Chiari decompression outcomes using ligamentum nuchae harvest and duraplasty in pediatric patients with Chiari malformation type I

2018 ◽  
Vol 22 (1) ◽  
pp. 47-51 ◽  
Author(s):  
Michael J. Cools ◽  
Carolyn S. Quinsey ◽  
Scott W. Elton

OBJECTIVEThe choice of graft material for duraplasty in decompressions of Chiari malformations remains a matter of debate. The authors present a detailed technique for harvesting ligamenta nuchae, as well as the clinical and radiographic outcomes of this technique, in a case series.METHODSThe authors conducted a retrospective study evaluating the outcomes of Chiari malformation type I decompression and duraplasty in children aged 0–18 years at a single institution from 2013 to 2016. They collected both intraoperative and postoperative variables and compared them qualitatively to published data.RESULTSDuring the study period, the authors performed 25 Chiari malformation decompressions with ligamentum nuchae graft duraplasties. Of the 25 patients, 10 were females, and the mean age at surgery was 8.6 years (range 13 months to 18 years). The median operative time was 163 minutes (IQR 152–187 minutes), with approximately 10 minutes needed by a resident surgeon to harvest the graft. The mean length of stay was 3 nights (range 2–6 nights), and the mean follow-up was 12.6 months (range 0.5–43.5 months). One patient (4%) developed a CSF leak that was repaired using an oversewing patch. There were no postoperative pseudomeningoceles or infections. Of the 19 patients presenting with a syrinx, imaging showed improvement in 10 (53%) and 8 (42%) had stable syrinx size on imaging. Of 16 patients presenting with a symptomatic Chiari malformation, 14 (87.5%) experienced resolution of symptoms and in 1 (4%) symptoms remained the same. One patient (4%) presented with worsening syrinx and symptoms 1.5 months after initial surgery and underwent repeat decompression.CONCLUSIONSThe authors describe a series of clinical and imaging outcomes of patients who underwent Chiari malformation decompression and duraplasty with a harvested ligamentum nuchae. The rates of postoperative CSF leak are similar to established techniques of autologous and artificial grafts, with similarly successful outcomes. Further study will be needed with larger patient cohorts to more directly compare duraplasty graft outcomes.

2015 ◽  
Vol 15 (2) ◽  
pp. 161-177 ◽  
Author(s):  
Aska Arnautovic ◽  
Bruno Splavski ◽  
Frederick A. Boop ◽  
Kenan I. Arnautovic

OBJECT Chiari malformation Type I (CM-I) is a hindbrain disorder associated with elongation of the cerebellar tonsils, which descend below the foramen magnum into the spinal canal. It occurs in children and adults. Clinical symptoms mainly develop from alterations in CSF flow at the foramen magnum and the common subsequent development of syringomyelia. METHODS The authors reviewed English-language reports of pediatric, adult, and combined (adult and pediatric) surgical series of patients with CM-I published from 1965 through August 31, 2013, to investigate the following: 1) geographical distribution of reports; 2) demographics of patients; 3) follow-up lengths; 4) study durations; 5) spectrum and frequency of surgical techniques; 6) outcomes for neurological status, syrinx, and headache; 7) frequency and scope of complications; 8) mortality rates; and 9) differences between pediatric and adult populations. Research and inclusion criteria were defined, and all series that contained at least 4 cases and all publications with sufficient data for analysis were included. RESULTS The authors identified 145 operative series of patients with CM-I, primarily from the United States and Europe, and divided patient ages into 1 of 3 categories: adult (> 18 years of age; 27% of the cases), pediatric (≤ 18 years of age; 30%), or unknown (43%). Most series (76%) were published in the previous 21 years. The median number of patients in the series was 31. The mean duration of the studies was 10 years, and the mean follow-up time was 43 months. The peak ages of presentation in the pediatric studies were 8 years, followed by 9 years, and in the adult series, 41 years, followed by 46 years. The incidence of syringomyelia was 65%. Most of the studies (99%) reported the use of posterior fossa/foramen magnum decompression. In 92%, the dura was opened, and in 65% of these cases, the arachnoid was opened and dissected; tonsillar resection was performed in 27% of these patients. Postoperatively, syringomyelia improved or resolved in 78% of the patients. Most series (80%) reported postoperative neurological outcomes as follows: 75% improved, 17% showed no change, and 9% experienced worsening. Postoperative headaches improved or resolved in 81% of the patients, with a statistical difference in favor of the pediatric series. Postoperative complications were reported for 41% of the series, most commonly with CSF leak, pseudomeningocele, aseptic meningitis, wound infection, meningitis, and neurological deficit, with a mean complication rate of 4.5%. Complications were reported for 37% of pediatric, 20% of adult, and 43% of combined series. Mortality was reported for 11% of the series. No difference in mortality rates was seen between the pediatric and adult series. CONCLUSIONS Before undergoing surgical treatment for CM-I, symptomatic patients and their families should be given clear information about the success of treatment and potential complications. Furthermore, surgeons may benefit from comparing published data with their own. In the future, operative CM-I reports should provide all details of each case for the purpose of comparison.


