Transverse Microincisions of the Outer Layer of the Dura mater Combined with Foramen Magnum Decompression as Treatment for Syringomyelia with Chiari I Malformation

Summary Numerous surgical procedures have been proposed for treatment of syringomyelia associated with Chiari I malformation, but the optimal treatment has not yet been uniformly standardised. The main aim of the surgical treatment of syringomyelia/Chiari I complex is directed toward restoration of physiological cerebrospinal fluid dynamic at the craniovertebral junction. We report the surgical results of eight patients, affected by syringomyelia and Chiari I malformation, age range from 18 to 62 years, treated by bony foramen magnum decompression combined with transverse microincisions of the outer layer of the dura mater. In an average postoperative follow-up period of two years neurological symptoms and signs improved in seven patients. Postoperative Magnetic Resonance showed a decrease in size of the syrinx in seven patients. These results suggest that foramen magnum decompression combined with transverse microincisions of the outer layer of the dura 1) is an effective and safe treatment option for syringomyelia and Chiari I malformation, 2) corrects the circulatory disturbances of cerebrospinal fluid dynamic, 3) leads to a decrease in size of the syrinx and to a significant improvement in neurological signs and symptoms, 4) avoids complications of intradural approaches and syringosubarachnoid shunting.     

Hiccups attributable to syringobulbia and/or syringomyelia associated with a Chiari I malformation: case report

OBJECTIVE AND IMPORTANCE: Approximately 20 to 50% of patients with syringomyelia associated with Chiari malformations exhibit cranial nerve or cerebellar symptoms. However, hiccups represent a rare clinical manifestation of this disorder. We report a case of intractable hiccups resulting from syringobulbia associated with a Chiari I malformation, which was successfully treated with foramen magnum decompression. CLINICAL PRESENTATION: We report the case of a patient who presented with syringomyelia and syringobulbia associated with a Chiari I malformation, manifested as intractable hiccups and neurological deficits. Magnetic resonance imaging scans demonstrated syringobulbia in the dorsal medullary region and a large cervical syrinx from C2 to C6-C7, associated with a Chiari I malformation. INTERVENTION: Foramen magnum decompression and a C1 laminectomy were performed. One month later, the intractable hiccups disappeared and the neurological symptoms demonstrated improvement. CONCLUSION: Postoperative magnetic resonance imaging scans demonstrated enlargement of the subarachnoid space in the posterior fossa and disappearance of the syringobulbia. There has been no recurrence of intractable hiccups and syringobulbia in 6 months after surgery. Magnetic resonance imaging of the brainstem is an important diagnostic procedure for intractable hiccups, because syringobulbia associated with a Chiari malformation represents a surgically treatable disorder, although the incidence is low.     

Chiari malformation (type I) associated with bilateral abducens nerve palsy: case report

There are still many questions concerning the pathology and therapeutic strategy for Chiari malformation. We recently encountered a case of Chiari malformation associated with only bilateral abducens nerve palsy which was successfully treated by foramen magnum decompression. The pathology and mechanisms of Chiari malformation (type I) were evaluated in this case. The patient was a 12-year-old girl who had visited our institute 1 month previously complaining of diplopia. She had no particular past or family history and her general appearance was also normal. Bilateral abducens nerve palsy was the only neurological deficit. Her cerebrospinal pressure was 9 cm H2O. Neuroradiological findings revealed basilar impression, C4-6 syringomyelia and Chiari malformation (type I), but no hydrocephalus or meningocele. Foramen magnum decompression with lateral extension, upper cervical laminectomy and open duraplasty were performed. The position of both eyes recovered fully shortly after surgery, and diplopia occurred only when the patient looked sideways. Eye movements also returned completely to normal 3 months after surgery. MRI showed no syringomyelia 4 months after surgery, the upward shift of the pendulous portion of the cerebellar tonsil, and the expansion of the subarachnoidal space of the pons-oblongata junction. In conclusion, it was thought that the mechanism of bilateral abducens nerve palsy in this case was due to the strangulation and the downward traction of the pons-oblongata junction. The nerve was relieved from this traction by foramen magnum decompression.     

