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CASE REPORT
Year : 2017  |  Volume : 5  |  Issue : 1  |  Page : 40-42

Anal stenosis with H-type rectourethral fistula in a male: A rare anorectal malformation


Department of Paediatric Surgery, TNMC and BYL Nair Hospital, Mumbai Central, Mumbai, Maharashtra, India

Date of Web Publication12-Apr-2017

Correspondence Address:
Hemanshi Shah
Department of Paediatric Surgery, TNMC and BYL Nair Hospital, Mumbai Central, Mumbai - 400 008, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2320-3846.204415

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  Abstract 

H-type rectourethral fistula in males is a rare congenital anorectal malformation. It is associated with anal stenosis at normal site which generally delays diagnosis. We describe a case of a 6-month-old male child who presented with intermittent passage of fecal matter in urine along with ribbon-shaped stools through anal orifice, on straining. Investigations revealed an H-shaped rectourethral fistula with anal stenosis. Minimal sagittal anorectoplasty with fistula ligation was done.

Keywords: Anorectal malformation, congenital, rectourethral fistula


How to cite this article:
Tiwari C, Shah H, Bothra J, Kumbhar V. Anal stenosis with H-type rectourethral fistula in a male: A rare anorectal malformation. Saudi Surg J 2017;5:40-2

How to cite this URL:
Tiwari C, Shah H, Bothra J, Kumbhar V. Anal stenosis with H-type rectourethral fistula in a male: A rare anorectal malformation. Saudi Surg J [serial online] 2017 [cited 2020 Oct 31];5:40-2. Available from: https://www.saudisurgj.org/text.asp?2017/5/1/40/204415


  Introduction Top


H-type rectourethral fistula is included in the rare variants of Krickenbeck classification due to the observation that this variant was commonly seen in the Asian continent. Its incidence in the West is about 3%.[1] It is predominant in females. Although rare, it is associated with major urethral anomalies in males. The embryology is not clear, and diagnosis is usually delayed. Radiologic contrast studies and endoscopy help in diagnosis. Multiple surgical approaches and operative procedures are described for its repair. Prognosis is good as far as anal continence is concerned because the anal sphincter complex development is normal.


  Case Report Top


A 6-month-old male child presented with intermittent passage of fecal matter in urine since birth and excessive straining during defecation with passage of ribbon-like stools through the anal orifice. There was no history of urinary tract infection, instrumentation, or trauma. The patient had left upper eyelid coloboma and congenital talipes equinovarus on left side. He had atrial septal defect detected at two-dimensional echocardiography.

On examination, his perineum was well developed and the external genitalia were normal. The anal opening was at normal site but stenotic and did not even admit the little finger [Figure 1]. The presence of fecal matter in urine was confirmed clinically. Examination of the spine was normal. Abdominal ultrasonography was normal.
Figure 1: Well-developed perineum with stenosed anus present at normal site (arrow)

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Examination under anesthesia was done and anal stenosis confirmed. Hegar's dilator no. 12 could not be negotiated. Diagnostic cystoscopy showed a fistula opening in the posterior wall of membranous urethra just distal to verumontanum. The rest of the posterior urethra and the whole of the anterior urethra were normal. This fistula was cannulated with a ureteric stent, and the end of stent was retrieved from the rectum [Figure 2]. The rectal opening was confirmed by rectoscopy.
Figure 2: Postcystoscopy X-ray with ureteric stent across the fistula in situ and a red rubber catheter (number 8) passed per anum into the rectum (arrow)

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Posterior minimal sagittal anorectoplasty was done without a protecting colostomy. The patient was 7 months of age at the time of definitive surgery. Intraoperatively, the fistula opening was seen 1 cm proximal to the dentate line on the anterior rectal wall [Figure 3]. The fistula was ligated, the proximal rectum was mobilized and neo-anus was reconstructed. The patient is continent for both urine and feces for 3 years of follow-up.
Figure 3: Intraoperative photo with fistulous opening (arrow) seen proximal to the dentate line on the anterior rectal wall

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  Discussion Top


H-type anorectal malformation (ARM) is an extremely rare variant in the spectrum of congenital ARM.[1] This malformation is found more commonly in Asia (12% of ARM) and there is female preponderance.[1],[2],[3],[4] This type of malformation which has been reported to be commoner in females from Asia is not often congenital and has been reported to follow some sort of inflammatory process.[4] In females, it has been described by various authors as double termination of the “alimentary canal”, “perineal canal”, and “H” or “N” type fistula.[5] The incidence reported in Western countries is about 3%.[1],[2] This malformation is rare in male patients and is associated with other anatomic anomalies such as renal malformations, urethral hypoplasia or atresia, and tracheoesophageal fistula.[5],[6] The H-type malformation in males presents as a fistula to the urinary tract along with a stenosed anus which may or may not be ectopic.[1],[5],[7]

