Current surgical treatment of Chiari type I malformation and Chiari I-syringomyelia complex

Chiari type I malformation and so-called Chiari-I/syringomyelia complex continue to inspire controversy. Disagreement on these malformations concern both etiopathogenesis and treatment. Our still poor understanding of the natural course of the disease, the lack of consensus regarding therapeutic indications and even disagreement on appropriate surgery all contribute to cause disagreement. Significant progress has been made in our understanding of contributing etiopathogenetic factors in recent years. The most widely accepted hypothesis is that anomalous embryonic development characterized by paraxial mesodermal insufficiency would put volumetric constraints on postnatal development of the posterior fossa. We review historical and current controversies regarding Chiari type I malformation and classic theories on causative and contributing factors. We also discuss the latest surgical treatments that have been suggested, as well as associated anomalies--mainly syringomyelia, hydrocephalus and malformed cranial-cervical articulation. Finally, we propose a protocol useful for the diagnosis and treatment of Chiari type I malformation associated with dilation of the ventricular system.     

One-step fluorescent probe product-enhanced reverse transcriptase assay

The Chiari type II malformation is the leading cause of death in infants with myelomeningocele. The authors report 17 cases of symptomatic Chiari type II malformation occurring in two distinct age dependent population. In Group I, 13 neonates and infants in the first year of life presented with cranial nerve and brain stem dysfunction characterized by vocal cord paralysis, apnea, dysphagia and laryngeal stridor. In Group II, 4 patients developed signs and symptoms after the first year of life. In this group, the presentation was more insidious and included neck pain and cerebellar manifestations. The surgical treatment consisted initially in shunt implantation or revision and when there was no improvement, posterior fossa decompression was performed. The response to the surgical treatment differed considerably between the two groups: older patients improved promptly after surgery and there was no casualties; in newborn and infants, especially those under 6 months of age, the mortality rate was 46.1%. The authors stress that prompt diagnosis and surgical intervention should be performed in order to produce a favorable outcome.     

Posterior fossa decompression without duraplasty in infants and young children for treatment of Chiari malformation and achondroplasia

Some children with Chiari malformation and achondroplasia require posterior fossa decompression that typically includes expansion of the dural tube with duraplasty. Infants and young children, however, may have a more distensible dura mater than do older patients. Furthermore, the structures that compress the hindbrain of young patients may be the bone and abnormally thickened atlantooccipital membrane, i.e., dural band, rather than the dura mater. We have treated 7 children who had Chiari malformation or achondroplasia with posterior fossa decompression without duraplasty. All children were symptomatic; 3 had Chiari-I malformations, 2 Chiari-II malformations, and 2 achondroplasia. The age range was 3 months to 2.5 years (mean 15.1 months). The exent of tonsillar herniation and other hindbrain anomalies was assessed on preoperative magnetic resonance imaging. The infants with Chiari-II malformations underwent cervical laminectomies, whereas the other young children with Chiari-I malformations or achondroplasia underwent suboccipital craniectomy as well as cervical laminectomy. In Chiari malformation, the dural band was divided; in achondroplasia, there was no identifiable dural band. Following bony decompression and division of the identifiable dural band, immediate expansion of the stenotic region with visible cerebrospinal fluid space posterior to the neural elements could be ascertained by intraoperative ultrasonography. During a follow-up period ranging from 4.5 months to 4 years (mean 22 months), all patients made improvements in their symptoms, 3 having complete resolution of their symptoms. This preliminary experience indicates that in children 2 years of age or younger, posterior fossa bony decompression without duraplasty can be effective treatment for Chiari malformations or achondroplasia.     

