Inappropriate surgical interventions for midline fusion defects cause secondary tethered cord symptoms: implications for natural history report of four cases

Introduction

The causes of tethered spinal cord are various. In order to release the tethering effect of these malformations, the surgical interventions must include removal of all tethering components, reconstruction of the neural tube and sectioning of tight filum terminale as well.

Cases

The cases reported in this paper have had an operation many years before for various developmental defects. After a certain period of time (5–10?years) of the first operation, the patients reapplied to the hospital with various symptoms of spinal cord tethering, either vertical or horizontal.

Discussion

At surgical intervention, it was noted that inappropriate surgical procedures caused retethering of the spinal cord in all patients. Postoperative period of all patients were uneventful. All patients declared relief in their symptoms. We would like to draw attention that untreated (or inappropriately treated) midline developmental defects will invariably cause syndrome of tethered cord. Consequently, prophylactic surgical untethering must be applied to all patients with developmental midline defects as soon as possible.

Conclusion

It looks that tethered cord symptoms invariably appear as enough negative influence accumulates as the time passes. Elapsed time may vary but unpleasant end result invariably arrives. While these cases with tethered spinal cord develop progressive neurological symptoms, prophylactic and appropriate surgical intervention should be considered as early as possible. There is no acceptable rationale to wait for the appearance of tethered cord syndrome symptoms to perform surgical untethering of the spinal cord because of the probability of irreversibility of the symptoms (incontinence of urine in particular) of tethered spinal cord syndrome.     

Holocord syringomyelia secondary to tethered spinal cord associated with anterior sacral meningocele and tailgut cyst: case report and review of literature

Background and importance

Anterior sacral meningoceles are lesions that are uncommonly reported and can be associated with other pathology including presacral masses, tethered spinal cord, and syringomyelia. Tethered spinal cord and syringomyelia can result in neurologic deficits, while large meningoceles and presacral masses can have gastroenterologic, urologic, reproductive, and oncologic consequences.

Clinical presentation

The authors report a case of a 14-year-old girl with an anterior sacral meningocele, tailgut cyst, and tethered cord with holocord syringomyelia who presented with a tethered cord syndrome, manifested by constipation, urinary retention, bilateral lower extremity weakness, and sensory deficits. After extensive radiographic and urodynamic workups were performed, the patient was treated by the neurosurgery and pediatric surgery teams with a posterior sagittal approach for cord detethering, resection of an intradural cystic mass, resection of the anterior sacral meningocele, and resection of the adjacent presacral mass. After surgical treatment, motor weakness and sensory deficits were resolved, though urinary symptoms persisted. The syrinx resolved after detethering alone. Pathology of the intradural cystic mass and the presacral mass inferior to the anterior sacral meningocele were consistent with tailgut cyst.

Conclusion

The patient’s clinical and surgical management are discussed, and a literature review related to anterior sacral meningoceles and their related pathologies is presented. An interdisciplinary approach is required for the best treatment of this constellation of findings.     

Surgical treatment of syringomyelia associated with spinal dysraphism

Clinical and radiological features of syringomyelia in 15 patients with spinal dysraphism are reported. There were 8 patients with occult spinal dysraphism (lumbosacral lipoma) and 7 with spina bifida aperta (meningomyelocele). Syringomyelia with spinal dysraphism can be radiologically divided into two types according to the dysraphic state. The syrinx in the patients with occult spinal dysraphism occurred immediately rostral to the lipoma and was localized to the lower thoracic to lumbar levels, while in the meningomyelocele patients the syrinx extended from the cervical to the thoracic level. Large syrinx formation was recognized in 1 of the 7 occult spinal dysraphism cases and 3 of the 8 meningomyelocele cases. For syringomyelia with occult spinal dysraphism, 4 patients underwent syringo-subarachnoid shunting (S-S shunt, 2 cases) or syringostomy (2 cases) during an untethering operation. In the case of meningomyelocele, S-S shunts were placed in 2 patients. Collapse of the syrinx was achieved in all 6 patients who underwent S-S shunting or syringostomy. Decreased size of the syrinx was also noted in 3 occult spinal dysraphism patients who underwent untethering operations alone. In conclusion, a large syrinx in the case of spinal dysraphism should be surgically treated. S-S shunting is effective in both types of syringomyelia. Foramen magnum decompression may be an alternative method of surgical treatment for syringomyelia in patients with meningomyelocele.     