The embryologic basis for this malformation is not clear, and multiple mechanisms have been proposed.[1] The embryologic etiology may not be the same for males and females.[1],[5] Stephens and Donnellan [5],[8] have proposed impaired alignment of the cranial and caudal septal dividing the internal cloaca as the etiology of the H-type fistula. De Vries and Friedland [3],[5] described the H-type fistula in males as the persistence of the cloacal duct of Reichel, which links anterior and posterior compartments of the cloaca.[1],[3],[9] The most accepted theory is interruption of the dorsal part of the cloacal membrane by an isolated defect.[4] The associated anomalies in the VACTERL spectrum are often seen because of the similar abnormality in septation.[1]

There is delay in diagnosis of this type of ARM because of the presence of a normally sited anal opening at birth. The patients usually present in childhood with a variety of symptoms such as stool in the urine in males and in vestibule in females, recurrent perineal infections, multiple episodes of urinary tract infections, and straining during defecation.[1],[5] High index of suspicion is required to diagnose this malformation. Radiological contrast studies (barium enema and micturating cystourethrography), magnetic resonance imaging, endoscopy, and examination under anesthesia aid in the diagnosis.[7]

Multiple approaches and surgical procedures alone or in combination have been described in literature including vestibuloanal pull-through, anterior rectal wall pull-through, limited posterior sagittal anorectoplasty (PSARP), endorectal pull-through, and direct excision.[5],[6],[7] The procedure may or may not be combined with a colostomy.[5] A diverting colostomy has not been shown to cure the fistula (except in cases of acquired anovestibular fistula) or to prevent recurrences.[5],[6] In cases with associated urethral stenosis and/or atresia, suprapubic cystostomy with diverting colostomy may be required followed by urethral dilatation till normal caliber is achieved and urethroplasty at a later date.[10] In our patient, PSARP was done so as to provide adequate exposure for fistula ligation.

Recurrence and wound dehiscence are the two frequently reported complications.[6],[7] Prognosis is good as far as anal continence is concerned as the anal sphincter complex development is normal.[1] In literature, all such reported cases were continent for both urine and stools.[11] In patients with major anomalies, associated morbidities may be present.

Review of literature of such patients reveals that most of these patients had an ectopic and stenosed anus (an incidence of 38%). Most of them (77%) had an associated anomaly: Cardiac, renal, spinal, tracheoesophageal fistula, limb and eye anomalies. The approach for definitive repair used was anterior perineal in most of the cases. Posterior Sagittal approach was used only in two patients.[1] Functional prognosis was good in all the reported cases.[11]


  Conclusion Top


H-type malformations are rare in males and often associated with associated congenital anomalies. Diagnosis is often delayed because of the presence of anal opening at the normal site though stenotic. The high index of suspicion coupled with radiologic and endoscopic procedures aid in diagnosis. Multiple operative procedures have been described for it. Prognosis is good in terms of continence.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Slater BJ, Fallon SC, Brandt ML, Lopez ME. H-type anorectal malformation: Case report and review of literature. J Pediatr Surg Case Rep 2014;2:89-92.  Back to cited text no. 1
    
2.
Kelleher DC, Henderson PW, Coran A, Spigland NA. The surgical management of H-type rectovestibular fistula: A case report and brief review of the literature. Pediatr Surg Int 2012;28:653-6.  Back to cited text no. 2
    
3.
Banu T, Hoque M, Laila K, Ul-Haq A, Hanif A. Management of male H-type anorectal malformations. Pediatr Surg Int 2009;25:857-61.  Back to cited text no. 3
    
4.
Lawal TL, Eighemhenrio A, Kumolalo FO. Modified transanal repair of congenital H-type rectovestibular fistula: A technique to avoid recurrence. Afr J Paediatr Surg 2013;10:38-40.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Jain P, Mishra P, Shah H, Parelkar S, Borwankar SS. Anovestibular fistula with normal anal opening: Is it always congenital? J Indian Assoc Pediatr Surg 2008;13:137-9.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Rintala RJ, Mildh L, Lindahl H. H-type anorectal malformations: Incidence and clinical characteristics. J Pediatr Surg 1996;31:559-62.  Back to cited text no. 6
    
7.
Lawal TA, Chatoorgoon K, Bischoff A, Peña A, Levitt MA. Management of H-type rectovestibular and rectovaginal fistulas. J Pediatr Surg 2011;46:1226-30.  Back to cited text no. 7
    
8.
Stephens FD, Donnellan WL. “H-type” urethroanal fistula. J Pediatr Surg 1977;12:95-102.  Back to cited text no. 8
    
9.
van der Putte SC. Normal and abnormal development of the anorectum. J Pediatr Surg 1986;21:434-40.  Back to cited text no. 9
    
10.
Al-Bassam A, Sheikh MA, Al-Smayer S, Al-Boukai A, Al-Damegh S. Congenital H-type anourethral fistula with severe urethral hypoplasia: Case report and review of the literature. J Pediatr Surg 1998;33:1550-3.  Back to cited text no. 10
    
11.
Kaselas C, Philippopoulos A, Petropoulos A. Evaluation of long-term functional outcomes after surgical treatment of anorectal malformations. Int J Colorectal Dis 2011;26:351-6.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]


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