Chiari I malformation associated with syringomyelia and scoliosis

STUDY DESIGN: A retrospective review of a series of 12 children who underwent suboccipital foraminotomy and duroplasty for Chiari I malformation. OBJECTIVE: To assess the effects of this surgery on associated syringomyelia and scoliosis. SUMMARY OF BACKGROUND DATA: Suboccipital foraminotomy for the treatment of syringomyelia associated with Chiari I malformation was greatly stimulated by Gardner's hydrodynamic theory, and its results proved to be encouraging. However, several authors reported improvement or stabilization of associated scoliosis after this surgery. METHODS: A retrospective review was conducted on 12 patients who underwent suboccipital foraminotomy for Chiari I malformation associated with syringomyelia. Neurologic Impairment, extent of syringomyelia, and severity of associated spinal deformity were assessed preoperatively and at a 4.5-year average follow-up (range, 2.1-12 years). Anomaly of superficial abdominal reflexes was found in all cases, and para or tetraparesis in three cases. Syringomyelia was of variable localization and extent. Scoliosis was present in 7 cases (greater than 40 degrees in 5 cases). RESULTS: Diminution or complete disappearance of syringomyelia was observed in 11 cases, 3 months to 1 year after surgery. Superficial abdominal reflexes anomaly improved in four cases. Minimal neurologic deficit persisted in one case. Scoliosis improved in one case, remained unchanged in one case, and progressed in the five cases with preoperative severe deformity, requiring instrumentation and fusion. CONCLUSIONS: Improvement of syringomyelia and neurologic deficit, observed with suboccipital foraminotomy, supports the theory that abnormal hydrodynamics of the cerebral spinal fluid is most likely to cause these deficits.     

Frontal foramina in pediatric skull in cases of congenital hydrocephalus

PURPOSE: To describe a new observation, frontal calvarial foramina, in pediatric patients with congenital hydrocephalus secondary to central nervous system malformation. MATERIALS AND METHODS: Frontal foramina were initially identified in three female patients with Chiari II malformation. Subsequently, head computed tomographic (CT) scans in 99 patients with congenital hydrocephalus were retrospectively reviewed. CT scans in a control group of 116 patients without hydrocephalus were also retrospectively reviewed. RESULTS: Frontal foramina were found in eight of 61 (13%) patients with Chiari II malformation, in one child with Dandy-Walker malformation, and in one child with occipital horn dilatation (colpocephaly), but not in control patients. Sequential CT examinations in three patients with frontal foramina depicted gradual closure after ventriculoperitoneal shunt placement. CONCLUSION: Frontal foramina may represent an abnormality variably expressed in certain central nervous system malformations that cause congenital hydrocephalus. The presence of frontal foramina palpated or visualized on plain radiographs may help in the diagnosis of congenital hydrocephalus and central nervous system malformation.     

Adult Arnold-Chiari malformation: a postpartum case presentation

Adult Arnold-Chiari malformation, also known as Chiari malformation type I, typically occurs in women during early adulthood and can be a cause of unexplained headaches, as well as associated syringomyelocele. In this unique case report, a 32-year-old, postpartum woman with posturally induced headache from Chiari malformation type I had symptoms occur for the first time during pregnancy. Magnetic resonance imaging of the brain confirmed the diagnosis and is the neuroimaging study of choice in such cases. Neurosurgical decompression, when performed early, is highly successful in symptomatic individuals.     

The role of pharmacy in the management of patients with temporomandibular disorders and orofacial pain

OBJECTIVE: To provide information regarding the current understanding of the etiology and treatment, both nonpharmacologic and pharmacologic, of orofacial pain conditions including temporomandibular disorders (TMDs). This review briefly discusses the etiology and pathophysiology underlying the development of TMDs, generally accepted nonpharmacologic methods of treatment, and the most common current pharmacologic management approaches. DATA SOURCES: Current medical literature and the authors' clinical experiences. DATA SYNTHESIS: TMDs encompass a number of diagnostic subgroups that involve the masticatory musculature, the temporomandibular joint(s), and associated structures. More than 10 million individuals in the United States are affected by TMDs. Most current pharmacologic management approaches in the treatment of orofacial pain conditions, including TMDs, involve the use of antidepressants, anticonvulsants, muscle relaxants, corticosteroids, and nonsteroidal anti-inflammatory drugs. CONCLUSION: Inclusion of pharmacists who are knowledgeable in the nonpharmacologic and pharmacologic treatment approaches on the TMD management team would improve therapeutic monitoring, follow-up, and outcomes in these patients.     

