Acquired Chiari I malformation secondary to spontaneous spinal cerebrospinal fluid leakage and chronic intracranial hypotension syndrome in seven cases

1998 ◽  
Vol 88 (2) ◽  
pp. 237-242 ◽  
Author(s):  
John L. D. Atkinson ◽  
Brian G. Weinshenker ◽  
Gary M. Miller ◽  
David G. Piepgras ◽  
Bahram Mokri

Object. Spontaneous spinal cerebrospinal fluid (CSF) leakage with development of the intracranial hypotension syndrome and acquired Chiari I malformation due to lumbar spinal CSF diversion procedures have both been well described. However, concomitant presentation of both syndromes has rarely been reported. The object of this paper is to present data in seven cases in which both syndromes were present. Three illustrative cases are reported in detail. Methods. The authors describe seven symptomatic cases of spontaneous spinal CSF leakage with chronic intracranial hypotension syndrome in which magnetic resonance (MR) images depicted dural enhancement, brain sagging, loss of CSF cisterns, and acquired Chiari I malformation. Conclusions. This subtype of intracranial hypotension syndrome probably results from chronic spinal drainage of CSF or high-flow CSF shunting and subsequent loss of brain buoyancy that results in brain settling and herniation of hindbrain structures through the foramen magnum. Of 35 cases of spontaneous spinal CSF leakage identified in the authors' practice over the last decade, MR imaging evidence of acquired Chiari I malformation has been shown in seven. Not to be confused with idiopathic Chiari I malformation, ideal therapy requires recognition of the syndrome and treatment directed to the site of the spinal CSF leak.

2001 ◽  
Vol 94 (2) ◽  
pp. 217-223 ◽  
Author(s):  
Jeffrey W. Brennan ◽  
David W. Rowed ◽  
Julian M. Nedzelski ◽  
Joseph M. Chen

Object. The aims of this study were to review the incidence of cerebrospinal fluid (CSF) leakage complicating the removal of acoustic neuroma and to identify factors that influence its occurrence and treatment. Methods. Prospective information on consecutive patients who underwent operation for acoustic neuroma was supplemented by a retrospective review of the medical records in which patients with CSF leaks complicating tumor removal were identified. This paper represents a continuation of a previously published series and thus compiles the authors' continuous experience over the last 24 years of practice. In 624 cases of acoustic neuroma the authors observed an overall incidence of 10.7% for CSF leak. The rate of leakage was significantly lower in the last 9 years compared with the first 15, most likely because of the abandonment of the combined translabyrinthine (TL)—middle fossa exposure. There was no difference in the leakage rate between TL and retrosigmoid (RS) approaches, although there were differences in the site of the leak (wound leaks occurred more frequently after a TL and otorrhea after an RS approach, respectively). Tumor size (maximum extracanalicular diameter) had a significant effect on the leakage rate overall and for RS but not for TL procedures. The majority of leaks ceased with nonsurgical treatments (18% with expectant management and 49% with lumbar CSF drainage). However, TL leaks (especially rhinorrhea) required surgical repair significantly more often than RS leaks. This has not been reported previously. Conclusions. The rate of CSF leakage after TL and RS procedures has remained stable. Factors influencing its occurrence include tumor size but not surgical approach. The TL-related leaks had a significantly higher surgical repair rate than RS-related leaks, an additional factor to consider when choosing an approach. The problem of CSF leakage becomes increasingly important as nonsurgical treatments for acoustic neuroma are developed.


1998 ◽  
Vol 88 (2) ◽  
pp. 243-246 ◽  
Author(s):  
Wouter I. Schievink ◽  
Vittorio M. Morreale ◽  
John L. D. Atkinson ◽  
Fredric B. Meyer ◽  
David G. Piepgras ◽  
...  

