Effectiveness of C1 Laminectomy for Chiari Malformation Type 1: Posterior Fossa Volume Expansion and Syrinx-Volume Decrease Rate.

PURPOSE: This study aimed to analyze the effect of foramen magnum decompression with C1 laminectomy (C1L) for Chiari malformation type 1 (CM-1) in terms of improving clinical symptoms, expanding posterior fossa volume, and decreasing syrinx volume. MATERIALS AND METHODS: Between January 2007 and June 2019, 107 patients with CM-1 were included. The median patient age was 13±13 years (range: 9 months-60 years), female-to-male ratio was 1:1, and average length of tonsil herniation was 13±5 mm (range: 5-24 mm). Surgical techniques were divided into four groups based on duraplasty or C1L usage. Among the study subjects, 38 patients underwent duraplasty and had their syrinx volumes measured separately on serial magnetic resonance imaging. A three-dimensional visualization software was used to evaluate the syrinx-volume decrease rate. RESULTS: Bony decompression exhibited a mere 20% volume expansion of the lower-half posterior fossa. C1L offered a 3% additional volume expansion, which rose to 5% when duraplasty was added (p=0.029). There were no significant differences in complication rate when C1L was combined with duraplasty (p=0.526). Syrinx volumes were analyzed in 38 patients who had undergone duraplasty. Among them, 28 patients who had undergone duraplasty without C1L demonstrated a 5.9% monthly decrease in syrinx volume, which was 7.5% in the remaining 10 patients with C1L (p=0.040). CONCLUSION: C1L was effective in increasing posterior fossa volume expansion, both with and without duraplasty. A more rapid decrease in syrinx volume occurred when C1L was combined with duraplasty.

Effect of mesenchymal stem cells therapy in experimental kaolin induced syringomyelia model.

BACKGROUND: Syringomyelia is a pathological cavitation of the spinal cord. In this study, we examined whether a syrinx cavity would limit itself with axonal regeneration and stem cell activity in the cavity, and we evaluated subjects on a functional basis. METHODS: Groups were designated as kaolin, trauma, kaolin-trauma, and saline groups. Also divided out of the syringomyelia treated groups were those given human mesenchymal stem cells (hMSCs). All groups were evaluated with immunohistochemistry, electron microscopy, confocal microscopy and functionally. RESULTS: The kaolin-trauma group had a significant correction of BBB score with hMSCs therapy. The syrinx cavity measurements showed significant improvement in groups treated with hMSCs. The tissue surrounding the syrinx cavity, however, appeared to be better organized in groups treated with hMSCs. The process of repair and regeneration of damaged axons in the lesion were more improved in groups treated with hMSCs. Using confocal microscopy, fluorescence of hMSCs was observed in the central canal, in the ependymal tissue, and around the lesion. CONCLUSIONS: It was concluded that axonal repair accelerated in groups receiving stem cells, and thus, stem cells may be effective in recovery of neural tissue and myelin damage in syringomyelia.

Long-term surgical outcome of Chiari type-I malformation-related syringomyelia: an experience of tertiary referral hospital.

OBJECTIVE: Syringomyelia is a common condition seen in patients with Chiari type-I malformation (CM1). The purpose of this retrospective study was to evaluate the long-term clinical and radiological outcomes of posterior fossa decompression with duraplasty (PFDD) with coagulation of tonsillar ectopia in consecutive surgically treated adult patients with CM1-related syringomyelia (CRS). METHODS: Over 9 years' duration (1993-2001), medical charts of diagnosed patient with CM1 at our neurosurgical center were reviewed retrospectively. This study included adult patients with CM1 who had syringomyelia and underwent PFDD with coagulation of tonsillar ectopia surgery. The differences between the pre- and postoperative syrinx/cord ratio (S/C), the syrinx length, and the regression of herniated cerebellar tonsils on coronal and midsagittal MRIs were evaluated. RESULTS: A total of 87 surgical procedures (46 primary operations, 7 ventriculoperitoneal shunts, and 34 additional operations) for CRS were performed on 24 males and 22 females. The mean preoperative S/C was 0.59 ± 0.12. The means of regression in herniated cerebellar tonsils on mid-sagittal and coronal images were 11.8 ± 2.3 mm and 10.2 ± 2.2 mm (p < 0.0001), respectively. 35 (76.1%) patients were discharged after showing signs of recovery or improvement. Different complications occurred in 16 (34.8%) patients. Negative correlations were noticed between postoperative recovery/improvement and the long symptoms' duration, the herniated tonsils' extent, S/C, and the persistence of the herniated tonsils on the coronal images. CONCLUSION: Early diagnosis of patients with CRS can improve surgical outcomes. Due to its efficacy in resolving clinical symptoms and syrinx cavities, PFDD is still an optimal surgical approach for CRS.

