scholarly journals Surgical Experience in Pediatric Patients with Chiari-I Malformations Aged ≤18 Years

2019 ◽  
Vol 10 (01) ◽  
pp. 85-88 ◽  
Author(s):  
Ghanshyam Das Singhal ◽  
Shakti Singhal ◽  
Gunjan Agrawal ◽  
Deepti Singhal ◽  
Vipin Arora
Keyword(s):  
Pediatric Patients ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Neurological Deficits ◽  
Resonance Imaging ◽  
Surgical Experience ◽  
Limb Weakness ◽  
Lower Limb Weakness ◽  
Chiari I

ABSTRACT Objective: The objective of this study was to retrospectively study Chiari I malformation patients (<18 years) treated surgically. Materials and Methods: Chiari I malformation patients (<18 years) treated surgically at our institute were retrospectively studied. Results: During the study period between January 1999 and June 2011, fifty patients, aged ≤18 years with Chiari malformation, were treated surgically and formed the basis for this series. There were 21 female children (42%) and 29 male children (58%), with a female-to-male ratio of 1:1. At the last follow-up, oropharyngeal symptoms were improved in 33% (n = 3/9). Headache/neck/back pain improved in 69.56% of children (n = 16/23). Upper-extremity pain/weakness/numbness improved in 73.91% of children (n = 17/23). Ataxia improved in 66.66% of children (n = 4/6). Lower-limb weakness/hyperreflexia improved in 83.33% of children (n = 5/6). At follow-up, magnetic resonance imaging for patients with syrinx was available for 75% of patients (n = 30/50) and not available for 25% of patients (n = 10/40). Syrinx was diminished in size or resolved in 66.33% of patients (n = 19/30) and the remaining was same for 36.66% of patients (n = 11/30). Conclusions: The main goal of surgery is to arrest the progression of neurological deficits. Foramen magnum decompression with a lax duroplasty is the surgical procedure of choice.

Hiccups Attributable to Syringobulbia and/or Syringomyelia Associated with a Chiari I Malformation: Case Report

Neurosurgery ◽  
2004 ◽  
Vol 54 (1) ◽  
pp. 224-227 ◽  
Author(s):  
Toshitaka Seki ◽  
Kazutoshi Hida ◽  
JangBo Lee ◽  
Yoshinobu Iwasaki
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Neurological Deficits ◽  
Resonance Imaging ◽  
Foramen Magnum Decompression ◽  
Chiari I ◽  
Cerebellar Symptoms ◽  
Intractable Hiccups

Abstract 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 with Syringomyelia

2019 ◽  
pp. 57-64
Author(s):  
Yiping Li ◽  
Bermans J. Iskandar
Keyword(s):  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Neurological Deficits ◽  
Csf Leakage ◽  
Craniocervical Instability ◽  
Chiari I ◽  
Spine Mri ◽  
Operative Monitoring ◽  
Associated Abnormalities ◽  
Cerebellar Tonsils

Chiari I malformation (CIM) is defined by pathological herniation of the cerebellar tonsils below the foramen magnum. Operative intervention for CIM is generally undertaken to treat neurological deficits associated with tonsillar herniation or with associated syringomyelia. A complete brain and spine MRI is indicated to rule out associated abnormalities and to identify and the presence and extent of syringomyelia. The type of surgical decompression remains controversial but may include bone-only decompression, bony decompression followed by duraplasty, and bony decompression followed by duraplasty and tonsillar shrinkage. Post-operative monitoring for CSF leakage, inadequate decompression, subtle chronic craniocervical instability, and hydrocephalus is critical.

scholarly journals Spinal cord compression secondary to vertebral echinococcosis

2016 ◽  
Vol 7 (01) ◽  
pp. 143-146 ◽  
Author(s):  
Abat Sahlu ◽  
Brook Mesfin ◽  
Abenezer Tirsit ◽  
Knut Wester
Keyword(s):  
Muscular Strength ◽  
Cord Compression ◽  
Lower Limbs ◽  
Resonance Imaging ◽  
Limb Weakness ◽  
Lower Limb Weakness ◽  
Cervical Approach ◽  
Anterior Cervical Approach ◽  
Anterior Plating

ABSTRACTWe describe a patient with progressive lower limb weakness and paresthesia 3 days after falling from a considerable height. Magnetic resonance imaging and computed tomography revealed collapsed Th2 and Th3 vertebrae. A tuberculous (TB) spondylitis was suspected, and anti-TB medication was started however with no clinical improvement. She was referred to our center and operated. A 3 level discectomy and 2 level corpectomy were performed with iliac bone grafting and anterior plating via an anterior cervical approach. The patient developed an esophagocutaneous fistula that was repaired and cured. The biopsy specimen showed a hydatid cyst of the vertebra as the cause of the lesion. After the result, she was started on oral albendazole. At follow-up nearly 4 months after surgery, the patient had regained significant power in her lower limbs with a muscular strength of 5/5 in both legs, thus making it possible to walk without support.