2018 ◽  
Vol 22 (3) ◽  
pp. 297-305 ◽  
Author(s):  
Brian J. Dlouhy ◽  
Arnold H. Menezes

OBJECTTechniques for combined extradural and intradural decompression with expansile duraplasty for Chiari malformation type I (CM-I) have been well described, with various allogenic and autologous materials used for duraplasty. However, the approach and surgical technique used for duraplasty in our treatment of CM-I and developed by the senior author in the 1990s has not been described.METHODSA prospective database was initiated in March 2003 to denote the use of cervical fascia for duraplasty and incorporate an ongoing detailed record of complications during the surgical treatment of children and adults with CM-I with and without syringomyelia. A total of 389 surgeries for CM-I were performed on 379 patients from March 2003 to June 2016. A total of 123 posterior procedures were performed on 123 patients in which both a posterior fossa extradural and intradural decompression with duraplasty (extra-intradural) was performed. In this paper the authors describe the surgical technique for harvesting and using cervical fascia for duraplasty in the surgical treatment of CM-I and analyze and discuss complications from a prospective database spanning 2003–2016.RESULTSThe authors found that cervical fascia can be harvested in patients of all ages (2–61 years old) without difficulty, and it provides a good substitute for dura in creating an expansile duraplasty in patients with CM-I. Cervical fascia is an elastic-like material with a consistency that allows for a strong watertight closure. Harvesting the cervical fascia graft does not require any further extension of the incision superiorly or inferiorly to obtain the graft. Complications were uncommon in this study of 123 children and adults. The risk of any type of complication (aseptic meningitis, CSF leak, pseudomeningocele, infection, development of hydrocephalus, and need for ventriculoperitoneal shunt) for the 78 patients in the pediatric age group was 0%. The risk of complication in the adult group was 6.7% (1 patient with aseptic meningitis and 2 patients with CSF leak).CONCLUSIONSAutologous cervical fascia is easy to obtain in patients of all ages and provides an effective material for duraplasty in the treatment of CM-I. Complications from the combination of both an extradural and intradural decompression with autologous cervical fascia duraplasty are uncommon.


2011 ◽  
Vol 8 (2) ◽  
pp. 177-183 ◽  
Author(s):  
Stephen R. Parker ◽  
Peggy Harris ◽  
Thomas J. Cummings ◽  
Timothy George ◽  
Herbert Fuchs ◽  
...  

