The Currarino triad: complex of anorectal malformation, sacral bony abnormality, and presacral mass

1984 ◽  
Vol 14 (4) ◽  
pp. 220-225 ◽  
Author(s):  
D. R. Kirks ◽  
D. F. Merten ◽  
H. C. Filston ◽  
W. J. Oakes
2008 ◽  
Vol 45 (5) ◽  
pp. 272-273 ◽  
Author(s):  
Toshiro Nagai ◽  
Rumiko Katoh ◽  
Tomonobu Hasegawa ◽  
Hirofumi Ohashi ◽  
Yoshimitsu Fukushima

1997 ◽  
Vol 32 (1) ◽  
pp. 58-61 ◽  
Author(s):  
Seong-Cheol Lee ◽  
Yong-Soon Chun ◽  
Sung-Eun Jung ◽  
Kwi-Won Park ◽  
Woo-Ki Kim

2021 ◽  
Vol 62 (2) ◽  
pp. 103-111
Author(s):  
Hongliang Xia ◽  
Mingcui Fu ◽  
Hangzhou Wang ◽  
Xu Cao ◽  
Xiangming Yan ◽  
...  

Currarino syndrome (CS) is a rare congenital disorder characterized by anorectal malformation, sacral agenesis and presacral mass. We performed video-urodynamics (VUDS) assessment of patients with CS to characterize the lower urinary tract function, individualize management plans and to follow outcomes. We conducted a cross-sectional study on 11 patients diagnosed with CS at the spina bifida multidisciplinary clinic. Lower urinary tract function was assessed by VUDS from three months to 12 months after neurosurgery. All patients had sacral agenesis; nine patients had anorectal malformation (9/11, 81.8%) and five patients had a presacral mass (5/11, 45.5%). The average age at neurosurgery was 7.9 months (range, 2–19). One patient had bilateral vesicoureteral reflux (VUR) with increasing detrusor pressure at the end of filling. In two patients, the detrusor activity showed weakening during urination, while no other lower urinary tract abnormality was identified on urodynamic evaluation. Six patients underwent VUDS before and after surgery; of these, two patients showed improved bladder function after surgery, while the remaining four patients showed no change in urodynamics. The average duration of follow-up was 27.5 months (range, 9–51). Renal function was normal in all patients. We can conclude that patients with CS often exhibit associated neurological abnormalities. VUDS assessment may help detect lower urinary tract dysfunction at an early stage and facilitate timely urological intervention to avoid kidney damage.


2015 ◽  
Vol 5 ◽  
pp. 10
Author(s):  
Sinan Akay ◽  
Bilal Battal ◽  
Bulent Karaman ◽  
Yalcin Bozkurt

Currarino syndrome is a hereditary pathology that is characterized by sacrococcygeal bone defect, presacral mass, and anorectal malformation. Sacrococcygeal bone defect is almost always a part of the syndrome. The complete form of this entity displays all three abnormalities and is very uncommon. In this report, we present the magnetic resonance imaging findings of a case with complete form of Currarino syndrome recognized in adulthood.


2001 ◽  
Vol 94 (1) ◽  
pp. 158-161 ◽  
Author(s):  
Masamichi Kurosaki ◽  
Hideki Kamitani ◽  
Yuichi Anno ◽  
Takashi Watanabe ◽  
Tomokatsu Hori ◽  
...  

✓ The Currarino triad is a unique complex of congenital caudal anomalies including anorectal malformation, sacral bone abnormality, and presacral mass. In this report, the authors describe three cases with the complete Currarino triad in a family. The authors treated a 30-year-old mother with an anterior sacral meningocele, her 1-year-old son with a combination of anterior sacral meningocele and dermoid cyst, and her 4-year-old daughter with an epidermoid cyst. These three patients had associated sacral agenesis and anorectal malformations. To the authors' knowledge, this is the first report describing radiological and operative findings of complete familial Currarino triad in which a mother and her two children were affected.


2018 ◽  
Vol 22 (5) ◽  
pp. 584-590 ◽  
Author(s):  
Michael D. Cearns ◽  
Samantha Hettige ◽  
Paolo De Coppi ◽  
Dominic N. P. Thompson