Presacral neuroendocrine tumors associated with the Currarino syndrome.

Currarino syndrome (CS) is an autosomal dominant syndrome caused by mutations in MNX1 and characterized by anorectal abnormalities, partial sacral agenesis, and presacral masses. The presacral masses are typically benign; however, malignant degeneration can occur, and presacral neuroendocrine tumors (NETs) have been reported in six cases. We report three individuals from two families affected by CS in which multiple individuals developed presacral NETs. The first family, 491, had six members with features of CS, including two siblings who presented with presacral, Grade 2 NETs, one of which had metastasized to bone and lymph nodes. A germline c.874C>T (p.Arg292Trp) mutation was found in a highly conserved region of MNX1 in three affected members who underwent sequencing. A second somatic variant/deletion in MNX1 was not detected in either patient's tumor. In the second family, 342, the proband presented with an incidentally discovered presacral NET. The proband's father had previously undergone resection of a presacral NET, and so genetic testing was performed, which did not reveal an MNX1 mutation or copy number variants. The lack of a second, somatic mutation in the tumors from family 491 argues against MNX1 acting as a tumor suppressor, and the absence of a germline MNX1 mutation in family 342 suggests that other genetic and anatomic factors contribute to the development of presacral NETs. These cases highlight the variable presentation of CS, and the potential for malignancy in these patients.

Transplantation of Human Neural Precursor Cells Reverses Syrinx Growth in a Rat Model of Post-Traumatic Syringomyelia.

Posttraumatic syringomyelia (PTS) is a serious condition of progressive expansion of spinal cord cysts, affecting patients with spinal cord injury years after injury. To evaluate neural cell therapy to prevent cyst expansion and potentially replace lost neurons, we developed a rat model of PTS. We combined contusive trauma with subarachnoid injections of blood, causing tethering of the spinal cord to the surrounding vertebrae, resulting in chronically expanding cysts. The cysts were usually located rostral to the injury, extracanalicular, lined by astrocytes. T2*-weighted magnetic resonance imaging (MRI) showed hyperintense fluid-filled cysts but also hypointense signals from debris and iron-laden macrophages/microglia. Two types of human neural stem/progenitor cells-fetal neural precursor cells (hNPCs) and neuroepithelial-like stem cells (hNESCs) derived from induced pluripotent stem cells-were transplanted to PTS cysts. Cells transplanted into cysts 10 weeks after injury survived at least 10 weeks, migrated into the surrounding parenchyma, but did not differentiate during this period. The cysts were partially obliterated by the cells, and cyst walls often merged with thin layers of cells in between. Cyst volume measurements with MRI showed that the volumes continued to expand in sham-transplanted rats by 102%, while the cyst expansion was effectively prevented by hNPCs and hNESCs transplantation, reducing the cyst volumes by 18.8% and 46.8%, respectively. The volume reductions far exceeded the volume of the added human cells. Thus, in an animal model closely mimicking the clinical situation, we provide proof-of-principle that transplantation of human neural stem/progenitor cells can be used as treatment for PTS.

Hypothesis on the pathophysiology of syringomyelia based on analysis of phase-contrast magnetic resonance imaging of Chiari-I malformation patients.