Object Posterior fossa decompression with duraplasty for Chiari malformation Type I (CM-I) is a common pediatric neurosurgery procedure. Published series report a complication rate ranging from 3% to 40% for this procedure. Historically, many dural substitutes have been used, including bovine grafts, human cadaveric pericardium, synthetic dura, and autologous pericranium. The authors hypothesized that a recently observed increase in complications was dependent on the graft used. Methods Between January 2004 and January 2008, 114 consecutive patients ≤ 18 years old underwent primary CM-I decompression using duraplasty. Records were retrospectively reviewed for short- and intermediate-term complications and operative technique, focusing on the choice of duraplasty graft with or without application of a tissue sealant. Results The average age of the patients was 8.6 years. The dural graft used was variable: 15 were treated with cadaveric pericardium, 12 with Durepair, and 87 with EnDura. Tisseel was used in 75 patients, DuraSeal in 12, and no tissue sealant was used in 27 patients. The overall complication rate was 21.1%. The most common complications included aseptic meningitis, symptomatic pseudomeningocele, or a CSF leak requiring reoperation. The overall complication rates were as follows: cadaveric pericardium 26.7%, Durepair 41.7%, and EnDura 17.2%; reoperation rates were 13%, 25%, and 8.1%, respectively. Prior to adopting a different graft product, the overall complication rate was 18.1%; following the change the rate increased to 35%. Complication rates for tissue sealants were 14.8% for no sealant, 18.7% for Tisseel, and 50% for DuraSeal. Nine patients were treated with the combination of Durepair and DuraSeal and this subgroup had a 56% complication rate. Conclusions Complication rates after CM-I decompression may be dependent on the dural graft with or without the addition of tissue sealant. The complication rate at the authors' institution approximately doubled following the adoption of a different graft product. Tissue sealants used in combination with a dural substitute to augment a duraplasty may increase the risk of aseptic meningitis and/or CSF leak. The mechanism of the apparent increased inflammation with this combination remains under investigation.


2007 ◽  
Vol 6 (3) ◽  
pp. 216-221 ◽  
Author(s):  
Atsushi Ono ◽  
Futoshi Suetsuna ◽  
Kazumasa Ueyama ◽  
Toru Yokoyama ◽  
Shuichi Aburakawa ◽  
...  

Object The clinical characteristics of pediatric scoliosis associated with syringomyelia have been reported in previous studies, but scoliosis associated with syringomyelia in adults is rarely treated, and there is a paucity of detailed studies. In the present study of adult syringomyelia associated with Chiari malformation Type I, the authors investigated the relationships among the syrinx, scoliosis, and neurological data. Methods The population was composed of 27 patients (≥ 20 years of age) who underwent foramen magnum decompression for the treatment of syringomyelia. The patients were divided into two groups: those with scoliosis of 10° or more (Group A) and those without scoliosis (Group B). The authors assessed the length of the syrinx, duration of morbidity, and clinical status before and after surgery based on the Japanese Orthopaedic Association (JOA) Scale. There were 15 cases in Group A and 12 in Group B. The mean length of the syrinx was 12.8 vertebral bodies (VBs) in Group A and 7.2 VBs in Group B. The mean duration of morbidity was 14.2 years in Group A and 6.8 years in Group B. The mean preoperative JOA score was 10.1 in Group A and 14.4 in Group B, whereas the mean postoperative JOA scores were 11.9 and 15.8, respectively. There were significant differences between Groups A and B in length of the syrinx, duration of morbidity, and pre- and postoperative JOA scores. Conclusions In patients with syringomyelia and scoliosis the syringes spanned a greater number of VBs, the duration of morbidity was greater, neurological dysfunction was more severe, and surgical results were poorer. Scoliosis could be a predicting factor of the prognosis in patients with syringomyelia and Chiari malformation Type I.


2011 ◽  
Vol 31 (3) ◽  
pp. E1 ◽  
Author(s):  
Benjamin D. Schanker ◽  
Brian P. Walcott ◽  
Brian V. Nahed ◽  
Kristopher T. Kahle ◽  
Yan Michael Li ◽  
...  