scholarly journals CSF goes astray no matter what is in the way: occipital intradiploic pseudomeningocele and de novo syringomyelia following foramen magnum decompression for Chiari I malformation in a pediatric patient

2020 ◽  
Vol 25 (5) ◽  
pp. 529-534
Author(s):  
Arthur R. Kurzbuch ◽  
Shailendra Magdum
Keyword(s):  
Revision Surgery ◽  
De Novo ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Foramen Magnum Decompression ◽  
Dural Closure ◽  
First Case ◽  
Chiari I ◽  
Lost To Follow Up

Nontraumatic intradiploic pseudomeningoceles and de novo syringomyelia formation are very rare entities. The authors have previously reported the case of a 4-year-old girl who underwent foramen magnum decompression without dural closure for Chiari I malformation. Three years after the operation an intradiploic pseudomeningocele was documented, but the patient was lost to follow-up without undergoing revision surgery. Four years later, at the age of 11 years, the patient returned for treatment of intensifying symptoms. Radiological imaging then showed an increase in the size of the intradiploic pseudomeningocele and a new cervical syrinx. The patient underwent a first revision surgery in which a part of the internal layer of the occipital bone was removed and arachnoid scar lysis was performed. Two months later the syrinx had worsened, and in a second revision surgery a pseudomeningocele-peritoneal shunt was placed. Here, the authors describe what is to their knowledge the first case of an intradiploic pseudomeningocele and de novo syringomyelia formation following Chiari I decompressive surgery.

Spontaneous Resolution of Chiari I Malformation and Syringomyelia: Case Report and Review of the Literature

Neurosurgery ◽  
2001 ◽  
Vol 48 (3) ◽  
pp. 664-667 ◽  
Author(s):  
Jörg Klekamp ◽  
Giorgio Iaconetta ◽  
Madjid Samii
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cerebrospinal Fluid ◽  
Case Report ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Spontaneous Resolution ◽  
Magnetic Resonance Imaging Scan ◽  
Resonance Imaging ◽  
Chiari I ◽  
Resonance Imaging Scan

Abstract 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.

Posterior atlantooccipital membrane for duraplasty

2002 ◽  
Vol 97 (2) ◽  
pp. 266-268 ◽  
Author(s):  
R. Shane Tubbs ◽  
John C. Wellons ◽  
Jeffrey P. Blount ◽  
W. Jerry Oakes
Keyword(s):  
Foreign Body ◽  
Postoperative Complications ◽  
Pediatric Patients ◽  
Chiari I Malformation ◽  
Posterior Cranial Fossa ◽  
Content Type ◽  
Chiari I ◽  
Cranial Fossa ◽  
Fine Print

✓ The authors describe the use of autogenetic posterior atlantooccipital (PAO) membrane for duraplasty following after posterior cranial fossa surgery. The PAO membrane is routinely exposed for procedures of the posterior cranial fossa and merely needs to be dissected free of the underlying dura mater. Recently this membrane was obtained in several pediatric patients following procedures of the posterior cranial fossa such as duraplasty in case of Chiari I malformation. No postoperative complications were found at 6-month follow-up examination. The advantages of this intervention include less manipulation of muscle and fascia than that involved in other procedures and, therefore, seemingly less postoperative pain and the negation of issues inherent with foreign-body graft sources. The authors believe this structure to be of use as a dural substitute in small dural openings of the posterior cranial fossa.

Association of Chiari I Malformation, Mental Retardation, Speech Delay, and Epilepsy: A Specific Disorder?

Neurosurgery ◽  
2001 ◽  
Vol 49 (5) ◽  
pp. 1099-1104 ◽  
Author(s):  
Salvatore Grosso ◽  
Roberto Scattolini ◽  
Galluzzi Paolo ◽  
Rosanna Maria Di Bartolo ◽  
Guido Morgese ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Mental Retardation ◽  
Magnetic Resonance ◽  
Incidental Finding ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Speech Delay ◽  
Resonance Imaging ◽  
Cavum Septum Pellucidum ◽  
Chiari I

ABSTRACT OBJECTIVE The Chiari I malformation is defined as tonsillar herniation of at least 3 to 5 mm below the foramen magnum. Although Chiari I malformation is considered to derive from a mesodermal disorder resulting in underdevelopment of the posterior fossa relative to its content, evidence for a possible heterogeneous etiology also has been reported. The aim of the present study is to elucidate the relationship between Chiari I malformation and mental retardation, speech delay, and epilepsy to consider a possible specific pathogenetic background. METHODS Thirty-five patients with Chiari I malformations were identified by use of magnetic resonance imaging during a period between 1993 and 1999. The study consisted of nine patients (four boys and five girls) who were affected by mental retardation, speech delay, and epilepsy. All patients underwent electroencephalography and brain and cervical spine magnetic resonance imaging. RESULTS All patients were mentally retarded with a mean intelligence quotient of 50. Seven patients had a positive history for speech delay, and five were epileptic. Electroencephalograms demonstrated abnormalities in seven patients. The mean tonsillar displacement was 10.1 mm. A thin corpus callosum and a wide cavum septum pellucidum were present in three patients. Neither hydromyelia nor scoliosis was observed. No correlation between the degree of the ectopia and clinical manifestation was noted. CONCLUSION The association of Chiari I malformation with epilepsy, speech delay, and mental retardation may not be a mere incidental finding but may be a marker for a different pathogenetic background.

