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Year : 2020  |  Volume : 25  |  Issue : 5  |  Page : 328-329

Currarino syndrome variant: Revisited

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

Date of Submission28-Dec-2019
Date of Decision07-Nov-2019
Date of Acceptance04-Jan-2020
Date of Web Publication1-Sep-2020

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

DOI: 10.4103/jiaps.JIAPS_148_19

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How to cite this article:
Gupta R, Gupta AK, Tanger R, Lal M. Currarino syndrome variant: Revisited. J Indian Assoc Pediatr Surg 2020;25:328-9

How to cite this URL:
Gupta R, Gupta AK, Tanger R, Lal M. Currarino syndrome variant: Revisited. J Indian Assoc Pediatr Surg [serial online] 2020 [cited 2020 Oct 26];25:328-9. Available from: https://www.jiaps.com/text.asp?2020/25/5/328/294001


Currarino syndrome refers to a specific malformation complex characterized by a triad of anorectal malformation (ARM), sacral defect, and a presacral mass.[1] The presacral mass may be an anterior sacral meningocele (most common) or a teratoma (approximately 40%) or hamartoma or an enteric cyst or neuroenteric cyst or a combination of these.[1],[2] Variants of Currarino syndrome were earlier described as partial sacral agenesis, ARM, and Altman's Type II sacrococcygeal teratoma (SCT).[2] We are adding one more neonate with Altman's Type II SCT with both internal (pelvic) and external components, which should be considered a variant of Currarino syndrome.

A 2-day-old preterm (<37 weeks) female neonate weighing 1940 g presented with sacrococcygeal swelling and absent anal opening. Antenatal ultrasounds were not done; there was a history of birth trauma with deformity and swelling of the left thigh. On examination, the neonate was hemodynamically stable. The abdomen was soft and the anal opening was absent; the patient was passing meconium from vestibular fistula. A sacrococcygeal swelling, extending up to the posterior margin of the anal dimple, was present [Figure 1].
Figure 1: Clinical photographs (a, b and c) showing vestibular fistula (yellow arrows), anal dimple (red arrows), and a large 5 cm × 4.5 cm sacrococcygeal swelling (blue arrows) in prone (a) and supine (b) positions; intraoperative image in prone jackknife position showing intact rectum after tumor excision (c). Abdomino-thoracic radiographs (d and e) showing multiple thoracic vertebral anomalies (black arrow), absence of lower sacral (S4–S5) vertebrae (blue arrows), and soft-tissue shadow (yellow arrows) with fracture of the shaft of the left femur (red arrows). Computerized tomography films (f and g) showing upper and lower multiple thoracic vertebral anomalies (yellow arrows) in coronal section (f); hypodense multi-septated sacrococcygeal mass with pelvic component (yellow arrows) in axial section (g)

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Laboratory investigations revealed leukocytosis (23,600/mm) and indirect hyperbilirubinemia (total bilirubin – 13.08 mg/dl); alpha-fetoprotein (AFP) levels were >1000 IU/ml. Abdominothoracic X-ray revealed the absence of sacral (S4–S5) vertebrae and fracture of the shaft of the left femur. Ultrasound abdomen revealed a sacrococcygeal multiseptated cystic mass. Computerized tomography scans revealed Altman's SCT Type II [Figure 1].

After preoperative optimization, complete excision of SCT along with its intrapelvic component was performed. Histopathological evaluation revealed mature teratoma. Postoperative course of the neonate was uneventful, became jaundice free, and is doing well on follow-up.

Currarino syndrome is extremely rare (1–9/100,000 population) with approximately 300 cases reported till date.[3] The Currarino triad was first described by Kennedy (1926), but its recognition as syndrome was done by Guido Currarino.[1],[2] Currarino syndrome has a female predilection including its variants, especially among the sporadic cases (1/3rd), while cases with familial inheritance (2/3rd) have equal gender representation.[2],[3],[4]

Associated malformations are female internal genital anomalies, urinary tract anomalies, neonatal intestinal obstruction, recurrent perianal sepsis, and tethered spinal cord.[1],[2],[3],[4] The patient of Currarino syndrome is usually diagnosed late in childhood as the lesion may remain asymptomatic or present with nonspecific symptoms such as constipation, urinary symptoms, and meningitis. On the contrary, variants of Currarino syndrome present in the neonatal period due to apparent swelling.[3]

Both low and high types of ARM have been reported with Currarino syndrome. It is characterized by a partial sacral agenesis (typically involving S2–S5).[2] Surgical management with complete excision of SCT is recommended.[2] Repair of ARM in the next setting is preferable if adequate decompression of the bowel is occurring through the fistula.[2] Follow-up with AFP and ultrasound is important to detect any local recurrence. The risk of malignancy developing in SCT is lower in Currarino syndrome than for isolated SCT.[3] The long-term prognosis in Currarino syndrome is favorable.[3]

We propose that malformation complex seen in Currarino syndrome and its variants is due to the defective migration of epiblast cells from the primitive node and proximal part of the primitive streak.[2] Failure of some of these cells to migrate from the primitive node may persist in the sacrococcygeal region as a teratoma.[2] Presence of cystic renal dysplasia, undifferentiated mesenchyme in renal interstitium, bladder agenesis, absence of external and internal genital organs in autopsy specimen of fetus with Currarino syndrome goes in favor of our hypothesis.[5]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


We are sincerely thankful to the Department of Paediatric Surgery, SMS Medical College, Jaipur, for helping in our endeavor.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Currarino G, Coln D, Votteler T. Triad of anorectal, sacral, and presacral anomalies. AJR Am J Roentgenol 1981;137:395-8.  Back to cited text no. 1
Gupta R, Sharma SB, Mathur P, Goyal RB. Variants of Currarino syndrome: Embryological association and review of pertinent literature. Int J Embryol 2014;2014:1-5. Doi: 10.1155/2014/636375.  Back to cited text no. 2
Brem H, Beaver BL, Colombani PM, Zinreich J, Scherer LR, Carson BS, et al. Neonatal diagnosis of a presacral mass in the presence of congenital anal stenosis and partial sacral agenesis. J Pediatr Surg 1989;24:1076-8.  Back to cited text no. 3
Lee S, Gower DJ, Mcwhorter JM, Albertson DA. The role of MRI in the diagnosis and treatment of anterior sacral meningocoele. J Neurosurg 1988;69:628-31.  Back to cited text no. 4
Patil N, Patel G, Gupta S. Currarino syndrome – Autopsy features of a rare case. Sch J Med Case Rep 2018;6:58-61.  Back to cited text no. 5


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