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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 55  |  Issue : 2  |  Page : 64-66

Sacrococcygeal teratoma Type IV presenting as anal canal duplication: Lessons learned


Department of Paediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India

Date of Submission12-Nov-2021
Date of Decision08-Feb-2022
Date of Acceptance09-Feb-2022
Date of Web Publication25-Apr-2022

Correspondence Address:
Rahul Gupta
Department of Paediatric Surgery, SMS Medical College, Jaipur, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/fjs.fjs_229_21

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  Abstract 


We present an extremely rare association of anal canal duplication with sacrococcygeal teratoma Altman's Type IV. A 3-year-old female child presented with anal discharge, itching, and vague perineal pain. On examination, a small second opening of 5 mm in size and 2.5 cm in length was located just posterior to the normal anus at 6 'o'clock position. The lumen ended blindly without connection to the normal anorectum. Ultrasound and radiological investigations were normal. During excision of the duplicated tract, it was seen arising from a presacral mass which was completely excised along with coccygectomy. Biopsy was confirmed mature teratoma and anal canal duplication.

Keywords: Altman's Type IV, anal canal, association, duplication, sacrococcygeal teratoma


How to cite this article:
Gupta R, Saini G, Gupta A. Sacrococcygeal teratoma Type IV presenting as anal canal duplication: Lessons learned. Formos J Surg 2022;55:64-6

How to cite this URL:
Gupta R, Saini G, Gupta A. Sacrococcygeal teratoma Type IV presenting as anal canal duplication: Lessons learned. Formos J Surg [serial online] 2022 [cited 2022 May 26];55:64-6. Available from: https://www.e-fjs.org/text.asp?2022/55/2/64/343816




  Introduction Top


Anal canal duplications are very rare congenital malformations with approximately 50 cases reported in the literature to date.[1] The presentation is usually in older children, especially females with constipation, diarrhea, mucous discharge, recurrent fistulas, abdominal pain, and perianal inflammation/pruritis.[1],[2] We present an extremely rare association of anal canal duplication with sacrococcygeal teratoma (SCT) Altman's Type IV in a female child who presented with perianal symptoms.


  Case Report Top


A 3-year-old female child presented with anal discharge, along with itching and vague perineal pain. There was no history suggestive of congenital malformations in the family. On examination, the anus was slightly patulous; there was discharge from the anus. A small 5 mm size second opening was located just behind her normal anus at 6 'o'clock position [Figure 1]. Probing under sedation revealed a tubular lesion, about 2.5 cm in length and the lumen ended blindly without connection to the normal anorectum [Figure 1]. Abdominal and systemic examinations were unremarkable. Baseline blood investigations were normal. The absence of any sacral defects was seen on sacral X-ray. The anteroposterior sacral ratio was 0.83. Ultrasounds were normal; radiological investigations, contrast enema, and fistulogram were unremarkable. Hence, a working diagnosis of anal canal duplication was kept.
Figure 1: Clinical photographs: in lithotomy position (a) a 5-mm sized orifice just behind the normal anus at 6 'o'clock position (blue arrow); in prone Jackknife position (b) normal perianal pigmentation around the opening (blue arrow) of the anal duplication tract

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The patient was placed in a prone Jackknife position for excision of the anal canal duplication tract [Figure 2]. A posterior sagittal incision was made incorporating the abnormal opening. During excision of the duplication tract, it was seen arising from a presacral mass. The mass was firm in consistency, 3 cm × 2.5 cm × 3 cm in size, irregular shape, solid-cystic, and arising from the coccyx. The duplication tract and the presacral lesion were completely excised along with coccygectomy [Figure 2]. The histopathology report confirmed mature teratoma and anal canal duplication. Postoperative radiological evaluation was normal. Two years after surgery, the patient is doing well. She is continent with good cosmesis and there is no recurrence of the tumor. The patient is being followed up with serial monitoring of alpha-fetoprotein levels and computerized tomography (CT) scans.
Figure 2: Intraoperative photographs (a) excised presacral mass (blue arrow) encountered at the proximal part of the anal duplication tract. (b) the pathological specimen which was excised en bloc with coccyx (black arrow). Cystic component of the presacral mass (yellow arrow) is seen in relation to the anal duplication tract (red arrow)

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  Discussion Top


SCT comprises 70% of all teratomas in the pediatric age group and is the most common germ cell tumor in the neonatal period with an incidence of 1:40,000 live births.[3],[4] SCT is found predominantly in females with an F: M ratio of 3–4:1.[3],[4] These tumors arise from embryologically multipotent cells of Hensen's node, which lies within the coccyx.[4] Most of these tumors are benign cystic, whereas 1%–2% are malignant.[3],[4] They are of four types according to Altman's classification with Type I being the most common and usually discovered in the neonatal period. Our case was Altman's Type IV, having a completely presacral location.[3],[4] Type IV usually presents late with urinary and bowel symptoms.[3] In our case, these symptoms were absent owing to the small size of the tumor and the absence of compression on adjacent structures.

Association of SCT with other congenital anomalies may be present in one out of four cases.[5] Spinal dysraphism, anorectal malformations (ARMs), urological and gastrointestinal malformations, including Currarino syndrome, have been described in the literature.[6] Association with hindgut duplication, especially anal canal duplication, is extremely rare.[1],[7] We report a rare case of anal duplication with SCT Type IV.