SYMPTOMATIC CHIARI TYPE I MALFORMATION AFTER RADIATION THERAPY IN AN INFANT

Neurosurgery ◽  
2007 ◽  
Vol 60 (4) ◽  
pp. E782-E782 ◽  
Author(s):  
Caitlin E. Hoffman ◽  
Eric Lis ◽  
Suzanne L. Wolden ◽  
Mark M. Souweidane
Keyword(s):  
Magnetic Resonance Imaging ◽  
Radiation Therapy ◽  
Magnetic Resonance ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Type I ◽  
Malignant Rhabdoid Tumor ◽  
Resonance Imaging ◽  
Anterior Cranial Base ◽  
Chiari I

Abstract OBJECTIVE One previous case report has described the development of a Chiari I malformation in a child after fractionated radiation therapy to the anterior cranial base. The authors present an additional case of a 17-month-old girl treated for an isolated, malignant, rhabdoid tumor of the right neck with neoadjuvant chemotherapy, surgical exploration, and radiation therapy. CLINICAL PRESENTATION The patient developed a Chiari I malformation and cervical syringomyelia 1.5 years after the treatment. The patient experienced two episodes of unresponsiveness before diagnosis, and the malformation was then identified on magnetic resonance imaging scans. INTERVENTION Suboccipital decompressive craniectomy, and C1 laminectomy with autologous duraplasty were performed. RESULTS Postoperative magnetic resonance imaging scans revealed reconstitution of cerebrospinal fluid at the foramen magnum and ascent of the cerebellar tonsils. The patient remains asymptomatic 5.5 years after treatment. CONCLUSION The importance of this association is discussed in the context of the increasing use of radiation therapy in young children and infants.

Treatment of Chiari type I malformation in patients with and without syringomyelia: a consecutive series of 66 cases

2001 ◽  
Vol 11 (1) ◽  
pp. 1-9 ◽  
Author(s):  
Juan C. Alzate ◽  
Karl F. Kothbauer ◽  
George I. Jallo ◽  
Fred J. Epstein
Keyword(s):  
Chiari I Malformation ◽  
Neurological Deficits ◽  
Motor Deficits ◽  
Consecutive Series ◽  
Spinal Cord Tissue ◽  
Type I ◽  
Excellent Outcome ◽  
Dural Graft ◽  
Chiari I

Object The authors describe the results of performing a standard posterior craniovertebral decompression and placement, if indicated, of a syringosubarachnoid shunt for the treatment of patients with Chiari I malformation with and without syringomyelia. Methods This is a retrospectively analyzed consecutive series of 66 patients (mean patient age 15 years, range 1–53 years). The uniform posterior craniovertebral decompression consisted of a small suboccipital craniectomy, a C-1 laminectomy, microsurgical reduction of the cerebellar tonsils, and dural closure with a synthetic dural graft to increase the cerebrospinal fluid space at the craniocervical junction. The presence of a large syrinx, with significant thinning of the spinal cord tissue and obliteration of the spinal subarachnoid space, particularly when combined with syrinx-related symptoms, was an indication for the placement of a syringosubarachnoid shunt. In 32 patients Chiari I malformation alone was present, and 34 in patients it was present in combination with syringomyelia. Clinical findings included pain, neurological deficits, and spinal deformity. The presence of syringomyelia was significantly associated with the presence of scoliosis (odds ratio 74.4 [95% confidence interval 8.894–622.4]). All patients underwent a posterior craniovertebral decompression procedure. In 22 of the 34 patients with syringomyelia a syringosubarachnoid shunt was also placed. The mean follow-up period was 24 months (range 3–95 months). Excellent outcome was achieved in 54 patients (82%) and good outcome in 12 (18%). In no patient were symptoms unchanged or worse at follow-up examination, including four patients who initially required a second operation for persistent syringomyelia. Pain was more likely to resolve than sensory and motor deficits after decompressive surgery. Radiological examination revealed normalization of tonsillar position in all patients. The syrinx had disappeared in 15 cases, was decreased in size in 17, and remained unchanged in two. Conclusions Posterior craniovertebral decompression and selective placement of a syringosubarachnoid shunt in patients with Chiari I malformation and syringomyelia is an effective and safe treatment. Primary placement of a shunt in the presence of a sufficiently large syrinx appears to be beneficial. The question of if and when to place a shunt, however, requires further, preferably prospective, investigation.

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