mobilised rectum (black arrow), vagina (yellow arrow) and Foley catheter in the urethra.
Fig. 3.
Completed repair with packs in the rectum and vagina and Foley catheter in the urethra.
Table 2.
Distribution of cases based on the spectrum of anomaly.
| Spectrum of cloacal anomaly | No of cases |
|---|---|
| Very short common channel(<1 cm) | 2 |
| Short common channel(1–3 cm) | 3 |
| Short UGS with high rectal insertion | 1 |
| Cloaca with hydrocolpos | 1 |
| Long common channel(>3 cm) | 2 |
| Posterior cloaca | – |
| Cloaca with duplication of vagina and uterus | – |
Excluding the child with the solitary kidney with ureterostomy, five children (63%) had spontaneous voiding and remained dry in the intervening period. Three patients (37%) had poor urinary stream with dribbling and high postvoid residue requiring clean intermittent catheterisation (CIC). Six patients had soiling (66%); four had daily soiling and two had occasional soiling. Four patients had constipation (44%); one required laxatives and three were resistant to diet and laxatives. Seven patients (77%) required daily enemas for bowel evacuation and to remain dry (Table 3).
Table 3.
Urinary and fecal continence outcomes.
| Patient Sl No | Voluntary voiding | Stooling outcome |
|---|---|---|
| 1 | Yes | Enema |
| 2 | CIC | Enema Soiling + |
| 3 | Yes | Diet, laxative, ± enema |
| 4 | Yes | Enema |
| 5 | Ureterostomy | Diet, laxative, ± enema |
| 6 | Yes | Diet, laxative, ± enema |
| 7 | Yes | Enema Soiling+ |
| 8 | Yes | Enema |
| 9 | Yes | Enema |
CIC, clean intermittent catheterisation.
Discussion
We managed nine cases of cloaca over a four-year period from 2015 to 2019. Cloacal anomalies are the severest and most complex of all ARM. Repair of cloaca presents a serious challenge even for an experienced surgeon. It requires delicate, meticulous technique, as well as creativity, imagination and innovative thinking on the part of the surgeon.2 Cloaca per se cannot be detected on antenatal ultrasound scans. Presence of cystic structure in pelvis with bilateral hydroureteronephrosis may be a clue to the presence of cloacal anomaly.3 More commonly they are detected in the newborn period during evaluation for absent anal opening in a female neonate and examination reveals a single perineal opening with a small vestibule. Thorough clinical examination of the perineum differentiates them from the other common and benign variants such as rectovestibular fistula.
Management in the newborn period involves diversion of fecal stream as a colostomy. Hydrocolpos may cause bladder outlet obstruction and necessitates a vaginostomy and vesicostomy. The second step involves a definitive surgery to create the neoanus, vaginal and urethral opening. The final stage involves closure of colostomy. We had only one such presentation with hydrocolpos and bladder outlet obstruction, although literature describes it more frequently.4 Almost 60% of cloacal anomalies have short CC.5 We had short CC (up to 3 cm) in 7 cases (78%) and long CC in 2 cases (22%).
Workup for other associated anomalies must be carried out as there is a high incidence of urinary tract abnormalities (33–83%), mullerian structure defects (30%) and oesophageal atresia (10%).1,6 In our series, there were two renal anomalies (22%) and one oesophageal atresia (11%). The renal anomalies in our case were solitary kidney with reflux and bilateral VUR.
Timing of definitive repair is not clear and generally can be safely performed between 6 and 12 months. The aim of surgery is to create a continent anal and urethral opening with an adequate size vagina for later sexual and reproductive functions. Traditionally, these patients have been managed with posterior sagittal ano-recto-vagino-urethroplasty which involves dissection of the urethra from the vagina and is associated with a high incidence of urethrovaginal fistula, vaginal stenosis and urinary incontinence.7 Pena8 revolutionized the management of these anomalies with his concept of TUM, which involves rectal separation and mobilization of urethra and vagina as a single unit, thereby reducing the complication rates.9 All the patients in this study underwent rectal separation with TUM as a single or a two-staged procedure. Seven patients had a single-stage reconstruction with rectal separation and TUM and two patients underwent rectal separation first followed by TUM after six months. We did not encounter any urethrovaginal fistula. Vaginal stenosis was noted in one case who underwent rectal separation as an initial procedure, and during the TUM this was corrected with a modified Fortunoff Flap as described by Freitas et al.10
Management of cloaca with CC more than 3 cm poses a tough technical challenge. In our two cases with CC > 3 cm, the channel length was 4 cm and was split and rotated as a spiral flap to create a wide mucosa-lined vestibule.11
Evaluation of urinary and fecal continence is ideally evaluated when the child is 3–4 years old and is toilet-trained, whereas the mean age at follow-up in our study was 38 months. Our need for bowel management programme was 77% in comparison to 14–65% as quoted in Versteegh et al. review.12 In our study, soiling was seen in 66% of patients with the majority being severe in nature and constipation was seen in 44% of which the majority were resistant to both diet and laxatives. According to Versteegh et al.12 review, voluntary bowel movement occurred in 57% (41–60%), constipation in 30–80% and minor soiling in 71%.
Five of our patients (63%) were continent with spontaneous voiding and good urinary stream. Three patients (37%) had dribbling with high postvoid residue on ultrasound and are on CIC. A systematic review by Versteegh et al,12 found spontaneous voiding in 46% (22–54%) and CIC was required in 42% cases. Similar rates of 46% (22–54%) are reflected in recent studies for spontaneous voiding after TUM.
According to Levitt et al,13 urinary continence rate of 74% in patients with CC < 3 cm and 28% in those with CC > 3 cm can be achieved. A recent report brings out that it is not the length of CC but the distance of bladder neck from the confluence which determines continence with a shorter distance predisposing to incontinence.11
To the best of our knowledge, this is the first study from the Indian subcontinent to chronicle the urinary and stooling outcomes of this rare entity. The weakness of this study is that it is a single centre observational study with small number of cases and limited follow-up period.
Conclusion
Cloacal anomalies are rare and complex ARM. They require careful evaluation and meticulous surgery tailored to suit each variant. Satisfactory urinary and bowel continence rates can be achieved even in these complex anomalies.
Conflicts of interest
The authors have none to declare.
References
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