H-type anorectovaginal fistula with anterior ectopic anus in a 15-month-old girl: a case report

Abstract

Introduction and importance:

H-type anorectovaginal fistula is a rare anorectal malformation characterized by an abnormal communication between the rectum and vagina despite a normally formed or near-normal anus. Diagnosis may be delayed due to the absence of obvious external anatomical abnormalities, making clinical recognition essential, particularly in resource-limited settings.

Case presentation:

We report a 15-month-old girl presenting with fecal vaginal discharge and chronic constipation since early infancy. Clinical examination revealed an anterior ectopic stenotic anus with vaginal fecal contamination, consistent with H-type anorectovaginal fistula. Diagnosis was established clinically due to clear findings and financial constraints limiting access to advanced imaging modalities. Abdominal radiography demonstrated a loaded rectosigmoid colon, while abdominal ultrasonography was normal. The patient underwent limited posterior sagittal anorectoplasty with fistula division and reconstruction. The postoperative course was uneventful, and bowel management was initiated early. At 2-year follow-up, the patient demonstrated age-appropriate continence based on clinical assessment and parental reporting consistent with expected developmental stage, with no recurrence or complications.

Clinical discussion:

H-type anorectovaginal fistula is an uncommon variant of anorectal malformations and may present diagnostic challenges due to subtle external findings. Management remains controversial because of anatomical variability; however, posterior sagittal anorectoplasty provides direct access for precise anatomical correction. In selected patients, single-stage repair without diversion can achieve favorable outcomes when conditions are optimal.

Conclusion:

Careful clinical evaluation remains the cornerstone of diagnosis in rare anorectal malformations. Individualized surgical management based on anatomy and reconstructive principles can achieve good functional outcomes even in resource-limited environments.

Introduction

Anorectal malformations (ARMs) are congenital anomalies of the distal hindgut and perineum with an incidence of 1 in 4000–5000 live births^[1^]. They range from minor anal ectopia to complex cloacal malformations. The Krickenbeck classification has improved anatomical description, communication, and surgical planning^[2^].

HIGHLIGHTS

• This is a rare case of an H-type anorectovaginal fistula with an anterior ectopic anus in a young child.

• Careful physical examination allowed us to make the diagnosis without relying on advanced imaging.

• We performed a limited posterior sagittal anorectoplasty to repair the fistula and reposition the anus.

• The patient recovered well, and 2-year follow-up showed normal bowel function and continence.

• Individualized surgical planning can achieve excellent outcomes even in resource-limited settings.

H-type anorectovaginal fistula is a rare subtype characterized by epithelial communication between the rectum and the vagina despite a normal or near-normal anus^[3^]. As the external anatomy may appear normal, diagnosis is often delayed, with presenting features including vaginal fecal discharge, recurrent contamination, constipation, or urinary tract infections^[4^].

The coexistence of an H-type fistula with an anterior ectopic and stenotic anus, as seen in this case, is rare and may reflect abnormal cloacal development^[5^].

Diagnosis is mainly clinical, supported by perineal examination and examination under anesthesia with catheter probing^[6^]. Imaging, such as MRI or fistulography, may assist but is not essential when clinical findings are clear^[7^].

Surgical repair remains the definitive treatment; however, no single technique is universally accepted due to anatomical variability^[8^]. Options include PSARP, ASARP, transanal, transvaginal, vestibuloanal, or local fistula division approaches depending on anatomy and surgeons' experience^[9^]. In selected cases, single-stage repair without diversion may be appropriate^[10^].

Long-term outcome depends on precise anatomical reconstruction and consistent postoperative bowel management^[8–10^].

This report has been written in line with the SCARE 2025 guidelines^[11^].

Case presentation

Patient information

A 15-month-old girl was referred to our pediatric surgery unit at a tertiary hospital in Mogadishu, Somalia, with a history of fecal discharge through the vagina since early infancy. The parents also reported the passage of stool through both the anus and the vagina, associated with constipation beginning at approximately 6 months of age. There was no history of fever, urinary symptoms, trauma, prior surgical intervention, or failure to thrive.

The patient had achieved normal developmental milestones. There was no known antenatal history of maternal illness or drug exposure. The family history was non-contributory, and there was no history of consanguinity.

Clinical findings

On presentation, the child was hemodynamically stable with normal vital signs. General physical examination revealed no dysmorphic features. The abdominal examination showed mild distension. The external genital examination was normal.