A single-center experience with eccentric syringomyelia found with pediatric Chiari I malformation

Introduction

Eccentric syringes associated with Chiari I malformation have received scant attention in the medical literature. Herein, we describe our experience and long-term outcome in patients with this finding.

Materials and methods

A retrospective analysis of a Chiari I database was performed. Patients known to have an associated syringomyelia were then further analyzed for the type of syrinx present. When an eccentric syrinx was noted, the symptoms and postoperative course of these patients were analyzed.

Results

Of well over 500 operative cases of Chiari I malformation, roughly 70?% (pre-syrinx and minimally dilated central canals were excluded) were found to have an associated syringomyelia. Of these, four patients were found to have an eccentrically positioned syrinx. Three of these cases presented with symptoms referable to the side of the eccentric syrinx. Postoperatively, cases with both a central and eccentrically located syrinx were found to have a greater decrease in the size of the central portion of their syrinx compared to the eccentrically located portion. Symptoms decreased in all patients.

Conclusions

The minority of our patients with hindbrain-induced syringomyelia were found to have an eccentrically located syrinx. Of these, most will have symptoms localized to the abnormal fluid-filled cavity, and these may not decrease in size as much as centrally located syringes following posterior fossa decompression. However, all symptoms decreased in those operated. Based on the literature, non-hindbrain-induced syringomyelia is more likely to result in an eccentrically placed syrinx. The mechanism for this is yet to be elucidated.     

Syringobulbia associated with posterior fossa meningioma: a review of the literature

Purpose

Syringomyelia can be defined as a degenerative, progressive and chronic spinal cord disease. Its association with tumors of the posterior cranial fossa (PCF) is a rare condition.

Methods

The authors report a rare case of syringobulbia consequent to a meningioma originating from PCF in a 17-year-old female, discussing the pathogenetic mechanism of development and the resolution of the syrinx cavity after surgical procedure.

Results

The postoperative period was uneventful without complications. At 6-month follow-up, MRI revealed complete tumor removal with resolution of the syrinx cavity.

Conclusions

In cases of syringomyelia and tonsillar herniation associated with PCF meningioma, the tumor resection allows to eliminate the mass effect and increases the size of the posterior fossa with the progressive ascent of the cerebellar tonsils and the consequent reduction of their downward movement with systolic pulsation. The re-establishment of a normal anatomical condition led to the gradual disappearance of syrinx and hydrocephalus.     

The role of flexible hollow core carbon dioxide lasers in resection of lumbar intraspinal lipomas

Purpose

The authors assess the role of flexible hollow core CO₂ laser system (Omniguide system) in resection of intraspinal lipomas.

Methods

Eight patients with intraspinal lipomas were operated using an Omniguide laser system over a 22-month period. The age range varied between 6?months and 16?years. All of them had lipoma of the conus medullaris associated with lumbar subcutaneous lipoma. Two of these had previous surgery with resection of the subcutaneous part of the lipoma; in one of them, a partial resection of the conus medullaris lipoma had also been performed. In six, there were no obvious motor or sensory deficits whereas two had gross neurologic deficits, with the deficits occurring in one after the previous decompression. Resection of the conus lipoma, untethering of the spinal cord, and reconstruction of the decompressed conus was performed in all aided with Omniguide laser system. The power settings of the laser system ranged from 4 to 8?W.

