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

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.

Clinical Case of Castleman’s Disease Located at the Mesorectum

Introduction: A rare benign lymphoid proliferative disorder called Castleman’s disease is the most commonly presented as a solitary mass in the mediastinum, although sometimes with sites at the neck, axilla, mesentery, pancreas, pelvis and retroperitoneum. We report a case of Castleman’s disease in the mesorectum. According to the literature, such localization was visualized just in few cases all over the world. Case History: The patient was a 48-year-old man, who complained of lumbar pain. MRI was performed in 2014 on which the formation in the presacral mass of mesorectal fat (size up to 4,9*5,2*4,0 cm) on the level of S2 vertebrae was found, which is considered as a lymph node with the tendency to increase during 3, 5 years (+1cm). Upon admission to the clinic, differential diagnosis with lymphoma and malignant tumor were carried out. According to the colonoscopy, there were no evidence about any malignancies in the bowel. After laparoscopic removing of the mesorectal tumor and histological examination of the specimen immunohistochemistry assay was recommended. Following the analysis, the hyaline-vascular type of Castleman's disease was confirmed. Chemotherapy wasn’t recommended, because of radical surgical treatment. According to the results of monitoring in two years, no data of recurrence were identified. Conclusion: Castleman’s disease should be taken into consideration in the differential diagnosis of lymphoid formations in mesorectum.

Clinical Quiz—A Rare Case of Anal Canal Duplication in the Context of Currarino Syndrome

Currarino syndrome (CS) is a rare condition that presents with any combination of a sacral defect, a presacral mass, and an anorectal malformation. This collection, referred to as Currarino's triad, may not necessarily present as all three abnormalities in the diagnosis of the syndrome. Anal canal duplication (ACD) is an even rarer occurrence. We present a case that lies on the CS spectrum with an associated ACD and discuss a complex surgical challenge that necessitated a customized management plan, devised through a multidisciplinary approach.

Asymptomatic Presacral Paraganglioma: Management of an Unpredictable Intraoperative Finding

Paragangliomas are rare neuroendocrine tumors originating from the embryological neural crest. We report a rare case of a patient with an asymptomatic presacral mass (incidentaloma) who experienced an unpredictable intraoperative hypertensive crisis after manipulation of the tumor. Presacral neoplasms pose a diagnostic and therapeutic challenge due to their obscure anatomical location and the difficulty in performing an R0 excision. Furthermore, the management of asymptomatic paragangliomas requires a high level of clinical suspicion and expertise due to potential life-threatening intraoperative complications.

Neurosurgical management of Currarino syndrome: A case series and review of literature

Background: The Currarino syndrome (CS), defined by the triad of anorectal malformations, sacral bone deformities, and presacral masses, is rare. There are few surgical series that discuss conservative management versus the surgical approaches to these lesions. Here, we describe utilizing a combined anterior and posterior approach for resecting these lesions in four patients. Methods: Four patients with CS were treated with two-stage approaches performed by a multidisciplinary team, including pediatric neurosurgery and general surgery. The first anterior laparoscopic approach mobilized the presacral mass from its ventral attachments. The second posterior procedure detethered the spinal cord, repaired the dural defect, and facilitated removal of the presacral mass. Results: Gross total resection of all four presacral masses was accomplished without intraoperative complication; all patients clinically improved. Conclusion: The CS is characterized by a large presacral mass. Here, one must rule out malignancy and also consider diagnosis/resection due to the risks for malignant transformation. The operative approach we described in four patients utilized standard anterior mobilization of the mass, followed by posterior detethering, dural repair, and ultimate resection.

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.