Spontaneous resolution of Chiari I malformation and syringomyelia: case report and review of the literature

OBJECTIVE AND IMPORTANCE: Indications for surgery and the surgical technique of foramen magnum decompression for patients with Chiari I malformation and syringomyelia are controversial issues. This case report supports the view that observation may be adequate for patients without progressive symptoms or with mild clinical symptoms. CLINICAL PRESENTATION: A 37-year-old woman presented with a 3-month history of burning dysesthesias and hypesthesia in her right arm. A neurological examination revealed hypesthesia in the right trigeminal distribution. A magnetic resonance imaging scan revealed a Chiari I malformation with syringomyelia between C2 and T2. No hydrocephalus was observed. CLINICAL COURSE: Because the patient's symptoms regressed spontaneously, surgery was not performed. Thirty-two months after her initial examination, the patient was asymptomatic. A second magnetic resonance imaging scan was obtained, which demonstrated complete spontaneous resolution of the Chiari I malformation and syringomyelia. CONCLUSION: We attribute the regression of the patient's symptoms to spontaneous recanalization of cerebrospinal fluid pathways at the foramen magnum, which most likely was due to rupture of the arachnoid membranes that had obstructed cerebrospinal fluid flow.     

Circumferential decompression of the foramen magnum for the treatment of syringomyelia associated with basilar invagination

Posterior fossa decompression utilizing suboccipital craniectomy and duraplasty remains the standard surgical treatment for Chiari-associated syringomyelia. In the presence of basilar invagination, anterior decompression, typically transoral odontoidectomy, or posterior decompression may be performed. We report two cases in which anterior and posterior (circumferential) decompression of the foramen magnum was used to treat cervical syringomyelia successfully. These cases demonstrate that circumferential decompression of the foramen magnum may be necessary in some cases of cervical syringomyelia associated with basilar invagination and Chiari malformation.     

Chiari malformation (type 1) with regeneration of removed C1 lamina after foramen magnum decompression: a case report

We present the case of a 20-year-old female who underwent foramen magnum decompression and laminectomy of C1-C3 as well as incision of only the outer layer of the dura, for her symptomatic Chiari malformation (type 1) with syringomyelia, at the age of 9 years. She required additional surgery with removal of regenerated bone and dural plasty for the remaining and recurrent stenosis. The remaining stenosis may have been caused by the use of a technique without dural plasty and insufficient decompression of the bony structure. Further, the regeneration of C1 lamina could have been responsible for the recurrent stenosis. Her symptoms, which included gait disturbance, hypesthesia, excessive sweating, and nocturnal enuresis, manifested themselves when she was at the age of 2 years and progressed since that time. These symptoms didn't improve after the first surgery. However, after the second surgery, the gait disturbance and nocturnal enuresis were partially resolved. It is rare for new bone generation to necessitate reoperation. However, it should be noticed that careful follow-up is important after decompressive surgery for Chiari malformation in young patients.     

Malformations of the craniocervical junction (chiari type I and syringomyelia: classification,diagnosis and treatment)

Chiari disease (or malformation) is in general a congenital condition characterized by an anatomic defect of the base of the skull, in which the cerebellum and brain stem herniate through the foramen magnum into the cervical spinal canal. The onset of Chiari syndrome symptoms usually occurs in the second or third decade (age 25 to 45 years). Symptoms may vary between periods of exacerbation and remission. The diagnosis of Chiari type I malformation in patients with or without symptoms is established with neuroimaging techniques. The most effective therapy for patients with Chiari type I malformation/syringomyelia is surgical decompression of the foramen magnum, however there are non-surgical therapy to relieve neurophatic pain: either pharmacological and non-pharmacological. Pharmacological therapy use drugs that act on different components of pain. Non-pharmacological therapies are primarly based on spinal or peripheral electrical stimulation.     

Neuraxial analgesia during labor in a patient with Arnold-Chiari type I malformation and syringomyelia

Chiari type I malformation is a caudal displacement of the cerebellum with tonsillar herniation through the foramen magnum, frequently associated with syringomyelia, a syndrome characterized by cyst-like cavities in the spinal cord; each of the conditions leads to characteristic neurologic abnormalities. Pregnant patients with these types of malformation are considered to have an increased risk of brainstem compression and/or progression of the disease during labor. We present the case of a patient in labor with a diagnosis of syringomyelia associated with Chiari type I malformation and describe the anesthetic management. The patient revealed during labor that she had syringomyelia associated with Chiari type I malformation, after having made no mention of it in previous history taking. Finally, we review the pathology observed in these patients.     