Results

Subtotal to near-total resection was achieved in all. None of the patients developed any new motor or sensory deficits. Three had postoperative CSF leaks which initially required reoperation with graft resuturing and subsequently placement of lumboperitoneal shunt. One child with preexisting neurologic deficits improved in power over the next 12?months.

Conclusion

Flexible CO₂ laser system was precise and convenient in decompressing the conus lipomas and untethering of the cord in lumbar spinal lipomas.     

Communicating syringomyelia associated with ventriculoperitoneal shunt malfunction verified with a cerebrospinal fluid dynamic study: case report

History

A 20-year-old male presented with neck pain and motor impairment of the upper extremities because of recurrent syringomyelia caused by ventriculoperitoneal shunt malfunction.

Examination and operation

A computed tomography scan after shuntgraphy demonstrated opacity in the intracranial ventricular system and cervical syrinx with contrast medium, which indicated communication between the fourth ventricle and syrinx. His symptoms resolved immediately after peritoneal catheter replacement, and magnetic resonance images obtained 1 week after surgery showed the complete resolution of hydrocephalus and syringomyelia.

Conclusion

Syringomyelia associated with ventriculoparitoneal shunt malfunction is a well-known complication in myelodysplastic patients; however, this is the first case in which communicating syringomyelia was verified with a cerebrospinal fluid dynamic study. As the present case involved communicating syringomyelia, it could only be resolved by shunt revision surgery.     

Pedicle subtraction osteotomy for adult tethered cord syndrome with lumbar canal stenosis: report of two cases

Tethered cord syndrome with spinal lipoma is the most common form of occult spinal dysraphism. For the symptomatic patients, surgical treatment is recommended; however, there are many patients who have not been encouraged to seek medical attention until adulthood, since their symptoms are not severe enough to interfere with their daily activities. We performed pedicle subtraction osteotomy (PSO) to achieve indirect untethering and neural decompression in two senior patients with tethered cord syndrome, who showed deteriorating neurological condition due to coexisting lumbar canal stenosis. Here we report two patients (aged 56 and 60 years) who underwent PSO of L3 or L4. The pain disappeared and the bladder dysfunction recovered significantly after surgery. Complete bone union and untethering were achieved in both patients. PSO is an alternative surgical technique for senior patients with tethered cord syndrome caused by lumbosacral spinal lipoma, when the syndrome occurs along with lumbar canal stenosis.     

Massive cystic dilatation within a tethered filum terminale causing cauda equina compression and mimicking syringomyelia in a young adult patient

Background

The ventriculus terminalis (VT) is formed during early embryonic development of the spinal cord and can only be identified histologically in both children and adults. Cystic dilatation of the VT can be seen in young children, but it rarely persists through adulthood.

Clinical case

We describe a 27-year-old female with paraparesis secondary to a massive and tethered cystic dilatation of the VT mimicking syringomyelia. Symptoms appearing in early childhood were ignored, probably leading to the much prominent presentation in early adulthood. The preoperative presentation and surgical treatment are discussed in relation to childhood history.

Conclusions

Although extremely rare, symptomatic dilatation of the VT can be seen in young adults, usually with previous manifestations in early childhood. This entity should be considered while treating tethered cord spectrum.     

Recurrent tethered cord: radiological investigation and management

Introduction

Recurrent tethered cord (RTC) is almost the rule after the repair of myelomeningocele and quite frequent after the repair of lipomyelomeningocele, resulting from the adhesions of the placode within a too narrow spinal canal. About one-third of patients with myelomeningocele and 10 % of those with spinal lipoma develop symptomatic RTC, mainly caused by the ischemic–metabolic injuries due to the cord stretching. The goal of this review is to provide information about the pathophysiology, the radiological picture, and the management of RTV according to the pertinent literature and the authors’ experience.

Radiological investigation

The magnetic resonance imaging (MRI) picture is characterized by a low position of the conus and by tethering of the spinal cord to the subcutaneous scar or to the inner surface of the spinal canal. The radiological work-up always includes brain MRI, to rule out other possible causes of late neurological deterioration (as shunt malfunction), and MRI of the whole spinal cord, to detect possible associated lesions (syringomyelia, dermoids, etc.). X-rays and/or computed tomography scan of the spine is required for the assessment of scoliosis or other bony malformations.

