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Currarino Syndrome: Repair of the Dysraphic Anomalies and Resection of the Presacral Mass in a Combined Neurosurgical and General Surgical Approach

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Currarino Syndrome: Repair of the Dysraphic Anomalies and Resection of the Presacral Mass in a Combined Neurosurgical and General Surgical Approach CLINICAL ARTICLE J Neurosurg Pediatr 22:584–590, 2018 Currarino syndrome: repair of the dysraphic anomalies and resection of the presacral mass in a combined neurosurgical and general surgical approach Michael D. Cearns, MBBS, BSc,1 Samantha Hettige, FRCS(SN),1 Paolo De Coppi, MD, PhD,2 and Dominic N. P. Thompson, FRCS(SN)1 1Department of Paediatric Neurosurgery and 2Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital, London, United Kingdom OBJECTIVE It 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. METHODS Children with a confirmed CS diagnosis who had undergone the combined operative approach were identi- fied 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. RESULTS Between 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 spi- nal cord. CONCLUSIONS Although 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 compres- sion 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. https://thejns.org/doi/abs/10.3171/2018.5.PEDS17582 KEYWORDS Currarino syndrome; anorectal malformation; spinal dysraphism; anterior meningocele; teratoma; tethered spinal cord; spine URRARINO syndrome (CS) is a rare constellation defined as the triad of sacral dysgenesis, anorectal malfor- of congenital malformations affecting the caudal mation, and presacral mass, CS is now recognized to be a spine and its surrounding structures and is consid- more complex clinical spectrum that can involve various Cered to be a form of caudal regression anomaly that occurs pathologies.5,7,17 These include occult spinal dysraphism, during embryonic development.1,5 Although classically spinal cord tethering, renal/ureteral anomalies, female in- ABBREVIATIONS CS = Currarino syndrome. SUBMITTED October 20, 2017. ACCEPTED May 22, 2018. INCLUDE WHEN CITING Published online August 10, 2018; DOI: 10.3171/2018.5.PEDS17582. 584 J Neurosurg Pediatr Volume 22 • November 2018 ©AANS 2018, except where prohibited by US copyright law Unauthenticated | Downloaded 09/28/21 12:01 PM UTC Cearns et al. ternal genital or uterine malformations, and various types ed and the preoperative radiological images (plain radio- of fistulas (rectoperineal, rectourethral, rectovestibular, graphs and MR images) were analyzed to record features rectocloacal, and neurenteric fistulas have all been de- of the presacral mass as well as the nature of the dysraphic scribed).7,13,17 and sacral anomalies. The histopathological nature of the Currarino syndrome has a genetic basis, and in familial resected presacral mass was determined from histologi- cases, inheritance is autosomal dominant with low pen- cal reports, and clinical records were used to assess peri- etrance. The homeobox gene motor neuron and pancreas operative complications and functional status at the last homeobox-1 (MNX1, formerly HLXB9) is implicated in follow-up. almost all familial cases and 30% of sporadic cases of CS.1,4,19 Eighty-two causative mutations have been identi- Results fied on this gene, which is mapped to the 7q36 region and encodes a transcription factor, although these have so far Ten patients were eligible for inclusion in this study (6 not been correlated well to phenotype.4,13,19 female, 4 male), all of whom had undergone surgery be- Even within the triad originally described, the pheno- tween 2008 and 2015. All patients had evidence of sacral type is variable and the presence of two components of dysgenesis and a presacral mass (minimal requirements the triad is often termed “incomplete CS.”8,15 The typi- for incomplete CS) along with an associated spinal dysra- cal sacral anomaly is a scimitar-shaped hemisacrum with phism. Six of the 10 patients also had an anorectal malfor- sparing of the first sacral vertebra.1,7,23 The most common mation, encompassing the complete CS phenotype. Me- anorectal malformations are anal stenosis, rectoperineal dian age at the time of surgical intervention was 1.3 years fistula, and imperforate anus, and in most cases the pre- (mean 2.4 years, range 20 days–11 years). sacral mass is an anterior meningocele, a teratoma, or a combined pathology.7,9,11,17,22 Presentation It is recognized that spinal dysraphism, while not an Case-specific details, including presenting features and obligatory component of the syndrome, can be a feature radiological findings, are summarized in Table 1. By far of CS that may require neurosurgical intervention.7,9,13 the most common initial symptom was constipation, with Most commonly the dysraphism occurs in the form of an almost all patients (9/10) presenting with this sign. Among anterior meningocele, which in the context of CS often these 9 patients, 3 had clinical features of bowel obstruc- contributes to the presacral mass by herniation through tion with vomiting and abdominal distention soon after an anterior sacral defect. However, other distinct spinal birth. Anorectal malformation, which was diagnosed in pathologies have been described, including spinal lipoma, 6/10 patients, encompassed a combination of imperforate fatty filum with low-lying conus, syringomyelia, diaste- anus (3), anal stenosis (2), rectal stenosis (1), and anteri- matomyelia, and fistula between the colon or bladder and orly displaced anus (1). Four of the 10 patients had docu- the spinal canal.9,13,17 A recent literature review by Kole mented evidence of urinary tract dysfunction prior to the and colleagues13 revealed that occult spinal dysraphism combined intervention, comprising 1 case each of urinary was present in 60% of 110 patients and that 43% of 163 incontinence, urinary retention, mixed symptoms, and hy- patients underwent a procedure to correct a tethered spi- dronephrosis. Evidence was based on clinical symptoms nal cord. as well as a noninvasive preoperative bladder function as- In the current study, we present a case series of 10 CS sessment consisting of an upper and a lower urinary tract patients with associated spinal dysraphism who had pre- ultrasound, as well as an evaluation of urinary flow rates sented to our service between 2008 and 2015 and were and quantification of bladder emptying using pre- and treated with a combined neurosurgical and general surgi- postvoid bladder volumes averaged over three voids. Cu- cal approach to repair the spinal dysraphism and resect the taneous stigmata of occult spinal dysraphism in the form presacral mass in a single operation. of sacral swelling and/or hairy patch were present in 2/10 patients. Three of the 10 patients had evidence of bilat- eral leg weakness on neurological examination, and in 1 Methods case, CS was incidentally diagnosed on MRI performed Patients were identified from a prospectively collected for global developmental delay and prophylactic surgery departmental database containing all neurosurgical pro- was proposed. In another case, severe anal stenosis was cedures undertaken in the department, along with any ad- identified soon after birth. The child had undergone anal verse events or complications of surgery. The study group dilatation and examination under anesthesia at another comprised all patients with a diagnosis of CS who had unit. This was followed by the introduction of daily dilata- undergone a combined neurosurgical and general surgi- tion with Hagar dilators. The child presented at 10 weeks cal approach to repair the spinal dysraphism and resect with bilateral leg weakness and neurogenic bladder dys- the
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