Perineal examination revealed an anteriorly ectopic, stenotic anus, admitting a size 6 Hegar dilator. Stool was observed at the vaginal introitus; however, the fistulous opening could not be clearly delineated on clinical examination.

Diagnostic assessment

A systematic evaluation for associated congenital anomalies was performed. No limb abnormalities or external spinal deformities were identified. Cardiovascular examination was unremarkable; however, echocardiography could not be performed due to financial constraints. Abdominal ultrasonography demonstrated normal kidneys and urinary tract anatomy. Plain abdominal and pelvic radiographs showed no vertebral anomalies or sacral agenesis. Overall, there were no clinical or radiological features suggestive of VACTERL association.

A diagnosis of H-type anorectovaginal fistula associated with an anterior ectopic stenotic anus was made based on clinical findings. Abdominal radiography demonstrated marked fecal loading of the rectosigmoid colon (Fig. 1). Advanced imaging, including MRI and contrast fistulography, was not performed because the diagnosis was clinically evident and owing to financial constraints.

Figure 1.

Abdominal radiograph demonstrating fecal loading (arrow) within the rectosigmoid colon in a 15-month-old girl with an H-type anorectovaginal fistula and an anterior ectopic stenotic anus.

Preoperative management

The patient was admitted for preoperative optimization and managed with nil per os (NPO), intravenous fluids, broad-spectrum antibiotics (ceftriaxone and metronidazole), and rectal washouts for 2 days to reduce fecal loading. Routine preoperative laboratory investigations were within normal limits.

Following optimization and the absence of systemic infection, a single-stage definitive repair without a protective colostomy was planned in accordance with institutional practice and the surgical team's experience.

Therapeutic intervention

The procedure was performed by a consultant pediatric surgeon with substantial experience in managing anorectal malformations at a tertiary hospital in Mogadishu, Somalia (Shaafi Hospital). The patient was placed in the lithotomy position for examination under anesthesia (EUA). After sterile preparation, traction sutures were placed on the labia majora, and an 8-French Foley catheter was inserted.

An EUA confirmed an H-type anorectovaginal fistula. An 8-Fr feeding tube was passed through the vaginal opening into the anterior rectum approximately 3 cm from the perineum, confirming the fistulous communication (Fig. 2). The sphincter complex was identified using anatomical landmarks and low-current diathermy stimulation.

Figure 2.

Intraoperative identification of the H-type anorectovaginal fistula. An 8-Fr feeding tube was passed through the vaginal fistulous opening (blue arrow) into the anterior rectum (green arrow), while an 8-Fr Foley catheter was placed.

The patient was then repositioned in the prone position, and a limited posterior sagittal anorectoplasty (PSARP) was performed. A midline posterior sagittal incision was made, followed by careful dissection through the sphincter complex to expose the rectum. The perineal body was divided anteriorly to delineate the fistulous tract. The tract was opened, and the epithelial lining was excised. Circumferential traction sutures of 5-0 polyglactin 910 (Vicryl; Ethicon, Johnson & Johnson, Somerville, NJ, USA) were placed on the rectal wall to achieve uniform traction and facilitate controlled mobilization. The rectum was then mobilized circumferentially with close adherence to the rectal wall and was separated from the posterior vaginal wall, allowing tension-free reconstruction (Fig. 3A).

Figure 3.

Intraoperative views following limited posterior sagittal anorectoplasty (PSARP). (A) Mobilization of the rectum in the prone position with careful separation from the posterior vaginal wall. (B) Final intraoperative calibration demonstrating a tension-free neoanus accommodating a size-15 Hegar dilator, confirming adequate anal patency and successful reconstruction.

The anorectum was positioned centrally within the sphincter complex. The perineal body was reconstructed in layers and anoplasty was completed using 3-0 polyglactin 910 (Vicryl; Ethicon, Johnson & Johnson, Somerville, NJ, USA). Intraoperative calibration confirmed patency, allowing the passage of a Hegar size 15 dilator without tension (Fig. 3B).

Postoperative course

The postoperative course was uneventful. The patient was maintained nil per os (NPO) for 5 days following the repair, in accordance with our institutional protocol for reconstruction of anorectal malformations after PSARP without a protective colostomy. In our practice, the postoperative feeding strategy is differentiated based on the presence of fecal diversion: patients managed without a diverting colostomy are kept NPO for 5 days to minimize early fecal passage across fresh perineal and vaginal suture lines and to reduce the risk of wound contamination, dehiscence, and fistula recurrence, particularly in complex anorectal malformations. In contrast, early oral feeding is initiated on the same day or within 24 hours in patients with a synchronous protective colostomy. Oral feeding was subsequently reintroduced gradually without complications, and the patient tolerated feeds well. She was discharged in stable condition on postoperative day 8. No early postoperative complications occurred.