Syringo-subarachnoid shunt for syringomyelia associated with Chiari malformation (type 1)

Summary The authors report the surgical results of 28 patients with syringomyelia associated with Chiari malformation (type 1). 28 patients underwent 34 operative procedures. Syringo-subarachnoid shunt was performed in 28 patients, foramen magnum decompression with syringo-subarachnoid shunt in three, ventriculo-peritoneal shunt in one, terminal syringostomy in one, and foramen magnum decompression with terminal syringostomy in one. In an average postoperative follow-up period of 3 years and 9 months ranging from one year to 7 years and one month, neurological symptoms and signs improved in 24 out of 28 patients (82%). Some improvement was noted in sensory deficit and motor weakness. In 3 patients, the symptoms did not change. In 3 patients whose symptoms were unchanged, preoperative studies demonstrated atrophy of the spinal cord, in which irreversible changes were shown. The symptoms deteriorated in one patient. In one patient whose symptoms increased 3 months after syringo-subarachnoid shunt, shunt insufficiency due to postoperative adhesive arachnoiditis was responsible for neurological deterioration.The authors propose that syringo-subarachnoid shunt is effective as a surgical procedure for syringomyelia associated with Chiari malformation (type 1) if the patient does not have symptoms due to Chiari malformation or has only mild signs and symptoms which do not require foramen magnum decompression, such as nystagmus or atrophy of sternocleidomastoid muscle.     

Factors contributing to improvement of syringomyelia after foramen magnum decompression for Chiari type I malformation

Background

Although various surgical approaches have been proposed for treating syringomyelia associated with Chiari type I malformation, a standard method has yet to be established. we prospectively investigated the results of our surgical method: foramen magnum decompression combined with C1 laminectomy and excision of the outer layer of the dura mater.

Methods

Twenty patients underwent surgery between 2000 and 2010 at our hospital. After surgery, the size of the syrinx decreased in 11 patients (decreased group) but remained unchanged in nine patients (unchanged group). The following parameters were compared: age at the time of surgery, duration of morbidity, improvement of preoperative symptoms, morphological type and length of the syrinx, presence or absence of scoliosis, cervical alignment, basal and clivo-axial angles, and postoperative subarachnoid space at the foramen magnum level.

Results

Preoperative symptoms improved in all patients in the decreased group but in only one patient in the unchanged group. The average duration of morbidity was significantly shorter in the decreased group. Morphological examination revealed that the size of all central-type syrinxes decreased after surgery, whereas in all cases of deviated-type syrinx, size was unchanged. The average length of preoperative syrinx was significantly shorter in the decreased group. The postoperative subarachnoid space at the foramen magnum was enlarged in the entire decreased group, whereas residual narrowing of the space was observed in 44 % of patients in the unchanged group. No significant intergroup differences were observed in the other factors.

Conclusions

In patients with syringomyelia, a longer and deviated type of syrinx, a longer duration of morbidity, and postoperative residual narrowing of the subarachnoid space are associated with a poor prognosis after the surgical procedure. The pathogenesis of syringomyelia is inconsistent, and the choice of surgical technique for each pathological condition is important.     

Foramen magnum decompression for syringomyelia associated with basilar impression and Chiari I malformation--report of three cases.

Anterior or posterior decompression of the foramen magnum was performed in three patients with syringomyelia associated with basilar impression and Chiari I malformation. The operative results were evaluated using the pre- and postoperative magnetic resonance (MR) images. Two patients with combined anterior and posterior cervicomedullary compression due to basilar impression and tonsillar descent received suboccipital craniectomy, upper cervical laminectomy, and dural plasty without any intradural manipulations via the posterior approach. One patient with prominent anterior cervicomedullary compression due to basilar impression and a sharp clivoaxial angle was operated on by the transoral anterior approach. Postoperatively, all patients showed a sustained shrinkage of the syrinx and rounding of the flattened cerebellar tonsils. Two patients showed upward movement of the herniated tonsils. All patients had improved symptoms during 2-4 years follow-up. Treatment of syringomyelia associated with basilar impression and Chiari I malformation requires more efficient decompressive procedures at the foramen magnum based on neurological and MR findings.     