Management

The surgical treatment is planned after a multidisciplinary neurological, urological, orthopedic, physiatric, and radiological evaluation. The surgical detethering is carried out cautiously, possibly with electrophysiological intraoperative monitoring. Surgery ensures improvement or stability of the clinical picture in 70–80 % of cases, the remaining 20–30 % of patients needing multiple operations for their recovery. Complications may affect up to one-third of operated patients, being mainly represented by CSF leak, pseudomeningocele, and shunt malfunction.     

Progression of Chiari I malformations while on growth hormone replacement: a report of two cases

Purpose

The effect of growth hormone replacement on Chiari I malformation (CIM) associated with growth hormone deficiency is not clear.

Case reports

Two patients are presented, who were found to have CIM and growth hormone deficiency. While on hormone replacement therapy, both experienced disease progression with development of syringomyelia and required surgical intervention.

Conclusion

Growth hormone replacement for CIM associated with growth hormone deficiency does not uniformly halt or reverse syrinx progression. If a trial of hormone replacement is attempted, patients should be followed closely for progression of syringomyelia or the development of symptoms.     

A bony human tail causing tethered cord syndrome: case report

Introduction

Dorsal cutaneous appendages, sometimes referred to as “human tails,” are considered to be markers of underlying occult spinal dysraphism. Rarely, these tail-like structures can themselves be the cause of tethered cord syndrome. However, to date, a “bony human tail” causing tethered cord has not been reported in the literature. One such rare lesion is being reported.

Discussion

A 2-days-old female child was brought for neurosurgical consultation with a skin-covered bony protuberance in the lower back. Examination of the child did not reveal any neurological deficits. Plain radiographic and CT evaluation showed a dorsal bony protuberance arising from the posterior elements of L1 vertebra. MRI showed the cord to be displaced posteriorly and adherent to the undersurface of the bony tail through a lipoma. During surgery, the bony “tail” was excised, and the cord was untethered with excision of the lipoma, which was tethering the cord to the bony “tail.” When examined 1 year later, the child was developing normally without any focal neurological deficits.

Conclusions

This case is being reported for its rarity and to highlight the hitherto unreported occurrence of “bony human tail” causing tethered cord syndrome.     

Charles Prosper Ollivier d’Angers (1796–1845) and his contributions to defining syringomyelia

Introduction

Distinguished as an anatomist, pathologist, and clinician, the Frenchman Charles Prosper Ollivier d’Angers dedicated his life to accelerating the forefront of neuroscience. At a young age, he explored the diseases and disorders of the spinal cord during a time when clinical neurological investigation scarcely existed. Ollivier d’Angers coined the term “syringomyelia.”

Conclusion

The coinage of the term syringomyelia by d’Angers shed light on the disorder causing more practitioners to investigate the spinal cord and its defects.     

The association of neural axis and craniovertebral junction anomalies with scoliosis in Rubinstein–Taybi syndrome

Object

Rubinstein–Taybi syndrome (RSTS) is a rare condition with characteristic genetic and clinical features. The presence of variable vertebral and neural axis abnormalities has been reported in the literature. We describe the possible association of multiple different spinal anomalies in these patients.

Results

The radiological exams of two RSTS patients (a female and male of 11 and 13 years) have been reviewed. Both patients presented the simultaneous association of craniovertebral junction bony abnormalities (occipito-C1 condyle subluxation and posterior C2–C3 arches fusion), Chiari I malformation, spinal cord syrinx, low-lying conus medullaris, and scoliosis.

Conclusion

An association of different spinal cord anomalies is possible in RSTS patients and has to be investigated with a comprehensive neuroimaging study in order to address the proper treatment and prevent the development of neurologic deficits.     