Background: Despite several hypotheses, our understanding of syringomyelia's pathophysiology remains limited. The hypothesis proposed by Oldfield et al. suggests that piston-like movement of the cerebellar tonsils propels the cerebrospinal fluid (CSF) into the syrinx via the spinal perivascular space. However, a significant question remains unanswered: how does the CSF enter and stay in the syrinx, which has a higher pressure than the subarachnoid space. In the current study, we attempted to verify Oldfield's hypothesis using phase-contrast magnetic resonance imaging (MRI) data from patients with syringomyelia. Methods: We analyzed phase-contrast MRI scans of 18 patients with Chiari-I malformation associated with syringomyelia, all of whom underwent foramen magnum decompression, and 21 healthy volunteers. We obtained velocity waveforms for CSF and brain tissue from regions of interest (ROI) set at the various locations. These waveforms were synchronized at the peak timing of downward CSF flow. We compared the preoperative patient data with the control data and also compared the preoperative patient data with the postoperative patient data. Results: The syrinx shrank in 17 (94%) of the patients, and they experienced significant clinical improvement. When comparing pre- and postoperative MRI results, the only significant difference noted was the preoperative elevated velocity of the cerebellar tonsil, which disappeared post-surgery. The CSF velocities in the subarachnoid space were higher in the preoperative patients than in the controls, but they did not significantly differ in the postoperative MRI. The tonsillar velocity in the preoperative MRI was significantly lower than that of the CSF, suggesting that the elevated tonsillar velocity was more of an effect, rather than the cause, of the elevated CSF velocity. Conclusions: Given these findings, a completely new paradigm seems necessary. We, therefore, propose a novel hypothesis: the generative force of syringomyelia may be the direction-selective resistance to CSF flow in the subarachnoid space.

Rapid deterioration of an asymptomatic lumbosacral lipoma due to formation of an extracanalicular syrinx: case report.

The authors present the case of a 1-month-old girl with a lumbosacral lipoma who then developed an extracanalicular syrinx and experienced rapid deterioration. The patient's initial MRI study, obtained before she became symptomatic, revealed a spinal lipoma with a syrinx in contact with the lipoma-cord interface. She was initially asymptomatic but developed loss of motor function in the left leg 14 days after MRI. Emergency surgery was performed. Intraoperative findings revealed a swollen spinal cord. Lipomatous tissue on the caudal side of the conus was removed subtotally, and the central canal was opened. Expansion of the syrinx was observed intraoperatively. Postoperatively, the patient's left leg paresis remained. Postoperative MRI revealed rostral and extracanalicular expansion of the syrinx. This is the first report on the rapid deterioration of a conus lipoma due to extracanalicular expansion of a syrinx. Careful follow-up and repeat MRI should be considered for patients with spinal lipomas with syringomyelia, especially when the syrinx is attached to the lipoma-cord interface.

Chronic extradural compression of spinal cord leads to syringomyelia in rat model.

BACKGROUND: Syringomyelia is a common spinal cord lesion. However, whether CSF blockage is linked to the formation and enlargement of syringomyelia is still controversial. The current model of syringomyelia needs modification to more closely mimic the clinical situation. METHODS: We placed cotton strips under the T13 lamina of 40 8-week-old rats and blocked CSF flow by extradural compression. After 4 and 8 weeks, MRI was performed to evaluate the morphology of syringomyelia and the ratio of spinal cord diameter to syrinx diameter calculated. Locomotor function was evaluated weekly. Spinal cord sections, staining and immunohistochemistry were performed 8 weeks after surgery, the ratio of the central canal to the spinal cord area was calculated, and ependymal cells were counted. In another experiment, we performed decompression surgery for 8 rats with induced syringomyelia at the 8th week after surgery. During the surgery, the cotton strip was completely removed without damaging the dura mater. Then, the rats received MRI imaging during the following weeks and were sacrificed for pathological examination at the end of the experiment. RESULTS: Syringomyelia formed in 82.5% (33/40) of rats at the 8-week follow-up. The Basso, Beattie and Bresnahan (BBB) scores of rats in the experimental group decreased from 21.0±0.0 to 18.0 ±3.9 in the first week after operation but returned to normal in later weeks. The BBB score indicated that the locomotor deficit caused by compression is temporary and can spontaneously recover. MRI showed that the syrinx is located in the center of the spinal cord, which is very similar to the most common syringomyelia in humans. The ratio of the central canal to the spinal cord area reached (2.9 ± 2.0) × 10-2, while that of the sham group was (5.4 ± 1.5) × 10-4. The number of ependymal cells lining the central canal was significantly increased (101.9 ± 39.6 vs 54.5 ± 3.4). There was no syrinx or proliferative inflammatory cells in the spinal cord parenchyma. After decompression, the syringomyelia size decreased in 50% (4/8) of the rats and increased in another 50% (4/8). CONCLUSION: Extradural blockade of CSF flow can induce syringomyelia in rats. Temporary locomotor deficit occurred in some rats. This reproducible rat model of syringomyelia, which mimics syringomyelia in humans, can provide a good model for the study of disease mechanisms and therapies.