Syringobulbia Caused by Delayed Postoperative Tethering of the Cervical Spinal Cord – Delayed Complication of Foramen Magnum Decompression for Chiari Malformation

Summary  Postoperative tethering of the high cervical spinal cord is a rare cause of neurological deterioration after foramen magnum decompression (FMD) with duraplasty for Chiari type I malformation. A review of the literature revealed that only 5 cases have been reported. This entity is not widely known to occur as a complication of the common surgical procedure for Chiari type I malformation. A 17-year-old boy experienced rapidly progressive neurological deterioration over a 3-month period. FMD and duraplasty with lyophilized cadaver dura had been performed 8 years previously. Follow-up MR images showed that the cerebrospinal fluid (CSF) space dorsal to the cord was gradually disappearing and that syringobulbia had developed. Opening the dura mater of the posterior fossa revealed dense fibrous scarring, arachnoid thickening over the cervicomedullary area, and tethering the cord to the dura from the medulla to C2. The adhesions were dissected free, and the tethering was released. A syringosubarachnoid (SS) shunt was inserted and duraplasty was performed with an expended polytetrafluoroethylene sheet (Gore-Tex). Postoperative MR images demonstrated that the syringobulbia had completely collapsed and that a dorsal CSF space was present. Follow-up MR images provided significant information on the cervical spinal cord tethering after FMD with duraplasty for Chiari malformation. We encourage sharp surgical detethering and duraplasty with Gore-Tex to avoid retethering. Early recognition and treatment of this unusual but important complication are emphasized.     

Surgical treatment of syringomyelia. Selection of surgical procedures

The surgical approach to syringomyelia is controversial. In this study, the authors evaluated the results of various operative procedures applied in 31 patients with syringomyelia. Syringomyelia was associated with Chiari malformation in 17 cases, with spinal adhesive arachnoiditis in nine, with trauma in three, and with epidural arachnoid cyst in one. One case was idiopathic. A total of 38 operations were performed. Syringosubarachnoid shunting was applied in 17 patients, syringoperitoneal shunting in 11, terminal syringostomy in three, ventriculoperitoneal shunting in three, lumboperitoneal shunting in two, foramen magnum decompression (suboccipital craniectomy plus upper cervical laminectomy) and terminal syringostomy in one, and foramen magnum decompression with syringosubarachnoid shunting in one. The postoperative follow-up period ranged from 2 to 55 months (average, 26 months). Of the 31 patients, 23 showed neurological improvement, five were unchanged, and three deteriorated. Among the last three, one patient with Chiari malformation developed shunt malfunction due to arachnoiditis after syringosubarachnoid shunting. In two patients with syringomyelia secondary to adhesive arachnoiditis, the spinal cord was damaged by extensive separation of the arachnoid membrane at surgery. On the basis of the results in these 31 cases, the authors conclude that syringosubarachnoid shunting is effective for syringomyelia associated with Chiari malformation if syringomyelia is responsible for the clinical symptoms. Post-traumatic syringomyelia and syringomyelia secondary to adhesive arachnoiditis should be treated by syringoperitoneal shunting. If hydrocephalus is present, ventriculoperitoneal shunting is indicated. Finally, terminal syringostomy is no more effective than syringosubarachnoid or syringoperitoneal shunting.     

Radiological outcome after surgical treatment of syringomyelia-Chiari I complex in adults: a systematic review and meta-analysis

Neurosurgical Review - Foramen magnum decompression (FMD) is widely accepted as the standard treatment for syringomyelia associated with Chiari type I malformation (CMI). Despite extensive clinical...     

The surgical treatment of Chiari I malformation

Summary A retrospective study was undertaken on 133 patients with a Chiari I malformation treated within the last 16 years at the Departments of Neurosurgery at the Nordstadt Hospital Hannover, Germany, and the University of California, Los Angeles, U.S.A. Ninety-seven patients presented with symptoms related to accompanying syringomyelia and 4 with associated syringobulbia. They underwent 149 surgical procedures and were followed for a mean of 39±52 months. A decompression at the foramen magnum was performed in 124 patients, while 22 of those with syringomyelia were treated by shunting (7 syringosubarachnoid shunts, 15 syringoperitoneal or -pleural shunts), and 3 by ventriculoperitoneal shunts for hydrocephalus.Except for ventriculoperitoneal shunting, at least a short-term decrease in size of an associated syrinx was observed for all procedures in the majority of cases. However, no long-term benefit was observed for syrinx shunting operations. The best clinical longterm results were obtained with decompression of the foramen magnum in patients with (86% free of a clinical recurrence) and without syringomyelia (77% free of a clinical recurrence). We advise against syrinx shunting, a large craniectomy, and obex plugging which are associated with higher recurrence rates. Instead, surgery should consist of a small craniectomy, opening of the dura, archnoid dissection to establish normal cerebrospinal fluid (CSF) outflow from the 4th ventricle, and a fascia lata durai graft.     