The tragedy of Syrinx

Introduction

Today, the term “syrinx” is used by many clinicians. Most users of the term, however, are not familiar with the origin of the word. The present paper reviews the history of the word syrinx, which is based in Greek mythology.

Conclusions

Many modern terms have a derivation that is based on a rich history that has often been forgotten. The myth of Syrinx lives on in modern times by the continued use of syrinx in various words such as syringomyelia and syringe.     

Occult tethered cord syndrome: a review

Background

Tethered cord syndrome is a well-defined condition, the management of which is fairly uniform. In contrast, occult tethered cord syndrome is a recently defined entity, where the management is still controversial. The pathophysiology is unclear and may be conceptually incongruent with current understanding of typical tethered cord syndrome. Presentation, investigation, and management of this condition are reviewed, and current understanding is presented.

Purpose

The aim of this study is to review the presentation, pathophysiology, investigation, and management of occult tethered cord syndrome.

Methods

Literature review.

Results

Patients with occult tethered cord syndrome presents predominantly with urologic symptoms. Adult and pediatric patients vary slightly in their presentation with a higher incidence of pain in the former and incontinence in the latter. Operative management for these patients is associated with consistent improvement in urologic function in particular, although surgery is also associated with risk of worsening symptoms. The natural history of untreated patients is unknown.

Conclusions

As occult tethered cord syndrome becomes increasingly recognized, it is important to be aware of the potential benefits of operative intervention for appropriately selected patients. Given that the natural history of this entity remains unknown, a clinical trial is currently underway that may assist in defining the role for operative management in treating this condition.     

The lumbosacral angle does not reflect progressive tethered cord syndrome in children with spinal dysraphism

Purpose  

Our goal was to validate the hypothesis that the lumbosacral angle (LSA) increases in children with spinal dysraphism who present with progressive symptoms and signs of tethered cord syndrome (TCS), and if so, to determine for which different types and/or levels the LSA would be a valid indicator of progressive TCS. Moreover, we studied the influence of surgical untethering and eventual retethering on the LSA.     

Syringomyelia and arachnoiditis.

Five patients with chronic arachnoiditis and syringomyelia were studied. Three patients had early life meningitis and developed symptoms of syringomyelia eight, 21, and 23 years after the acute infection. One patient had a spinal dural thoracic AVM and developed a thoracic syrinx 11 years after spinal subarachnoid haemorrhage and five years after surgery on the AVM. A fifth patient had tuberculous meningitis with transient spinal cord dysfunction followed by development of a lumbar syrinx seven years later. Arachnoiditis can cause syrinx formation by obliterating the spinal vasculature causing ischaemia. Small cystic regions of myelomalacia coalesce to form cavities. In other patients, central cord ischaemia mimics syringomyelia but no cavitation is present. Scar formation with spinal block leads to altered dynamics of cerebrospinal fluid (CSF) flow and contributes to the formation of spinal cord cystic cavities.     

A new technique of placing the peritoneal end of syringoperitoneal shunt

Objective

Syringoperitoneal shunt is an established method of treating syringomyelia. Syringeal end of the shunt is usually inserted to the patient in prone position, and the peritoneal end is inserted in supine position. This necessitates intraoperative repositioning of patient. To avoid this inconvenience, a new technique of placing the peritoneal end through posterior approach is described in this paper.

Methods and materials

In the new technique, peritoneal cavity is approached through posterior route lateral to erector spinae at the level of L3–L4. An infant who presented with sacral meningocele, tethered cord, and syringomyelia was treated using this new technique along with excision of meningocele and de-tethering of the cord.

Results

There were no complications, and the short-term results at 3-year follow-up are encouraging. Theoretical advantages of the new technique include reduced risk of shunt migration and improved cosmesis of surgical scar.

Conclusion

Posterior approach of placing the peritoneal end of syringoperitoneal shunt is technically feasible, and it appears to have advantages over the traditional method of anterior approach.