Object. Spontaneous spinal cerebrospinal fluid (CSF) leaks are an increasingly recognized cause of intracranial hypotension and may require neurosurgical intervention. In the present report the authors review their experience with the surgical management of spontaneous spinal CSF leaks. Methods. Between 1992 and 1997, 10 patients with spontaneous spinal CSF leaks and intracranial hypotension were treated surgically. The mean age of the seven women and three men was 42.3 years (range 22–61 years). Preoperative imaging showed a single meningeal diverticulum in two patients, a complex of diverticula in one patient, and a focal CSF leak alone in seven patients. Surgical exploration in these seven patients demonstrated meningeal diverticula in one patient; no clear source of CSF leakage could be identified in the remaining six patients. Treatment consisted of ligation of the diverticula or packing of the epidural space with muscle or Gelfoam. Multiple simultaneous spinal CSF leaks were identified in three patients. Conclusions. All patients experienced complete relief of their headaches postoperatively. There has been no recurrence of symptoms in any of the patients during a mean follow-up period of 19 months (range 3–58 months; 16 person-years of cumulative follow up). Complications consisted of transient intracranial hypertension in one patient and leg numbness in another patient. Although the disease is often self-limiting, surgical treatment has an important role in the management of spontaneous spinal CSF leaks. Surgery is effective in eliminating the headaches and the morbidity is generally low. Surgical exploration for a focal CSF leak, as demonstrated on radiographic studies, usually does not reveal a clear source of the leak. Some patients may have multiple simultaneous CSF leaks.


2003 ◽  
Vol 99 (5) ◽  
pp. 840-842 ◽  
Author(s):  
Wouter I. Schievink ◽  
M. Marcel Maya ◽  
Mary Riedinger

Object. Intracranial hypotension due to a spontaneous spinal cerebrospinal fluid (CSF) leak is an increasingly recognized cause of postural headaches, but reliable follow-up data are lacking. The authors undertook a study to determine the risk of a recurrent spontaneous spinal CSF leak. Methods. The patient population consisted of a consecutive group of 18 patients who had been evaluated for consideration of surgical repair of a spontaneous spinal CSF leak. The mean age of the 15 women and three men was 38 years (range 22–55 years). The mean duration of follow up was 36 months (range 6–132 months). The total follow-up time was 654 months. A recurrent spinal CSF leak was defined on the basis of computerized tomography myelography evidence of a CSF leak in a previously visualized but unaffected spinal location. Five patients (28%) developed a recurrent spinal CSF leak; the mean age of these four women and one man was 36 years. A recurrent CSF leak developed in five (38%) of 13 patients who had undergone surgical CSF leak repair, compared with none (0%) of five patients who had been treated non-surgically (p = 0.249). The recurrent leak occurred between 10 and 77 months after the initial CSF leak, but within 2 or 3 months of successful surgical repair of the leak in all patients. Conclusions. Recurrent spontaneous spinal CSF leaks are not rare, and the recent successful repair of such a leak at another site may be an important risk factor.


1996 ◽  
Vol 84 (4) ◽  
pp. 598-605 ◽  
Author(s):  
Wouter I. Schievink ◽  
Fredric B. Meyer ◽  
John L. D. Atkinson ◽  
Bahram Mokri

✓ Spinal cerebrospinal fluid (CSF) leaks are often implicated as the cause of the syndrome of spontaneous intracranial hypotension, but they have rarely been demonstrated radiographically or surgically. The authors reviewed their experience with documented cases of spinal CSF leaks of spontaneous onset in 11 patients including their surgical observations in four of the patients. The mean age of the six women and five men included in the study was 38 years (range 22–51 years). All patients presented with a postural headache; however, most had additional symptoms, including nausea, emesis, sixth cranial-nerve paresis, or local back pain at the level of the CSF leak. All patients underwent indium-111 radionucleotide cisternography or computerized tomographic (CT) myelography. The location of the spontaneous CSF leak was in the cervical spine in two patients, the cervicothoracic junction in three patients, the thoracic spine in five patients, and the lumbar spine in one patient. The false negative rate for radionucleotide cisternography was high (30%). Subdural fluid collections, meningeal enhancement, and downward displacement of the cerebellum, resembling a Chiari I malformation, were commonly found on cranial imaging studies. In most patients, the symptoms resolved in response to supportive measures or an epidural blood patch. Leaking meningeal diverticula were found to be the cause of the CSF leak in four patients who underwent surgery. In three patients these diverticula could be ligated with good result but in one patient an extensive complex of meningeal diverticula was found to be inoperable. Two patients had an unusual body habitus and joint hypermobility, and two other patients had suffered a spontaneous retinal detachment at a young age. In conclusion, spontaneous spinal CSF leaks are uncommon, but they are increasingly recognized as a cause of spontaneous intracranial hypotension. Most spinal CSF leaks are located at the cervicothoracic junction or in the thoracic spine, and they may be associated with meningeal diverticula. The radiographic study of choice is CT myelography. The disease is usually self-limiting, but in selected cases our experience with surgical ligation of leaking meningeal diverticula has been satisfactory. An underlying connective tissue disorder may be present in some patients with a spontaneous spinal CSF leak.