Surgical outcomes of foramen magnum decompression for syringomyelia associated with Chiari I malformation: relation between the location of the syrinx and body pain

Background  

There have been few reports about the relation between the morphology of syrinxes and body pain in syringomyelia associated with Chiari I malformation. To investigate this phenomenon, the relation between the location of the syrinx and body pain before and after foramen magnum decompression (FMD) were evaluated.     

Chiari I malformation accompanied by assimilation of the atlas, Klippel-Feil syndrome, and syringomyelia: case report

BACKGROUND: Chiari I malformation, accompanied by superposed bony anomaly of the craniovertebral junction, is comparatively rare. We report a case of Chiari I malformation accompanied by assimilation of the atlas, Klippel-Feil syndrome, and syringomyelia. CASE DESCRIPTION: The patient was a 61-year-old woman demonstrating numbness of the extremities, sensory impairment, muscular weakness, and tendon hyper-reflexia. X-ray images and CT scans demonstrated assimilation of the atlas to the occipital bone, C2 and C3 fusion, abnormal passage of the vertebral arteries, and an anomalous bony mass on the right lateral mass of the atlas protruding into the spinal column. The odontoid process was also deviated to the left. Magnetic resonance images demonstrated bilateral descent of the cerebellar tonsils and syringomyelia extending from C6 to T8. Computed tomographic scans with the head rotated to the right demonstrated increased narrowing of the vertebral column caused by the right lateral mass of the atlas, and MR images confirmed exaggerated deformation of the spinal cord at the same region. This deformation manifested no neurologic symptoms, and we therefore performed foramen magnum decompression and duraplasty using Gore-Tex (W.L. Gore & Associates, Inc., Flagstaff, AZ). In the early postoperative period, neurologic symptoms improved. CONCLUSION: We believe it is important that a treatment plan for Chiari I malformation accompanied by bony anomaly of the craniovertebral junction be determined based on morphologic investigation of the region supplemented by dynamic imaging-based evaluation of instability, or a careful inspection for atypical passage of the vertebral arteries, a frequent site of complication.     

Elucidating the pathophysiology of syringomyelia.

OBJECT: Syringomyelia causes progressive myelopathy. Most patients with syringomyelia have a Chiari I malformation of the cerebellar tonsils. Determination of the pathophysiological mechanisms underlying the progression of syringomyelia associated with the Chiari I malformation should improve strategies to halt progression of myelopathy. METHODS: The authors prospectively studied 20 adult patients with both Chiari I malformation and symptomatic syringomyelia. Testing before surgery included the following: clinical examination; evaluation of anatomy by using T1-weighted magnetic resonance (MR) imaging; evaluation of the syrinx and cerebrospinal fluid (CSF) velocity and flow by using phase-contrast cine MR imaging; and evaluation of lumbar and cervical subarachnoid pressure at rest, during the Valsalva maneuver, during jugular compression, and following removal of CSF (CSF compliance measurement). During surgery, cardiac-gated ultrasonography and pressure measurements were obtained from the intracranial, cervical subarachnoid, and lumbar intrathecal spaces and syrinx. Six months after surgery, clinical examinations, MR imaging studies, and CSF pressure recordings were repeated. Clinical examinations and MR imaging studies were repeated annually. For comparison, 18 healthy volunteers underwent T1-weighted MR imaging, cine MR imaging, and cervical and lumbar subarachnoid pressure testing. Compared with healthy volunteers, before surgery, the patients had decreased anteroposterior diameters of the ventral and dorsal CSF spaces at the foramen magnum. In patients, CSF velocity at the foramen magnum was increased, but CSF flow was reduced. Transmission of intracranial pressure across the foramen magnum to the spinal subarachnoid space in response to jugular compression was partially obstructed. Spinal CSF compliance was reduced, whereas cervical subarachnoid pressure and pulse pressure were increased. Syrinx fluid flowed inferiorly during systole and superiorly during diastole on cine MR imaging. At surgery, the cerebellar tonsils abruptly descended during systole and ascended during diastole, and the upper pole of the syrinx contracted in a manner synchronous with tonsillar descent and with the peak systolic cervical subarachnoid pressure wave. Following surgery, the diameter of the CSF passages at the foramen magnum increased compared with preoperative values, and the maximum flow rate of CSF across the foramen magnum during systole increased. Transmission of pressure across the foramen magnum to the spinal subarachnoid space in response to jugular compression was normal and cervical subarachnoid mean pressure and pulse pressure decreased to normal. The maximum syrinx diameter decreased on MR imaging in all patients. Cine MR imaging documented reduced velocity and flow of the syrinx fluid. Clinical symptoms and signs improved or remained stable in all patients, and the tonsils resumed a normal shape. CONCLUSIONS: The progression of syringomyelia associated with Chiari I malformation is produced by the action of the cerebellar tonsils, which partially occlude the subarachnoid space at the foramen magnum and act as a piston on the partially enclosed spinal subarachnoid space. This creates enlarged cervical subarachnoid pressure waves that compress the spinal cord from without, not from within, and propagate syrinx fluid caudally with each heartbeat, which leads to syrinx progression. The disappearance of the abnormal shape and position of the tonsils after simple decompressive extraarachnoidal surgery suggests that the Chiari I malformation of the cerebellar tonsils is acquired, not congenital. Surgery limited to suboccipital craniectomy, C-I laminectomy, and duraplasty eliminates this mechanism and eliminates syringomyelia and its progression without the risk of more invasive procedures.     