Intradural Pathology Causing Cerebrospinal Fluid Obstruction in Syringomyelia and Effectiveness of Foramen Magnum and Foramen of Magendie Dredging Treatment.

OBJECTIVE: This article discusses the procedure of foramina magnum and Magendie dredging, summarizing the pathologic changes in the intradural region of the craniocervical junction in patients with syringomyelia and the pathophysiologic mechanism of cerebrospinal fluid (CSF) circulation obstruction. METHODS: Clinical data from 50 adult patients with syringomyelia treated at Xuanwu Hospital from July 2018 to January 2019 were collected and retrospectively analyzed. All operations were performed with foramina magnum and Magendie dredging, and all intradural factors that may have induced the obstruction of CSF circulation were recorded. RESULTS: Intradural pathology was found in all patients. The pathologic changes that may have caused obstruction of the CSF circulation include tonsil occupying the foramen magnum and overlying foramen of Magendie in 88% (44/50), intertonsillar arachnoid adhesions in 36% (18/50), tonsil to medulla arachnoid adhesions in 18% (9/50), medialized tonsils in 70% (35/50), vermian branch of posterior inferior cerebellar artery in 22% (11/50), arachnoid veil in 16% (8/50), cisterna magna cyst in 4% (2/50), and tonsil to dura mater arachnoid adhesions in 8% (4/50). Mean duration of follow-up was 13.3 months. The long-term effective rate was 96.0%. Postoperative magnetic resonance imaging revealed that the size of the syringomyelia was reduced or completely resolved in 88% of patients. The mean preoperative Japanese Orthopaedic Association score was 12.9 ± 3.1, which improved to 14.7 ± 3.2 (P < 0.05) at last clinical follow-up. CONCLUSIONS: Intradural pathology that causes CSF circulation obstruction exists in many forms. Relieving the obstruction of the foramen magnum and foramen of Magendie is key to surgical treatment.

Chiari 1 Formation Redefined-Clinical and Radiographic Observations in 388 Surgically Treated Patients.

OBJECTIVE: The subject of Chiari formation is revisited and redefined. Results of surgical treatment of patients with Chiari formation by atlantoaxial fixation are presented. METHODS: Results were analyzed of 388 patients with Chiari formation surgically treated during 2010 to June 2019. RESULTS: Two hundred and sixty-six patients had syringomyelia. Two hundred and three patients had no craniovertebral bone abnormality and 74 patients had group A and 111 patients had group B basilar invagination. Twenty-nine patients had been earlier treated by foramen magnum decompression surgery with or without duroplasty. Clinical parameters, analysis of video recordings both before and after surgery, and patient self-assessment were included in the analysis of outcome. Immediate postoperative and sustained clinical improvement was observed in 385 patients (99.4%). CONCLUSIONS: Satisfactory clinical outcome in most patients after atlantoaxial fixation and without any manipulation of neural structures, dura, or bone in the region of foramen magnum consolidates the viewpoint that atlantoaxial instability is the nodal point of pathogenesis of Chiari 1 formation. The study suggests that Chiari 1 formation may be a secondary natural neural alteration in the face of atlantoaxial instability. The role of foramen magnum decompression surgery needs to be reassessed.

Intrathecal Morphine in the Presence of a Syrinx in Pediatric Spinal Deformity Surgery.