Chiari malformations (Types I–IV) are abnormalities of the posterior fossa that affect the cerebellum, brainstem, and the spinal cord with prevalence rates of 0.1%–0.5%. Case reports of familial aggregation of Chiari malformation, twin studies, cosegregation of Chiari malformation with known genetic conditions, and recent gene and genome-wide association studies provide strong evidence of the genetic underpinnings of familial Chiari malformation. The authors report on a series of 3 family pairs with Chiari malformation Type I: 2 mother-daughter pairs and 1 father-daughter pair. The specific genetic causes of familial Chiari malformation have yet to be fully elucidated. The authors review the literature and discuss several candidate genes. Recent advances in the understanding of the genetic influences and pathogenesis of familial Chiari malformation are expected to improve management of affected patients and monitoring of at-risk family members.


2021 ◽  
pp. 1-10
Author(s):  
Michael M. McDowell ◽  
Michael Chiang ◽  
Hussam Abou-Al-Shaar ◽  
Georgios A. Zenonos ◽  
Eric W. Wang ◽  
...  

<b><i>Introduction:</i></b> Endoscopic endonasal surgery (EES) has been slower to gain popularity in early childhood due to anatomical challenges. We sought to describe the safety and efficacy of EES in early childhood. <b><i>Methods:</i></b> All patients younger than 7 years who underwent EES at a large Cranial Base Center from 2002 to 2019 were reviewed as a retrospective cohort study. <b><i>Results:</i></b> Thirty-six patients underwent EES before the age of 7 years. Four patients had two-stage EES. Two patients required combined transcranial and endonasal approaches. The mean age at the time of initial surgery was 4 years (range: 1–6). Twenty patients were male, and 16 were female. Of 21 tumors intended for resection, 11 patients had gross total resections, and 10 had near total (&#x3e;95% tumor removed) resections. Nine patients (43%) had recurrences, of which 6 were craniopharyngiomas (<i>p</i> = 0.01). There was no difference in recurrence rates based on the degree of resection (<i>p</i> = 0.67). Three cerebrospinal fluid (CSF) leaks occurred following primary EES (8%). Following an increase in nasoseptal flap usage (31–52%) and CSF diversion (15–39%) in 2008, there was only one CSF leak out of 23 patients (4 vs. 15%; <i>p</i> = 0.54). Postoperatively, 1 patient developed a permanent new cranial neuropathy, and 1 patient developed a permanent visual field cut. Six patients developed permanent postoperative panhypopituitarism, of which all were craniopharyngiomas (<i>p</i> &#x3c; 0.001). The mean follow-up was 64 months. <b><i>Conclusions:</i></b> Early childhood EES is both safe and technically feasible for a variety of pathologies.


Author(s):  
Elizabeth N Alford ◽  
Travis J Atchley ◽  
Tofey J Leon ◽  
Nicholas M. B Laskay ◽  
Anastasia A Arynchyna ◽  
...  

OBJECTIVE In Chiari malformation type I (CM-I), a variety of imaging findings have been purported to be important; however, results have been inconclusive, inconsistent, or not replicated in independent studies. The purpose of this study was to report imaging characteristics for a large cohort of patients with CM-I and identify the imaging findings associated with surgical decompression. METHODS Patients were identified using ICD-9 codes for CM-I for the period from 1996 to 2017. After review of the medical records, patients were excluded if they 1) did not have a diagnosis of CM-I, 2) were not evaluated by a neurosurgeon, or 3) did not have available preoperative MRI. Retrospective chart review was performed to collect demographic and clinical data. Imaging parameters were measured according to the Chiari I Malformation Common Data Elements. RESULTS A total of 731 patients were included for analysis, having a mean follow-up duration of 25.5 months. The mean age at presentation was 8.5 years. The mean tonsil position was 11.4 mm below the foramen magnum, and 62.8% of patients had a pegged tonsil shape. Two hundred patients (27.4%) underwent surgery for life-dominating tussive headache, lower cranial nerve dysfunction, syrinx, and/or brainstem dysfunction. Surgical treatment was associated with a syrinx (OR 20.4, 95% CI 12.3–33.3, p < 0.0001), CM-1.5 (OR 1.797, 95% CI 1.08–2.98, p = 0.023), lower tonsil position (OR 1.130, 95% CI 1.08–1.18, p < 0.0001), and congenital fusion of cervical vertebrae (OR 5.473, 95% CI 1.08–27.8, p = 0.040). Among patients with benign CM-I, tonsil position was statistically significantly associated with future surgery. CONCLUSIONS Comprehensive imaging characteristics for a large cohort of patients with CM-I are reported. Analysis showed that a lower tonsillar position, a syrinx, and CM-1.5 were associated with undergoing posterior fossa decompression. This study demonstrates the importance of considering imaging findings in the context of patient symptomatology.


