Triad of anorectal, sacral, and presacral anomalies

Currarino, Guido; Coln, Dale; Votteler, Theodore P.

doi:10.2214/ajr.137.2.395

Cited by 453 publications

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“…Ascra and Holder in 1965 reported the associa on of sacral mass with sacral defect and anal stenosis 14,15 . Associa on of SCT along with anorectal malforma on and sacral agenesis is called as Currarino triad 16 . In our study, one pa ent had Currarino triad with bladder and bowel symptoms.…”

Section: Resultsmentioning

confidence: 99%

Sacrococcygeal Teratoma: Experience with 36 Patients in a Teritiary Care Hospital

Kundal

Jadhav

Gupta

et al. 2015

J. Nepal Paedtr. Soc.

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Introduction: Sacrococcygeal teratomas (SCT) are the most common solid tumours in the neonatal period, originate from embryonic totipotent cells. Most of them are benign however some that present later in life and have major intra-pelvic component have greater malignant potential. Complete surgical excision provides an excellent prognosis. The aim of this study was to describe the clinical profile, age of presentation, sex distribution, diagnostic evaluation and management of children with a histological confirmed diagnosis of sacrococcygeal teratoma.Material and Methods: Patients with histological diagnosis of sacrococcygeal teratoma during a period from January 2011 to December 2012 were included in this retrospective study. Data collected included age of presentation, sex distribution, symptoms, associated anomalies, diagnostic evaluation, operative approach technique employed, tumour histology, operative complications and outcome.Results: Thirty six patients with diagnosis of sacrococcygeal teratoma were included in the study. Of these children, 58.33% were females and 41.66% were males. The median age at presentation was four days. 69.44% of patients presented during the neonatal period. 77.77% of patients presented with sacrococcygeal mass followed by lower abdominal mass in 11.11%. The tumour was resected by sacral approach in 27 patients and abdomino sacral approach in nine patients. Tumour was resectable in 34 patients and two had incomplete resection. Follow up duration varied from six months to 24 months.Conclusion: Sacrococcygeal teratomas represent a group of benign and malignant lesions of children. Neonates present with benign disease and aggressive lesions are seen as age progresses. Overall survival of SCT is high.J Nepal Paediatr Soc 2015;35(1):89-93

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Section: Resultsmentioning

confidence: 99%

Sacrococcygeal Teratoma: Experience with 36 Patients in a Teritiary Care Hospital

Kundal

Jadhav

Gupta

et al. 2015

J. Nepal Paedtr. Soc.

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“…This syndrom is known as Currarino Triad. It is an automsomal dominant genetic disorder [9][10][11]. It can be complicated with renal failure due to reflux.…”

Section: Discussionmentioning

confidence: 99%

Sacrococcygeal Masses Other Than Meningomyelocele

Agarwal¹,

Das²,

Ghosh³

et al. 2011

Indian J Surg

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This series comprises of a variety of sacrococcygeal masses other than meningomyelocele that presented to the department of pediatric surgery of Medical College Kolkata over last 10 years. In this series, 23 cases of sacrococcygeal masses are included. Barring meningomyelocele, teratoma constitutes a major group of cases. It also includes few other interesting and atypical masses such as presacral dermoid, degenerated nerve fiber, fibrofatty tissue, rhabdomyosarcoma, etc. This is an endeavor to enlighten ourselves so that the diagnosis and management of unfamiliar sacrococcygeal masses can be done.

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“…1,2 Moreover, diagnosis of a dominant familial disorder is important, both to detect other affected family members and to administer more accurate genetic counseling.…”

Section: Discussionmentioning

confidence: 99%

“…In 1981, G Currarino defined it as the association of a sacral agenesis, an anorectal malformation and a presacral mass. 2 In 1998, mutation in the HLXB9 gene at the chromosomal region 7p36 was identified as being responsible for this phenotype. [3][4][5] This gene encodes the nuclear transcription factor HB9, which plays a role in the early phases of embryogenesis.…”

Section: Discussionmentioning

confidence: 99%

Currarino syndrome as an etiology of a neonatal Escherichia coli meningitis

et al. 2007

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We report the case of a 29-day-old baby girl in whom Escherichia coli meningitis led to the diagnosis of Currarino syndrome (CS) (OMIM 176450), an autosomal-dominant genetic disorder associated with sacral agenesis, anorectal malformation, presacral masses and spinal cord malformations. Her condition improved with antibiotics and early surgical treatment. A familial study identified other genetically related individuals with similar symptoms. Journal of Perinatology (2007) 27, 589-591; doi:10.1038/sj.jp.7211783Keywords: neonate; anorectal malformation; bacterial meningitis; Currarino syndrome; HLXB9 gene Case report A 29-day-old baby girl was referred to our hospital due to a fever of 381C, anorexia and unusual behavior. Clinical examination identified symptoms of neurological problems and shock, including moaning and cutaneous hyperesthesia. A lumbar puncture revealed high protein (8.38 g/l) and low glucose (0 g/l) levels. The cerebrospinal fluid (CSF) was cloudy with 6740 cells/mm 3 containing 91% neutrophils. Culture of the fluid isolated Escherichia coli K1, and the infant was then given ceftriaxone (100 mg/kg/day) for 21 days. Recovery was observed after 2 days of treatment. Improvement was confirmed by biochemical examination of the CSF. After 10 days of treatment, culture of the CSF isolated Bacteroides, which led to the addition of metronidazole (20 mg/kg/day) to the treatment regimen.A cutaneous mark in front of the right sacroiliac joint region was observed on the infant during hospitalization, prompting a sacrum X-ray. The X-ray identified hemi-agenesis of the right sacral pieces with conservation of S1, indicating a sickle-shaped sacrum. Anorectal examination identified a rectal stenosis.Magnetic resonance imaging (MRI) revealed a presacral mass with a probable neurenteric fistula, a tethered cord with low-lying medullary conus below the L3-L4 level and an intraspinal lipoma (Figure 1). The cerebral MRI was normal. Currarino syndrome (CS) was diagnosed (OMIM 176450) based on these observations. A single-stage surgical treatment was performed with a posterior median sagittal approach 21 days after the beginning of antibiotherapy. The tethered cord was released, the presacral tumor and the intra-spinal lipoma were excised, the dural sheath was closed and the region between the rectum and the presacral mass was repaired. Histological examination of the excised mass confirmed a mature teratoma.At 1 year of age, the girl had normal psychomotor development. The follow-up was favorable, except for the persistence of chronic constipation.A familial study was then conducted as some intrafamilial correlation has been noted in half of CS cases, where autosomaldominant inheritance and a broad inter-and intrafamilial phenotypic variability are observed. This study identified at least five members of the family with sacral anomalies, which had not been previously classified (Figure 2). Patient III2 had Streptococcus agalactae and Bacteroides meningitis revealing a neurenteric cyst and a presacral anterior meningo...

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Triad of anorectal, sacral, and presacral anomalies

Cited by 453 publications

References 0 publications

Sacrococcygeal Teratoma: Experience with 36 Patients in a Teritiary Care Hospital

Sacrococcygeal Teratoma: Experience with 36 Patients in a Teritiary Care Hospital

Sacrococcygeal Masses Other Than Meningomyelocele

Currarino syndrome as an etiology of a neonatal Escherichia coli meningitis

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