Postoperative management and follow-up

Postoperative management included initiation of anorectal dilation according to the Peña protocol at 2 weeks, along with stimulant laxatives and enemas for 6 weeks, both of which were gradually tapered based on clinical progress. The patient subsequently achieved spontaneous bowel movements without ongoing bowel management.

Follow-up was conducted through regular outpatient visits, including clinical examinations and parental reports. Due to the patients’ ages, formal continence scoring was not applicable, and functional assessment was based on bowel habits, continence behavior, and clinical evaluation.

Outcomes

At the 2-year follow-up, the patient had normal voluntary bowel movements with age-appropriate continence. There was no persistent soiling, fecal vaginal discharge, constipation requiring treatment, anal stricture, fistula recurrence, or late complications. The parents expressed high satisfaction with the functional outcome.

Case presentation timeline (SCARE 2025)

During early infancy, at 6 months of age, the patient developed fecal vaginal discharge with constipation at 6 months of age. At 15 months, she presented for evaluation and underwent bowel preparation, followed by examination under anesthesia and definitive repair in the same session. Postoperatively, she recovered uneventfully, resumed oral feeding on day 5, and was discharged on day 8. A structured bowel management program with Peña anal dilatation was initiated at 2 weeks. At the 2-year follow-up, the patient had normal continence with no complications.

Discussion

H-type anorectovaginal fistula is a rare variant of anorectal malformations within the Krickenbeck classification, characterized by a persistent epithelial communication between the rectum and vagina despite the presence of a normally formed or near-normal anus. Because external anatomy may appear normal, affected patients often present late with fecal vaginal discharge, constipation, or perineal contamination, rather than neonatal intestinal obstruction, leading to frequent diagnostic delay^[2,3^]. This clinical masking effect explains the importance of maintaining a high index of suspicion in infants with persistent vaginal stool passage even in the presence of apparently normal perineal anatomy.

The majority of published literature describes H-type anorectovaginal fistula as an isolated entity associated with a normally positioned or near-normal anus. Large clinical series and reviews, including those by Rintala et al and Sharma and Gupta, have consistently emphasized delayed presentation and diagnostic difficulty, without reporting coexisting significant anal positional anomalies or stenosis^[3,5^]. Similarly, standard frameworks such as the Krickenbeck classification and outcome studies by Levitt and Peña primarily describe H-type fistulas as isolated fistulous tracts within an otherwise anatomically preserved anorectal complex^[1,8^].

Embryologically, an H-type fistula is attributed to incomplete separation of the cloaca by the urorectal septum, resulting in a persistent rectovaginal communication^[1,2^]. However, the additional presence of an anterior ectopic and stenotic anus in this case suggests that the defect may not be isolated but rather part of a broader disturbance in perineal descent and anorectal positioning. This combination is rarely reported in the literature and may represent a transitional phenotype between low anorectal malformation and H-type fistula, rather than a single embryological failure point.

In contrast, the present case demonstrates a rare combination of H-type anorectovaginal fistula with an anterior ectopic and stenotic anus. A review of the available literature did not identify a clearly documented identical case describing this specific combination of anomalies. While isolated reports exist of H-type fistulas and, separately, of anorectal ectopia or anal stenosis, the coexistence of these abnormalities in a single patient represents a rare phenotypic overlap within the spectrum of anorectal malformations rather than a distinct, previously defined subtype.

This distinction is clinically important because it expands the recognized phenotypic variability of H-type fistulas. Rather than representing a purely isolated embryologic defect, this case suggests that H-type fistulas may, in select rare circumstances, coexist with abnormalities of anorectal positioning and caliber.

From a diagnostic perspective, the case reinforces the continued value of meticulous clinical assessment in resource-limited environments. While imaging modalities such as MRI or fistulography can delineate anatomy more precisely, their role remains supportive rather than essential when examination under anesthesia and catheter probing clearly demonstrate the fistulous tract^[3,4^]. In this patient, the diagnosis was established clinically and confirmed intraoperatively, reflecting the practical realities of low-resource surgical settings where management decisions are frequently based on direct anatomical assessment rather than advanced imaging.