Anal canal duplication is a rare congenital malformation that was first described in 1956.[2],[8] More than 90% of these cases occur in females. The age may range from newborn to 24 years. Disease presentation is an abnormal orifice (second anal opening) located midline and just posterior to the normal anus. Most diagnoses are made incidentally.[1] The tract lies underneath the posterior part of the normal anal canal. It usually ends blindly without communication with the normal anus or intestinal tract.[1] Anal canal duplication essentially has three histological features, although these may differ based on the length of the duplication: (i) the caudal end should contain squamous epithelium, (ii) the cranial end should contain transitional epithelium, and (iii) smooth muscle cells should be present in the walls of the anal canal.[1] In our case, only the first and third characteristics were confirmed.

We agree with the hypothesis that anal canal duplications originate in early embryonic life from duplications of the dorsal cloacal membrane (cloaca) at the time of hindgut formation (1980).[5] The theory of “recanalization of excess dorsal cloacal membrane” in late embryonic life was later postulated in 1998.[9] Both anal canal duplication and ARM share a close embyopathogenesis. There is a likely common origin for anal canal duplication and ARM or Currarino's triad. Anal canal duplication may be considered as a variant form of ARM, although it is undefined.[10]

The differential diagnoses are fistula in ano and rectal duplications. A meticulous imaging evaluation including magnetic resonance imaging (MRI) (preferably) or CT (which were not contemplated in our case preoperatively) should be performed for its anatomical delineation and to rule out associated malformations.[1] There was an absence of intraspinal defect (especially tethered cord) in our case. MRI of the spine and pelvis is very useful and is the choice of investigation to effectively study the intraspinal and presacral space. The treatment is excision of the tract or mucosal striping through a perineal or posterior sagittal approach and taking care not to injure the normal anatomical structures responsible for the continence mechanism.[1] Although rare (10%), communication of duplicate anus with the normal anus may be present.[8]

In addition to the rare association of anal canal duplication with presacral teratoma, one case of colloid carcinoma of the anal duplication has been described in the literature.[10],[11] Other associations commonly described with anal duplications are midline cleft anomalies (lip/palate, spina bifida), heart defects, and genitourinary malformations.[10],[12] Rare associations are ARM (vestibular fistula) and neuronal intestinal dysplasia.[10]

The treatment of SCT is early complete tumor excision with coccygectomy.[3] The outcome is related to size, Altman's type, age at presentation, surgical technique, experience and presence of tumor spillage, presence of microscopic residue, and immature elements.[6] Failure to perform coccygectomy leads to recurrence in one-third of the cases.[7]


  Conclusions Top


A high index of suspicion for anal canal duplication should be present in a pediatric patient, especially a female child with an opening located posterior to the normal anus in the midline. Association of anal duplication with SCT Type IV is extremely rare and we are adding one more case to the world literature. This association is difficult to diagnose in preoperative imaging studies.

Declaration of patient consent

The authors certify that they have obtained appropriate patient's guardian consent form. In the form, the guardian has given the consent for the child's images and other clinical information to be reported in the journal. The guardian understands that the child's name and initial will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

Acknowledgments

I am indebted to the esteemed editorial board of Formosan Journal of Surgery and the esteemed Reviewers for a timely, professional and an excellent review of my previous manuscript.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Park J. Anal canal duplication associated with presacral teratoma in a 5-year old girl. J Pediatr Surg Case Rep 2017;19:6-8.  Back to cited text no. 1
    
2.
Narci A, Dilek FH, Cetinkurşun S. Anal canal duplication. Eur J Pediatr 2010;169:633-5.  Back to cited text no. 2
    
3.
Altman RP, Randolph JG, Lilly JR. Sacrococcygeal teratoma: American Academy of Pediatrics Surgical Section Survey-1973. J Pediatr Surg 1974;9:389-98.  Back to cited text no. 3
    
4.
Schey WL, Shkolnik A, White H. Clinical and radiographic considerations of sacrococcygeal teratomas: An analysis of 26 new cases and review of the literature. Radiology 1977;125:189-95.  Back to cited text no. 4
    
5.
Ein SH, Adeyemi SD, Mancer K. Benign sacrococcygeal teratomas in infants and children: A 25 year review. Ann Surg 1980;191:382-4.  Back to cited text no. 5
    
6.
Gupta R, Gupta AK. Sacrococcygeal teratoma with unilateral renal agenesis, spinal dysraphism, lumbar hernia, CTEV and natal teeth. Formos J Surg 2019;52:235-8.  Back to cited text no. 6
  [Full text]  
7.
Parelkar SV, Sanghvi BV, Sisodiya N, Patil SH, Mhaskar SS, Oak SN. Sacrococcygeal teratoma associated with hindgut duplication: A case report & review of literature. J Pediatr Surg Case Rep 2014;2:476-9.  Back to cited text no. 7
    
8.
Carpentier H, Maizlin I, Bliss D. Anal canal duplication: Case reviews and summary of the world literature. Pediatr Surg Int 2009;25:911-6.  Back to cited text no. 8
    
9.
Nievelstein RA, van der Werff JF, Verbeek FJ, Valk J, Vermeij-Keers C. Normal and abnormal embryonic development of the anorectum in human embryos. Teratology 1998;57:70-8.  Back to cited text no. 9
    
10.
Lisi G, Illiceto MT, Rossi C, Broto JM, Jil-Vernet JM, Lelli Chiesa P. Anal canal duplication: A retrospective analysis of 12 cases from two European pediatric surgical departments. Pediatr Surg Int 2006;22:967-73.  Back to cited text no. 10
    
11.
Dukes CE, Galvin C. Colloid carcinoma arising within fistulae in the anorectal region. Ann R Coll Surg Engl 1956;18:246-61.  Back to cited text no. 11
    
12.
Cheng LS, Courtier J, MacKenzie TC. Anal duplication in a one-year-old girl. J Pediatr Surg Case Rep 2013;1:373-4.  Back to cited text no. 12
    


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