2017 ◽  
Vol 3 (20;3) ◽  
pp. E465-E468
Author(s):  
Wei-Hung Lien

Intracranial hypotension syndrome (IHS) is generally caused by cerebrospinal fluid (CSF) leakage. Complications include bilateral subdural hygroma or haematoma and herniation of the cerebellar tonsils. Epidural blood patch (EBP) therapy is indicated if conservative treatment is ineffective. We reported the case of a 46-year-old man with a history of postural headache and dizziness. The patient was treated with bed rest and daily hydration with 2000 mL of fluid for 2 weeks. However, dizziness and headache did not resolve, and he became drowsy and disoriented with incomprehensible speech. Magnetic resonance imaging demonstrated diffuse dural enhancement on the postcontrast study, sagging of the midbrain, and CSF leakage over right lateral posterior thecal sac at C2 level. We performed EBP at the level of T10-T11. We injected 14 mL of autologous blood slowly in the Trendelenburg position. Within 30 minutes, he became alert and oriented to people, place, and time. We chose thoracic EBP as first line treatment in consideration of the risk of cervical EBP such as spinal cord and nerve root compression or puncture, chemical meningitis. Also we put our patient in Trendelenburg position to make blood travel towards the site of the leak. Untreated IHS may delay the course of resolution and affect the patient’s consciousness. Delivery of EBP via an epidural catheter inserted from the thoracic spine is familiar with most of anesthesiologists. It can be a safe and effective treatment for patients with IHS caused by CSF leak even at C2. Key words: Anaesthetic techniques, regional, thoracic; cerebrospinal fluid leakage; epidural blood patch; heavily T2-weighted magnetic resonance myelography; intracranial hypotension syndrome; Trendelenburg position


2014 ◽  
Vol 17;1 (1;17) ◽  
pp. E89-E94 ◽  
Author(s):  
Dr. Jose De Andres

Background: Spontaneous intracranial hypotension (SIH) and related headache after intrathecal pump implantation is a possibility. Knowing the exact location of the cerebrospinal fluid (CSF) leak is mandatory for managing with interventional therapies. Objectives: Radioisotope cisternography (RICG) is a common procedure in nuclear medicine and may be used as an additional procedure in the differential diagnosis of headaches due to intracranial liquor hypotension syndrome caused by microleakage of CSF. Study Design: We report an imaging case of complex chronic headache in a patient with an implanted drug delivery pump. The symptomatology of the patient was considered typical of spontaneous intracranial hypotension (SIH). Therefore, the initial suspicion was the loss of CSF due to the implanted pump catheter. The negativity of the routine headache test can exclude any leakage of CSF at the site of catheter insertion. Setting: Anesthesia, Critical Care, and Multidisciplinary Pain Management Department, General University General Hospital, Valencia, Spain. Results: Many patients are treated with implanted intrathecal infusion systems. In these patients, any neurological symptoms are readily attributed to the invasive neuroaxial system, especially severe cephalalgia. RICG was helpful in this particular case to make a diagnosis of exclusion of the diagnosis of SIH due to CSF leakage. Conclusion: RICG may be considered as an additional diagnostic technique in patients with chronic pain and implanted spinal infusion devices for whom standard detection procedures have been exhausted. Key words: Intrathecal drug delivery (IDD), chronic headache (CH), radiosotope cisternography (RICG), cerebrospinal fluid leakage, spontaneous intracranial hypotension (SIH)


1981 ◽  
Vol 55 (4) ◽  
pp. 604-609 ◽  
Author(s):  
Keasley Welch ◽  
John Shillito ◽  
Roy Strand ◽  
Edwin G. Fischer ◽  
Ken R. Winston

✓ Caudal herniation of the hindbrain, indistinguishable from the Chiari I deformity, may occur after the establishment of spinal subarachnoid shunts and become symptomatic years after the procedure. Examples are presented and others are cited from the literature. It is proposed that the force responsible for the displacement is the difference in pressure between the cranial and spinal compartments. On the basis of these observations and other considerations as well, a similar process, disproportionate absorption of cerebrospinal fluid from the spinal region, might account for the spontaneous form of the Chiari I deformity.