From a surgical perspective, previously reported H-type anorectovaginal fistulas have been managed using transanal repair, local fistula division, or posterior sagittal approaches, depending on fistula height and associated anatomy^[6,7^]. However, reported cases generally suggest that most patients have normal or near-normal anal anatomy, allowing simpler repair strategies. In the present case, the coexistence of anal ectopia and stenosis necessitated a limited PSARP to allow controlled exposure, precise sphincter mapping, and simultaneous correction of both anomalies within a single operative field.

The surgical management of H-type fistula remains debated, largely because of anatomical variability and the absence of a standardized operative algorithm^[5–7^]. PSARP remains a widely used and versatile approach due to its ability to provide direct visualization of the sphincter complex and precise control of rectal mobilization^[6^]. In contrast, anterior sagittal and transanal approaches are generally considered more suitable for isolated low lesions, although they may offer limited exposure when more complex or combined anomalies are present^[7^].

The decision for a single-stage repair without protective colostomy reflects a selective rather than a universal principle. While diversion is recommended in contaminated, complex, or recurrent cases^[5,9^], carefully selected patients with adequate bowel preparation, absence of infection, and favorable tissue planes may safely undergo primary repair. This approach aligns with published series from resource-limited settings, where staged procedures are not always required when intraoperative conditions are optimal^[9,12^].

Contemporary management of anorectal malformations increasingly emphasizes functional results over purely anatomical correction. Preservation of the sphincter complex and structured postoperative bowel management protocols remain key determinants of continence^[8–10^]. In this patient, early initiation of dilatation and structured follow-up resulted in satisfactory bowel function at 2 years, suggesting that precise anatomical reconstruction may contribute to durable functional success, even in complex variants.

Overall, this case illustrates that an H-type anorectovaginal fistula should not be viewed in isolation when associated anomalies of anorectal positioning are present. Instead, it may represent part of a broader spectrum of cloacal developmental disturbances requiring individualized operative planning. The successful outcome supports tailored PSARP-based management as both a diagnostic and a reconstructive strategy in selected patients.

The novelty of this report lies in the rare coexistence of H-type anorectovaginal fistula with anterior ectopic stenotic anus, the requirement for PSARP-based reconstruction rather than isolated fistula repair techniques, and the successful single-stage correction with a favorable 2-year functional outcome in a resource-limited setting.

Limitations

This case report has several limitations that should be acknowledged.

First, the absence of advanced imaging modalities, such as magnetic resonance imaging or contrast fistulography, limited detailed preoperative anatomical delineation of the fistulous tract. However, a structured examination under anesthesia combined with clinical assessment provided sufficient diagnostic accuracy to guide definitive surgical management.

Second, long-term functional follow-up into adolescence is not yet available. Although the patient demonstrated satisfactory bowel function at the 2-year follow-up, continence outcomes may evolve with growth, particularly after the completion of toilet training and the maturation of bowel control mechanisms.

Third, objective continence scoring systems, such as the Krickenbeck criteria, could not be formally applied due to the patient’s young age. Functional assessment was therefore based on validated clinical parameters and parental reporting, which remain standard practice in early pediatric follow-up but are inherently subjective.

Finally, as a single-case report, the findings cannot be generalized. However, given the rarity of the combined anomaly described, the report provides valuable insight into diagnosis, operative strategy, and postoperative management in resource-limited settings.

Conclusion

H-type anorectovaginal fistula with an anterior ectopic stenotic anus is a rare anorectal malformation. Diagnosis is primarily clinical and can be reliably made even in resource-limited settings with a careful examination.

Management must be individualized due to the absence of a single standardized surgical technique. In this case, a limited PSARP provided effective anatomical correction with a satisfactory functional outcome.

Ethical approval

Ethical approval was not required for this single case report, in accordance with the institutional guidelines at Shaafi Hospital in Mogadishu, Somalia.

Consent

Written informed consent was obtained from the patient’s legal guardian for publication of this case report and accompanying clinical images.

Sources of funding

No funding was received for this study.

Author contributions

All authors contributed to the study conception, data collection, manuscript drafting, and critical revision.

Conflicts of interest disclosure

The authors declare no conflict of interest.

Research registration unique identifying number (UIN)

Not applicable.

Guarantor

Zubair Mohamed Dahir Sed.

Provenance and peer review

Unsolicited manuscript; externally peer reviewed.

Data availability statement

Data supporting this case report are available from the corresponding author upon reasonable request.