BACKGROUND: Intrathecal morphine (IM) is a popular adjunct for pain relief during pediatric spinal deformity surgery. There is no literature regarding its usefulness and safety in the presence of a spinal cord syrinx for patients undergoing spinal instrumentation. Anesthesiologists have previously been reluctant to use IM in the presence of any syrinx. METHODS: We retrospectively reviewed all patients with a preoperatively diagnosed spinal cord syrinx undergoing spinal deformity surgery who received IM and did not receive IM (non-IM). We recorded location of the syrinx, surgical time, length of stay, unexpected pediatric intensive care unit (PICU) admission, IM related complications (neurological, respiratory depression, or pruritus, nausea/vomiting), and reason for no IM administration. Patients with a syrinx and myelodysplasia (8), tethered spinal cord (4), paraplegia (1), holocord (1), neuroblastoma (1), and spinal cord glioma (1) were not given IM. Other reasons included a failed attempt (1), expectedly short surgical time (1), and anesthesiologist declined (2). RESULTS: There were 42 patients who met the inclusion criteria. Twenty-two patients received IM, while 20 patients did not. Patients receiving IM had 4 cervical, 5 cervicothoracic, 12 thoracic syrinxes, and 1 holocord syrinx. The non-IM group had 8 cervicothoracic, 6 thoracic, 4 holocord syrinxes, and 2 had unclassified locations. There were no neurological complications in the IM group, and 1 patient experienced respiratory depression following a shorter than expected surgery and was observed overnight in the PICU. One patient in the non-IM group with a holocord syrinx had temporary lower extremity weakness postoperatively that completely resolved and 4 patients were unexpectedly admitted to the PICU. Pruritus and nausea/vomiting was mild and similar in both groups. CONCLUSIONS: Our study demonstrates that with careful preoperative evaluation, most patients with a spinal cord syrinx can safely be given IM. Certain patients, such as those with a spinal holocord syrinx may have anatomic reasons to avoid IM, but those who are deemed appropriate for IM can receive it safely. LEVEL OF EVIDENCE: Level III-therapeutic study; retrospective comparative study.

Malignant neuroendocrine tumour in an adult female diagnosed with Currarino syndrome.

SUMMARY: Currarino syndrome is a rare, autosomal dominant condition of caudal anomalies, usually diagnosed in childhood. Adult presentation is rare and malignant transformation of the associated presacral mass even more so. We report a case of a 60-year-old female diagnosed with a malignant neuroendocrine tumour in the presacral mass in Currarino syndrome and describe the surgical management and pathological findings.

Disproportionately large communicating fourth ventricle: two case reports.

BACKGROUND: Management of the disproportionately large communicating fourth ventricle is still problematic. CASE PRESENTATION: Two cases of disproportionately large communicating fourth ventricle were treated successfully. One was a case of a 51-year-old Han Chinese woman with a complaint of headache and dizziness of 1 year's duration. Magnetic resonance imaging (MRI) demonstrated hydrocephalus with a disproportionately large fourth ventricle. She underwent a ventriculo-peritoneal shunt of the right lateral ventricle. Her symptoms were relieved totally. Five years later, magnetic resonance imaging showed she had a normal ventricular system. The other case was a 24-year-old Han Chinese man with a 2-month history of headache and dizziness accompanied by progressive loss of bilateral vision. Magnetic resonance imaging revealed hydrocephalus with a disproportionately large fourth ventricle, crowded posterior cranial fossa, and syringomyelia extending from C1 to C5. He underwent suboccipital and C1 decompression and duraplasty. Shortly after the surgery, his symptoms were relieved completely, the syringomyelia completely disappeared, and the fourth ventricle became significantly smaller. CONCLUSIONS: The management of the disproportionately large communicating fourth ventricle should be individualized. If it coexists with crowded posterior cranial fossa or syringomyelia, posterior fossa decompression could be an option for initial management. If there is no sign of crowded posterior cranial fossa or syringomyelia, shunt of the lateral ventricles might be the first choice.

Spontaneous Resolution of Syringomyelia with a 16-Year Serial Magnetic Resonance Imaging Follow-Up: A Case Report and Literature Review.

BACKGROUND: The natural course of syringomyelia is unpredictable. Only a few cases of adults with spontaneous resolution of syringomyelia associated with Chiari malformation type I (CM-I) have been reported. The timing of surgical intervention for syringomyelia remains controversial. The present report has documented a case with the longest medical history of syringomyelia reported, the associated imaging findings over 16 years, the mild symptom progression after the spontaneous resolution of syringomyelia, and a review of the relevant reported data. The natural history of our patient provides a unique view regarding the factors that might lead to the development and spontaneous resolution of syringomyelia. CASE DESCRIPTION: We present the case of a 36-year-old woman who had been monitored for 16 years for mild symptomatic CM-I and syringomyelia. After regular follow-up examinations for the initial 10 years, magnetic resonance imaging revealed partial spontaneous resolution of the syringomyelia and unchanged symptoms. However, after 6 additional years of follow-up examinations, her symptoms had mildly progressed, and the size of the syringomyelia had obviously decreased. CONCLUSIONS: The findings from our patient suggest that the natural course of mild symptomatic CM-I in adult patients will be benign and nonprogressive. Patients will commonly present with very few or mild symptoms, despite the presence of a large syrinx. It would be reasonable to observe patients with similar mild symptoms and syringomyelia. Surgery will be indicated by the type, severity, and duration of symptoms at the first clinical visit. Periodic follow-up examinations are also essential after spontaneous resolution because the pathophysiology of syringomyelia is incompletely understood and recurrence possible.

