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Year : 2016  |  Volume : 21  |  Issue : 4  |  Page : 187-189

Currarino syndrome: Rare clinical variants

1 Department of Pediatric Surgery, All India Institute of Medical Sciences (AIIMS), Patna, Bihar, India
2 Department of Radiology, All India Institute of Medical Sciences (AIIMS), Patna, Bihar, India
3 Department of General Surgery, All India Institute of Medical Sciences (AIIMS), Patna, Bihar, India

Date of Web Publication19-Jul-2016

Correspondence Address:
Bindey Kumar
Department of Pediatric Surgery, All India Institute of Medical Sciences (AIIMS), Patna, Bihar
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9261.186550

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Currarino syndrome (CS) is a rare clinical condition. The classical presentation includes a triad of sacral anomaly, anorectal malformations, and presacral mass. This syndrome belongs to the group of persistent neuroenteric malformations. This article presents two cases of Currarino syndrome, where there was rare clinical variants such as rectal atresia in the first case and rectal stenosis in the second case. The clinical presentations were very deceptive as the first case presented as high anorectal malformation and the second case was simulating Hirschprung's disease.

Keywords: Anorectal malformation, currarino syndrome, hemisacrum, presacral mass

How to cite this article:
Kumar B, Sinha AK, Kumar P, Kumar A. Currarino syndrome: Rare clinical variants. J Indian Assoc Pediatr Surg 2016;21:187-9

How to cite this URL:
Kumar B, Sinha AK, Kumar P, Kumar A. Currarino syndrome: Rare clinical variants. J Indian Assoc Pediatr Surg [serial online] 2016 [cited 2023 Dec 2];21:187-9. Available from: https://www.jiaps.com/text.asp?2016/21/4/187/186550

   Introduction Top

Currarino syndrome (CS) is a rare clinical condition that was described by Currarino et al. in 1981. The classical presentation includes a triad of sacral anomaly, anorectal malformation, and presacral mass. [1] The sacral anomaly may include agenesis of the sacrum, coccyx, and hemisacrum. [2],[3],[4] Variations in anorectal malformation include rectoperineal fistula, rectourethral fistula, rectovestibular fistula, and anorectal stenosis. [5] Presacral mass may present as anterior meningocele, teratoma, duplication cyst of rectum, or dermoid cyst.

   Case reports Top

Case 1

A 1-year-old male child was referred to the outpatient department with complaints of not passing stool since birth with status transverse loop colostomy performed in the neonatal period at some peripheral center. Before colostomy, an attempt was made to calibrate the formed anal canal with Hegar dilator. The dilator did not go beyond a few centimeters.

Plain x-ray of the lumbosacral area revealed hemisacral deformity known as "SCIMITAR SIGN." Ultrasonography of the pelvis depicted a cystic swelling in the pelvis with complete obliteration of bowel lumen at the level of swelling. Magnetic resonance imaging (MRI) demonstrated anterior sacral meningocele and complete disruption of lumen of the bowel at the level of meningocele [Figure 1]. Operation was performed with the patient in the prone position. Lower sacral laminectomy was performed. The thecal sac was identified and the neck of thecal sac extending anterior to the sacrum was defined. Dural incision was given. No neural elements were seen entering into the meningocele sac. The neck was ligated with fine silk (4, 0) suture and dural repair was carried out. Complete rectal discontinuity was identified. Then gap between both ends was almost 1.5 cm. Posterior sagittal anorectoplasty was performed and rectal continuity was restored by mobilizing both the rectum and anal canal from above and below. The postoperative period was uneventful. After 6 weeks, colostomy closure was performed.
Figure 1: Magnetic Resonance (MR) demonstrated anterior meningocele

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In follow-up period of 8 years, the patient has been passing normal stool with preserved anorectal sensation. There are occasions of fecal soiling once or twice in 1 week.

Case 2

A 2-day-old newborn male baby was brought to the emergency outpatient department with gradual abdominal distension, bilious vomiting, and failure to pass meconium since birth. On clinical examination, the baby had pinpoint anal opening that could be negotiated only by 6 French infant feeding tube. Meconium staining of the infant feeding tube was present. Plain x-ray of the abdomen showed multiple air fluid levels. Ultrasonography of the pelvis was normal for other anomalies. On exploration, the rectosigmoid was dilated till peritoneal reflection. The clinical, radiological, and operative finding indicated Hirschsprung's disease as a possible provisional diagnosis. The baby underwent a sigmoid colostomy. The postoperative period was uneventful. Biopsy from the colostomy site revealed normal ganglion cells on histopathological examinations. In the follow-up period, distal loop colostogram showed ill-defined radio-opaque shadow anterior to the sacrum [Figure 2]. An x-ray of the lumbosacral spine showed partial sacral agenesis. The patient was planned for posterior sagittal anorectoplasty, keeping CS as a possibility. On exploration, a cyst in the pelvis was identified with fatty tissue and cheesy material inside it. The cyst was attached to the dura mater of the spinal canal and the rectum was compressed. Excision of the cyst and repair of the dura mater was done meticulously. Gross rectal stenosis was noted. Posterior sagittal anorectoplasty was performed with excision of the stenosed rectum. The excised cyst was sent for histopathological examination.
Figure 2: Colostogram revealing ill defined radio-opaque shadow anterior to sacrum