Improvement in brainstem auditory evoked potentials after suboccipital decompression in patients with chiari I malformations

OBJECT: The optimal treatment for patients with symptoms related to Chiari I malformation remains controversial. Although a suboccipital decompression with duraplasty is most commonly performed, there may be a subset of patients who improve in response to bone decompression alone. In an initial attempt to identify such patients, we performed a continuous study of intraoperative brainstem auditory evoked potentials (BAEPs) in patients undergoing a standard decompression with duraplasty and compared conduction times at three different time points: 1) baseline while the patient is supine (before positioning); 2) immediately after opening of the bone and release of the atlantooccipital membrane (that is, the dural band); and 3) after opening of the dura mater. METHODS: Eleven children and young adults (mean age 9.8 years) with symptoms related to Chiari I malformation underwent suboccipital decompression and duraplasty with intraoperative monitoring of BAEPs and somatosensory evoked potentials (SSEPs). Six patients (55%) had associated syringomyelia. At baseline, the I to V interpeak latency (IPL) for both sides (total 21 BAEPs) was 4.19 +/- 0.22 msec (mean +/- standard deviation). After complete bone decompression and before the dura mater was opened, the I to V IPL decreased to 4.03 +/- 0.25 msec (p = 0.0005). When the dura was opened, however, no further decrease in the I to V IPL was detected (4.03 +/- 0.25 msec; p = 0.6). The SSEPs remained stable throughout the procedure. CONCLUSIONS: In children and young adults undergoing suboccipital decompression with duraplasty for Chiari I malformation, the vast majority of improvement in conduction through the brainstem occurs after bone decompressionand division of the atlantooccipital membrane, rather than after opening of the dura. Additional studies are needed to establish whether the improvement seen with BAEP monitoring during bone decompression will predict long-term clinical improvement in these patients.     

Chiari type I malformations in adults: a morphometric analysis of the posterior cranial fossa

OBJECTIVE: Chiari type I malformation (CMI) is a congenital disorder characterized by caudal displacement of the cerebellar tonsils through the foramen magnum into the spinal canal. Recent studies suggest that overcrowding in the posterior cranial fossa (PCF) because of underdeveloped bony structures in the intrauterine life is the main cause of this malformation. For this reason, the authors want to contribute to the current literature, which focuses on bone abnormalities in the PCF in patients with CMIs. METHODS: We examined a retrospective cohort of 60 adult patients with CMIs, and multiple measurements were made on magnetic resonance imaging. The results were compared to 30 healthy adult control subjects. Mann-Whitney U test was used as a statistical method. RESULTS: All measurements except mean anteroposterior diameter of the foramen magnum were reduced in patients compared to control. An increase in the anteroposterior mid-sagittal distance of the foramen magnum in patients reached statistically significant difference compared to control. All patients had tonsillar herniation at least 5 mm below the plane of the foramen magnum. Chiari type I malformation in this study was associated with syringomyelia in 46 patients. CONCLUSION: This study with a limited number of patients suggests that the bony components of the PCF are not developed fully, supporting the current concept that CMI is a disorder of the para-axial mesoderm.