Epidural Lipomatosis and Syringomyelia in Adulthood: Case Report and Literature Review.

BACKGROUND: We review 2 previously published cases of epidural lipomatosis associated with syringomyelia. We also present the case of a 42-year-old woman with a medullary syrinx from C1 to T3, initially only with upper limb paresthesias that presented after 10 years of follow-up for left hemiparesis and paresthesias in the tongue. Chiari malformation and cerebral or spinal space-occupying lesions were ruled out, as were other causes of syrinx except the presence of epidural lipomatosis extending from T1 to T12. CASE DESCRIPTION: Right laminotomies were performed from T1 to T12, removing excessive epidural fat tissue. After surgery the symptoms remained stable. At discharge the patient progressively recovered from the hemiparesis, and in follow-up magnetic resonance imaging the resolution of the syrinx was confirmed. CONCLUSIONS: This is the third case of a patient with a spinal syrinx and no other related causes except from spinal lipomatosis, although with only 3 cases it is not possible to completely state that lipomatosis caused the syrinx. Our case supports the trend established by the 2 previous cases. For treatment, initially patients should be managed conservatively, trying to correct the underlying etiologies of spinal lipomatosis. In case of failed conservative treatment, removal of adipose epidural excess in the most appropriate way is preferred.

Intradural Extramedullary Epidermoid Cyst at the Conus Medullaris Level with Thoracic Syringomyelia: A Case Report.

Spinal epidermoid cysts are benign tumors. Syringomyelia secondary to intramedullary tumors are frequently observed. However, the association between syringomyelia and spinal intradural extramedullary epidermoid cyst in the conus medullaris region is extremely rare. We present the case of a 3-year-old male who was admitted with paraparesis and urinary retention. Magnetic resonance imaging (MRI) of the spine demonstrated intradural extramedullary lesion, compatible with epidermoid cyst, that at the conus medullaris level and a large syringomyelia extending from T4 to L1 vertebrae. Total microsurgical excision of the cyst was performed. No additional drainage was carried out for the syringomyelic cavity. Histopathological examination verified the diagnosis of the epidermoid cyst. Total excision of the cyst and disappearance of the syringomyelia were observed on MRI at 15 days postoperatively. We have clarified the etiology, clinical, histopathological and radiological features, differential diagnosis, and treatment modalities of spinal epidermoid cysts. In addition, we have discussed the possible mechanisms of syringomyelia formation in spinal intradural lesions.

Dandy-Walker syndrome associated with syringomyelia in an adult: a case report and literature review.

Dandy-Walker syndrome associated with syringomyelia is a rare condition, with few reports of adult cases. We describe an adult case of Dandy-Walker syndrome with concomitant syringomyelia. A 33-year-old man presented with a 3-month history of walking instability, numbness in the hands, memory deterioration, and urinary incontinence. A physical examination showed a positive Romberg sign. Brain computed tomography and magnetic resonance imaging showed hydrocephalus, a cyst in the posterior fossa, absence of the cerebellar vermis, hypoplasia of the corpus callosum and cerebella, and syringomyelia. All of these symptoms were consistent with the diagnosis of Dandy-Walker syndrome. Surgery involving arachnoid adhesiolysis and endoscopic third ventriculostomy was performed. At the 6-month follow-up, the symptoms were completely relieved. Magnetic resonance imaging showed that syringomyelia was greatly reduced and the hydrocephalus remained unchanged. Dandy-Walker syndrome with concomitant syringomyelia in adults is exceedingly rare. Early diagnosis and appropriate surgical treatment of this condition should be highlighted. Combined arachnoid adhesiolysis and endoscopic third ventriculostomy may be an effective approach.

Carcinoid transformation of presacral dermoid cyst in patient with currarino syndrome: a case report.

Currarino syndrome (CS) is a congenital disorder characterized by partial sacral agenesis, anorectal malformation and a presacral mass. Only three cases of carcinoid transformation of the presacral mass have been described in the literature. We present a case of carcinoid transformation of presacral dermoid cyst in patient with Currarino syndrome.