2004 ◽  
Vol 100 (4) ◽  
pp. 639-644 ◽  
Author(s):  
Wouter I. Schievink ◽  
M. Marcel Maya ◽  
James Tourje

Object. Spontaneous intracranial hypotension due to a spinal cerebrospinal fluid (CSF) leak is an important cause of new daily persistent headaches. Spinal neuroimaging is important in the treatment of these patients, particularly when direct repair of the CSF leak is contemplated. Retrospinal C1–2 fluid collections may be noted on spinal imaging and these are generally believed to correspond to the site of the CSF leak. The authors undertook a study to determine the significance of these C1–2 fluid collections. Methods. The patient population consisted of a consecutive group of 25 patients (18 female and seven male) who were evaluated for surgical repair of a spontaneous spinal CSF leak. The mean age of the 18 patients was 38 years (range 13–72 years). All patients underwent computerized tomography myelography. Three patients (12%) had extensive retrospinal C1–2 fluid collections; the mean age of this woman and these two men was 41 years (range 39–43 years). The actual site of the CSF leak was located at the lower cervical spine in these patients and did not correspond to the site of the retrospinal C1–2 fluid collection. Conclusions. A retrospinal fluid collection at the C1–2 level does not necessarily indicate the site of the CSF leak in patients with spontaneous intracranial hypotension. This is an important consideration in the treatment of these patients because therapy may be inadvertently directed at this site.


Author(s):  
Emma M. H. Slot ◽  
Kirsten M. van Baarsen ◽  
Eelco W. Hoving ◽  
Nicolaas P. A. Zuithoff ◽  
Tristan P. C van Doormaal

Abstract Background Cerebrospinal fluid (CSF) leakage is a common complication after neurosurgical intervention. It is associated with substantial morbidity and increased healthcare costs. The current systematic review and meta-analysis aim to quantify the incidence of cerebrospinal fluid leakage in the pediatric population and identify its risk factors. Methods The authors followed the PRISMA guidelines. The Embase, PubMed, and Cochrane database were searched for studies reporting CSF leakage after intradural cranial surgery in patients up to 18 years old. Meta-analysis of incidences was performed using a generalized linear mixed model. Results Twenty-six articles were included in this systematic review. Data were retrieved of 2929 patients who underwent a total of 3034 intradural cranial surgeries. Surprisingly, only four of the included articles reported their definition of CSF leakage. The overall CSF leakage rate was 4.4% (95% CI 2.6 to 7.3%). The odds of CSF leakage were significantly greater for craniectomy as opposed to craniotomy (OR 4.7, 95% CI 1.7 to 13.4) and infratentorial as opposed to supratentorial surgery (OR 5.9, 95% CI 1.7 to 20.6). The odds of CSF leakage were significantly lower for duraplasty use versus no duraplasty (OR 0.41 95% CI 0.2 to 0.9). Conclusion The overall CSF leakage rate after intradural cranial surgery in the pediatric population is 4.4%. Risk factors are craniectomy and infratentorial surgery. Duraplasty use is negatively associated with CSF leak. We suggest defining a CSF leak as “leakage of CSF through the skin,” as an unambiguous definition is fundamental for future research.


1999 ◽  
Vol 90 (4) ◽  
pp. 651-655 ◽  
Author(s):  
Marc S. Schwartz ◽  
James I. Cohen ◽  
Toby Meltzer ◽  
Michael J. Wheatley ◽  
Sean O. McMenomey ◽  
...  

Object. Reconstruction of the cranial base after resection of complex lesions requires creation of both a vascularized barrier to cerebrospinal fluid (CSF) leakage and tailored filling of operative defects. The authors describe the use of radial forearm microvascular free-flap grafts to reconstruct skull base lesions, to fill small tissue defects, and to provide an excellent barrier against CSF leakage.Methods. Ten patients underwent 11 skull base procedures including placement of microvascular free-flap grafts harvested from the forearm and featuring the radial artery and its accompanying venae comitantes. Operations included six craniofacial, three lateral skull base, and two transoral procedures for various diseases. Excellent results were obtained, with no persistent CSF leaks, no flap failures, and no operative infections. One temporary CSF leak was easily repaired with flap repositioning, and at one flap donor site minor wound breakdown was observed. One patient underwent a second procedure for tumor recurrence and CSF leakage at a site distant from the original operation.Conclusions. Microvascular free tissue transfer reconstruction of skull base defects by using the radial forearm flap provides a safe, reliable, low-morbidity method for reconstructing the skull base and is ideally suited to “low-volume” defects.


Sign in / Sign up

Export Citation Format

Share Document