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The postoperative period was uneventful. Histopathology of the cyst was consistent with dermoid cyst. Colostomy closure was performed after 3 months. The patient has been under close follow-up for the last 3 years and has normal continence of bowel with occasional soiling episode once or twice weekly.

   Discussion Top

CS develops from the failure of separation of the caudal cell mass from the hindgut endoderm dorsoventrally. Secondarily, neurulation and maldevelopment of the cloaca results in occult dysraphic malformations and anorectal anomalies. [6],[7] Mesenchymal maldevelopment, which surrounds the spinal cord and notochord, produces a spectrum of sacral anomalies. Dural primordial anomalies give rise to anterior sacral meningocele. The presacral mass could be anterior sacral meningocele, teratoma, or enterogenous cyst. Other associations are neonatal bowel obstruction, chronic constipation, and tethered cord syndrome. [8] Based on these developmental aberrations, CS has got a wide spectrum of clinical presentation. Martucciello et al. [9] has classified CS into three types:

  1. Complete CS: CS with full expressivity presenting with the hemisacrum, anorectal malformation, and presacral mass.
  2. Mild CS: Hemisacrum and one of the two anomalies, either anorectal malformation or presacral mass will be present.
  3. Minimal CS: Only the hemisacrum is present.

In addition to the above, many other congenital anomalies have been described such as urinary, gynecological, costal, and neural tube defects and intestinal dysganglionosis. Anorectal malformation with different fistulas has been described but rectal atresia has been rarely reported in CS. The need to keep this anorectal malformation anomaly in differential diagnosis is important. It will help to plan a better treatment. To deal with these malformations, posterior sagittal anorectoplasty is the best approach. [10]

   Conclusion Top

Due to rarity and varied presentation of the Currarino syndrome, there is always chance to miss the diagnosis at initial workup for anorectal malformation. It is always advisable to be suspicious when hemisacral anomaly is found on plain x-ray film. Necessary measures must be taken to rule out the diagnosis of Currarino syndrome. Otherwise, it may pose significant problem to both the patient and surgeon.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Currarino G, Coln D, Votteler T. Triad of anorectal, sacral and pre sacral anomalies. AJNR Am J Roentgenol 1981;137:395-8.  Back to cited text no. 1
Kenedy RL. An unusual rectal polyp: Anterior sacral Meningocele. Surg Gynecol Obstet 1926;43:803-4.  Back to cited text no. 2
Ashcraft KW, Holder TM. Congenital anal stenosis with presacral tertoma: Case reports. Ann Surg 1965;162:1091-5, 1100.  Back to cited text no. 3
Ashcraft KW, Holder TM. Hereditary presacral teratoma. J Pediatr Surg 1974;9:691-7.  Back to cited text no. 4
Dias MS, Azizkhan RG. A novel embryogenetic mechanism for Currarino′s triad: Inadequate dorsoventral separation of the caudal eminence from hindgut endoderm. Pediatr Neurosurg 1998;28:223-9.  Back to cited text no. 5
Cohn J, Bay-Nielsen E. Hereditary defect of the sacrum and coccyx with anterior sacral meningocele. Acta Paediatr Scand 1969;58:268-74.  Back to cited text no. 6
Anderson FM, Burke BL. Anterior sacral meningocele: A presentation of three cases. JAMA 1977;237:39-42.  Back to cited text no. 7
Emans PJ, van Alast J, van Heurn EL, Marcelis C, Kootstra G, Beets-Tan RG, et al. The Currarino triad: Neurosurgical considerations. Neurosurgery 2006;58:924-9.  Back to cited text no. 8
Martucciello G, Torre M, Belloni E, Lerone M, Pini Prato A, Cama A, et al. Currarino syndrome: Proposal of a diagnostic and therapeutic protocol. J Pediatr Surg 2004;39:1305-11.  Back to cited text no. 9
Otagiri N, Matsumoto Y, Yoshida Y. Posterior sagittal approach for currarino syndrome with anterior sacral meningocele: A case Report. J Pediatr Surg 2000;35:1112-4.  Back to cited text no. 10


  [Figure 1], [Figure 2]

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