2015 ◽  
Vol 66 (1) ◽  
pp. 28-35 ◽  
Author(s):  
Gloria Guerra Jiménez ◽  
Ángel Mazón Gutiérrez ◽  
Enrique Marco de Lucas ◽  
Natalia Valle San Román ◽  
Rubén Martín Laez ◽  
...  

2009 ◽  
Vol 111 (5) ◽  
pp. 1046-1052 ◽  
Author(s):  
Rémy Noudel ◽  
Nicolas Jovenin ◽  
Cristophe Eap ◽  
Bernard Scherpereel ◽  
Laurent Pierot ◽  
...  

Object The chronic tonsillar herniation defining Chiari malformation Type I (CMI) is thought to result from overcrowding of a normally developing hindbrain within a congenitally small posterior cranial fossa (PCF) due to occipital hypoplasia. The goals in the present study were to authenticate the cranioencephalic disproportion in a group of patients with CMI and to discuss new developmental aspects according to which part of the occipital bone was underdeveloped. Methods The authors retrospectively examined a group of 17 patients with CMI. Measurements of osteotentorial and neural structures of the PCF were made on MR images of the brain. The results were compared with findings in 30 healthy controls by using the Mann-Whitney U-test. Results Dimensions of the neural structures did not differ between the 2 groups of patients. The mean length of the basiocciput was significantly shorter in the CMI group (19.4 mm) compared with the control group (25.7 mm; p = 0.0003). The mean diameter of the foramen magnum was larger in the CMI group, but this difference was not statistically significant. The dimensions of the supraocciput and the mean angle of the cerebellar tentorium were identical in the 2 groups. Conclusions Data in this study support the idea that occipital hypoplasia is the main cause of overcrowding within the PCF. Basioccipital shortness is a cardinal feature of the resultant shallow PCF and could proceed from a congenital disorder of the cephalic mesoderm of the parachordal plate or occur later in the infancy because of premature stenosis of the sphenooccipital synchondrosis.


2015 ◽  
Vol 23 (6) ◽  
pp. 701-706 ◽  
Author(s):  
David A. Besachio ◽  
Ziyad Khaleel ◽  
Lubdha M. Shah

OBJECT Posterior odontoid process inclination has been demonstrated as a factor associated with Chiari malformation Type I (CM-I) in the pediatric population; however, no studies to date have examined this measurement in the adult CM-I population. The purpose of this study was to evaluate craniocervical junction (CCJ) measurements in adult CM-I versus a control group. METHODS The odontoid retroflexion, odontoid retroversion, odontoid height, posterior basion to C-2 line measured to the dural margin (pB-C2 line), posterior basion to C-2 line measured to the dorsal odontoid cortical margin (pB-C2* line), and clivus-canal angle measurements were retrospectively analyzed in adult patients with CM-I using MRI. These measurements were compared with normative values established from CT scans of the cervical spine in adults without CM-I. RESULTS A statistically significant difference was found between 55 adults with CM-I and 150 sex-matched controls (125 used for analysis) in the mean clivus-canal angle and the mean pB-C2 line. CONCLUSIONS These data suggest that there are sex-specific differences with respect to measurements at the CCJ between men and women, with women showing a more posteriorly inclined odontoid process. There were also differences between the CM-I and control groups: a more acute clivus-canal angle was associated with CM-I in the adult population. These CCJ findings could have an influence on presurgical planning.


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