Currarino syndrome: repair of the dysraphic anomalies and resection of the presacral mass in a combined neurosurgical and general surgical approach

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.

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Spinal development and spinal dysraphism

Oxford Textbook of Neurological Surgery ◽

10.1093/med/9780198746706.003.0085 ◽

2019 ◽

pp. 983-992

Author(s):

Dominic Thompson

Keyword(s):

Spinal Cord ◽

Spinal Dysraphism ◽

Radical Resection ◽

Management Plan ◽

Congenital Disorders ◽

Spinal Cord Development ◽

Precise Diagnosis ◽

Over Time ◽

Spinal Cord Tethering

The term spinal dysraphism encompasses a group of congenital disorders of spinal cord development. This potentially confusing array of conditions is best understood from an embryological perspective, and a unifying method of classification is presented. Spinal dysraphism is associated with neurological, urological, and orthopaedic deficits, these may be present at birth or may evolve over time due to the effects of spinal cord tethering. Precise diagnosis is essential to formulating an appropriate surgical management plan in order to optimize long-term neurological outcome. Contemporary and controversial surgical advances in the field are discussed including electrophysiology directed radical resection for spinal lipomas and antenatal surgery for myelomeningocele.

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SURGICAL TREATMENT OF TUMORS OF THE UPPER-THORACIC SPINE

Traumatology and Orthopedics of Russia ◽

10.21823/2311-2905-2010-0-2-143-145 ◽

2010 ◽

Vol 16 (2) ◽

pp. 143-145

Author(s):

A. L. Heylo ◽

A. G. Aganesov

Keyword(s):

Spinal Cord ◽

Surgical Treatment ◽

Surgical Procedures ◽

Thoracic Spine ◽

Surgical Approach ◽

Long Term Results ◽

Upper Thoracic Spine ◽

Short And Long Term ◽

Upper Thoracic

The experience of surgical treatment of 19 patients with tumors of the upper-thoracic spine is analyzed. All the patients had undergone decompressive-stabilizing surgical procedures. Surgical approach, form of decompression and fixation were determined depending on the signs of compression of the spinal cord and neural structures, etiology and degree of the vertebral body destruction. Good and satisfactory short- and long-term results were achieved in all the patients. The case of surgical treatment of female patient with giant neurofibromas of Th2 vertebrae is also reported.

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Infected congenital cervical dermal sinuses leading to spinal cord abscess: two case reports and a review of the literature

Child s Nervous System ◽

10.1007/s00381-020-04778-1 ◽

2020 ◽

Vol 37 (1) ◽

pp. 225-228

Author(s):

Rhian Bevan ◽

Paul Leach

Keyword(s):

Spinal Cord ◽

Case Reports ◽

Spinal Dysraphism ◽

Febrile Illness ◽

Surgical Excision ◽

Late Recurrence ◽

Cervical Region ◽

Developmental Defects ◽

Complete Surgical Excision

Abstract Purpose Congenital dermal sinuses are a rare form of spinal dysraphism. The developmental defects are located along the midline neuroaxis, with sinuses in the cervical region being the least common. Congenital dermal sinuses can be associated with intraspinal infection as they act as a direct route from the skin and subcutaneous tissues into the spinal cord. Methods The authors present two cases of cervical dermal sinuses complicated by intramedullary abscess. Both children presented with neurological decline and febrile illness. MRI showed intraspinal abscess. Both underwent prompt surgical excision of the sinus tract, exploration of the cord and intravenous antibiotics. Results Both patients demonstrated excellent neurological recovery. Conclusions Complete surgical excision of the sinus and tract in addition to long-term antimicrobials can yield excellent neurological outcomes. At surgery, do not expect to find pus when exploring the intramedullary component. Long-term follow-up is advocated due to potential late recurrence.

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Currarino Syndrome: lower urinary tract function, treatment plan and outcomes

Investigación Clínica ◽

10.22209/ic.v62n2a01 ◽

2021 ◽

Vol 62 (2) ◽

pp. 103-111

Author(s):

Hongliang Xia ◽

Mingcui Fu ◽

Hangzhou Wang ◽

Xu Cao ◽

Xiangming Yan ◽

...

Keyword(s):

Urinary Tract ◽

Anorectal Malformation ◽

Lower Urinary Tract ◽

Lower Urinary Tract Dysfunction ◽

Presacral Mass ◽

Currarino Syndrome ◽

Sacral Agenesis ◽

Lower Urinary Tract Function ◽

Tract Function ◽

Lower Urinary

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.

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Syringomyelia in children with closed spinal dysraphism: long-term outcomes after surgical intervention

Journal of Neurosurgery Pediatrics ◽

10.3171/2019.9.peds1944 ◽

2020 ◽

Vol 25 (3) ◽

pp. 319-325 ◽

Cited By ~ 1

Author(s):

Amy K. Bruzek ◽

Jordan Starr ◽

Hugh J. L. Garton ◽

Karin M. Muraszko ◽

Cormac O. Maher ◽

...