OBJECTIVEIt is well established that Currarino syndrome (CS) may be associated with spinal dysraphism. Here, the authors report on 10 CS patients with dysraphic anomalies who had undergone a combined neurosurgical and general surgical approach to repair the dysraphic anomalies and resect the presacral mass in a single operation. They discuss the spectrum of spinal dysraphism that may coexist in CS in the context of its developmental etiology.METHODSChildren with a confirmed CS diagnosis who had undergone the combined operative approach were identified from a departmental database. Presenting features were recorded and preoperative imaging was analyzed to record features of the presacral mass and the dysraphic anomalies. The histopathological nature of the resected presacral mass and the outcomes postoperatively and at the last follow-up were reviewed.RESULTSBetween 2008 and 2015, 10 patients presented with CS, 9 with constipation. Median age at the time of surgery was 1.3 years. Six of the 10 patients had anorectal malformation consisting of anal stenosis, rectal stenosis, or imperforate anus. Spinal anomalies included anterior meningocele (5 cases), low-lying conus (8), terminal syrinx (4), fatty filum (5), caudal lipoma (3), and intraspinal cyst (1). In all cases, the lumbosacral spinal canal was accessed via a midline approach with laminoplasty, allowing spinal cord untethering and repair of the dysraphic anomalies. Following dural closure, the incision was extended inferiorly to incorporate a posterior sagittal approach to resect the presacral mass. The histopathological nature of the mass was mature teratoma (8 cases), complex hamartomatous malformation (1), or neurenteric cyst (1). There were no new instances of neurological deterioration, with most instances of persisting morbidity related to constipation (6 cases) or neurogenic bladder dysfunction (8). There were no infective complications, no instances of cerebrospinal fluid fistula, no recurrences of the presacral mass, and no cases of retethering of the spinal cord.CONCLUSIONSAlthough not part of the original triad, spinal dysraphic anomalies are common in CS and in keeping with a disorder of secondary neurulation. Lumbosacral MRI is an essential investigation when CS is suspected. Children are at risk of sphincter impairment due to the anorectal malformation; however, both spinal cord tethering and compression from the presacral mass may further compromise long-term continence. A combined operative approach to repair the dysraphic anomalies and resect the presacral mass is described with good postoperative and long-term outcomes.


2017 ◽  
Vol 18 ◽  
pp. 19-23
Author(s):  
Sofia Morão ◽  
Fernando Chaves ◽  
Daniel Virella ◽  
Fátima Alves ◽  
Rui Alves ◽  
...  

2010 ◽  
Vol 80 (6) ◽  
pp. 470-470
Author(s):  
Elan Daniel Kaplan ◽  
Warren Matthew Rozen ◽  
Anand Murugasu ◽  
Paul Sitzler
Keyword(s):  

2016 ◽  
Vol 27 (06) ◽  
pp. 495-502 ◽  
Author(s):  
Clare Skerritt ◽  
Alejandra Vilanova Sánchez ◽  
Victoria Lane ◽  
Richard Wood ◽  
Geri Hewitt ◽  
...  

Background The authors of this article became aware of significant differences in the management of two females with a rectovestibular fistula and associated vaginal agenesis. In one patient, a sigmoid colovaginoplasty was performed at the time of the posterior sagittal anorectoplasty (PSARP), and the other underwent repair of the rectovestibular fistula with a primary PSARP, but the surgeons elected to delay the timing of vaginal reconstruction. We decided to review the literature, to establish if recommendations could be made to optimize the management of these children based on current evidence. Methods A literature review was conducted to determine the management and long-term outcomes in patients with an anorectal malformation and associated vaginal atresia. Specific gynecological outcomes assessed were menstrual egress and adequacy of the vaginal replacement for penile–vaginal intercourse. Results Eighty-eight cases were included in the review. Age at diagnosis had a bimodal distribution: 0 to 5 years, 56%; >10 years, 37%. Vaginal atresia was recognized before the operation in 45 patients. Types of vaginal atresia encountered were: (a) distal vaginal atresia (n = 17), (b) vaginal agenesis with absent Mullerian development (n = 47), (c) vaginal agenesis with variable Mullerian development (n = 17), and (d) cervico-vaginal agenesis (n = 7). Types of vaginal replacement used were sigmoid colovaginoplasty (n = 26), distal rectal fistula as neovagina (n = 30), terminal ileum (n = 5), vaginal pull-through (n = 9), and others/unknown (n = 8). Two patients followed a perineal dilatation program and nine patients await reconstruction. Median follow-up was 18 months (6 weeks to 31 years). Long-term menstrual outcomes were reported in 18 (21%) patients. Sexual function was reported in 10 (11%) patients. Three pregnancies were reported but none resulted in live births. Conclusion Vaginal atresia is often missed in association with ARMs. Management should be in collaboration with pediatric gynecologists. Due to a lack of long-term outcome data, no definite conclusion can be drawn for the best technique, tissue, or timing of vaginal replacement. The opportunity to perform vaginal replacement in conjunction with the rectal repair may be worth considering because of a shallower pelvis, nonscarred tissue planes, and the excellent surgical exposure.


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