Keyword(s):

Spinal Cord ◽

Clinical Symptoms ◽

Spinal Dysraphism ◽

Tethered Cord ◽

Presenting Symptoms ◽

Age At Surgery ◽

Intradural Lipoma ◽

The Relationship

OBJECTIVEThe nature of the relationship between spinal cord syrinx and tethered cord is not well known. It is unclear if surgical cord untethering results in resolution or improvement of an associated syrinx. The objective of this study was to report the response of spinal cord syrinx to surgical cord untethering.METHODSThe authors retrospectively reviewed all patients with a syrinx and tethered cord who presented to a single institution over an 11-year interval. Patients with open neural tube defects were excluded. Thirty-one patients were identified, 25 of whom had both clinical and imaging follow-up after surgery. Patients were grouped according to etiology of the tethered cord. Clinical outcomes and syrinx characteristics were recorded.RESULTSOf the 25 patients with tethered cord, 68% (n = 17) were male. The average age at presentation was 2.5 years (0–10.1 years) and age at surgery was 3.7 years (range 1 day to 17 years). Etiologies of tethered cord were lipomyelomeningocele (n = 8), thickened/fatty filum (n = 7), intradural lipoma (n = 5), myelocystocele (n = 2), meningocele (n = 2), and diastematomyelia (n = 1). Twenty-three of the patients underwent primary untethering, whereas 2 patients had received untethering previously at another institution. The average syrinx length and width prior to surgery were 4.81 vertebral levels (SD 4.35) and 5.19 mm (SD 2.55 mm), respectively. Conus level ranged from L1 to S3. Patients were followed for an average of 8.4 years (1.35–15.85 years). Overall there was no significant change in syrinx length or width postoperatively; the average syrinx length increased by 0.86 vertebral levels (SD 4.36) and width decreased by 0.72 mm (SD 2.94 mm). Seven of 25 patients had improvement in at least one presenting symptom, including scoliosis, weakness, bowel/bladder dysfunction, and pain. Eight patients had stable presenting symptoms. Six patients were asymptomatic and 5 patients had new or worsening symptoms, which included scoliosis, pain, or sensory changes.CONCLUSIONSAlthough some syrinxes improved after surgery for tethered cord, radiological improvement was not consistent and did not appear to be associated with change in clinical symptoms. The decision to surgically untether a cord should be focused on the clinical symptoms and not the presence of a syrinx alone. Further studies are needed to confirm this finding.

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Currarino syndrome and spinal dysraphism

Journal of Neurosurgery Pediatrics ◽

10.3171/2014.3.peds13534 ◽

2014 ◽

Vol 13 (6) ◽

pp. 685-689 ◽

Cited By ~ 19

Author(s):

Matthew J. Kole ◽

Jared S. Fridley ◽

Andrew Jea ◽

Robert J. Bollo

Keyword(s):

Imperforate Anus ◽

Congenital Anomalies ◽

Rare Case ◽

Spinal Dysraphism ◽

Tethered Cord ◽

Tethered Cord Syndrome ◽

Presacral Mass ◽

Occult Spinal Dysraphism ◽

Spinal Lipoma ◽

Currarino Syndrome

Currarino syndrome is a rare constellation of congenital anomalies characterized by the triad of sacral dysgenesis, presacral mass, and anorectal malformation. It is frequently associated with other congenital anomalies, often including occult spinal dysraphism. Mutations in the MNX1 gene are identified in the majority of cases. The authors report a rare case of Currarino syndrome in an infant with tethered cord syndrome and a dorsal lipomyelomeningocele continuous with a presacral intradural spinal lipoma, in addition to an imperforate anus and a scimitar sacrum. They review the literature to highlight patterns of occult spinal dysraphism in patients with Currarino syndrome and their relationship to tethered cord syndrome. Approximately 60% of the patients with Currarino syndrome reported in the literature have an occult spinal dysraphism. Published studies suggest that the risk of tethered cord syndrome may be higher among patients with a lipoma and lower among those with a teratoma or anterior meningocele.

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Complete Currarino Syndrome Recognized in Adulthood

Journal of Clinical Imaging Science ◽

10.4103/2156-7514.152339 ◽

2015 ◽

Vol 5 ◽

pp. 10

Author(s):

Sinan Akay ◽

Bilal Battal ◽

Bulent Karaman ◽

Yalcin Bozkurt

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Bone Defect ◽

Anorectal Malformation ◽

Imaging Findings ◽

Resonance Imaging ◽

Presacral Mass ◽

Currarino Syndrome ◽

Complete Form

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.

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