Post polio syndrome: an update for the primary health care provider

Post Polio Syndrome, or PPS, is defined as a clinical syndrome of new weakness, fatigue, and pain in people who have previously recovered from acute paralytic poliomyelitis. Other common symptoms include cold intolerance, dysphagia, dyspnea, and overuse syndromes. PPS afflicts an estimated 50% of polio survivors, a population estimated at 1.6 million people, and begins roughly 30 years after the acute disease. The main impact of PPS is on mobility related activities affecting one's daily routine. With an insidious onset, and several differential diagnoses for each symptom, PPS can be difficult to diagnose and to validate. However, once identified, there are treatment plans and many avenues of support for this disabling syndrome. The purpose of this article is to provide an overview of the pathophysiology of both acute paralytic poliomyelitis as well as PPS. This article also reviews the current literature concerning the etiology and pathophysiology of both poliomyelitis and PPS, symptom evaluation and differential diagnoses, and treatment recommendations. The psychosocial impact and care of the client are also identified, and several resources for support and education of both the client and provider are provided.     

Chiari Type I malformation and syringomyelia in unrelated patients with blepharophimosis. Report of two cases

Syringomyelia is a rare, mainly sporadic disease of the spinal cord, which is associated with 80% of cases in which a Chiari Type I malformation is also present. A mendelian transmission of syringomyelia (autosomal dominant or recessive) has been proposed in approximately 2% of reported cases. The association of syringomyelia with hereditary diseases (Noonan's syndrome, phacomatoses) has been mentioned frequently in the literature. The authors report the presence of a Chiari Type I malformation accompanied by syringomyelia in two unrelated patients affected by a familial Type II blepharophimosis-ptosis-epicanthus inversus syndrome (BPES). The first patient was a 35-year-old woman who presented with a right C-8 root paresia. The second case involved a 20-year-old man who complained of cervical radicular pain. Both belong to families in which BPES was segregated in an autosomal dominant modality, but other family members had no known neurological symptoms. To the authors' knowledge, such a combination has never been described. Perhaps the possible involvement of a genetic component in some cases of Chiari Type I-associated syringomyelia will someday be debated.     

Technique of lateral canthoplasty for the correction of macropalpebral fissure in the dog

Chiari III malformations are extremely rare hindbrain malformations that are associated with a high early mortality rate, or severe neurologic deficits in the survivors. The preferred treatment is early operative closure and CSF shunting. We report a case of a newborn infant with a Chiari III malformation with displacement of the brainstem and cerebellum into the cervical encephalocele which precluded immediate operative closure of the defect. Instead, a ventriculoperitoneal shunt was placed and the patient was followed with serial imaging studies. The child survived. The shunt allowed the brainstem and cerebellum to regress into the cervical spinal canal as the dilated cerebral aqueduct and fourth ventricle decompressed. A delayed closure of the cervical encephalocele was performed at 30 months of age. Cerebrospinal fluid diversion with delayed closure may be an option for large lesions.     

Epilepsy in older adults: causes, consequences and treatment

OBJECTIVES: Seizures and epilepsy are common problems in older adults. Although, the highest incidence of seizures and epilepsy occurs in individuals more than 65 years of age, the magnitude of this public health problem, and its consequences on the quality of life of older adults, are not appreciated. Moreover, there is no consensus on the most appropriate way to diagnose or manage epilepsy in this population. This report reviews the current literature on all aspects of epilepsy in older people. DESIGN/METHODS: The medical literature was reviewed for all articles pertaining to pathophysiology, diagnosis, and treatment of epilepsy in older people. RESULTS: Epidemiology, etiology, diagnosis, use of diagnostic tests including EEG and imaging, new medical and surgical treatments, and psychosocial issues as they relate to older epilepsy patient are discussed. Several questions that merit future systematic investigation are presented. CONCLUSION: Seizures and epilepsy in older people are much more common than is generally known. There are unique issues related to this population with regard to diagnosis and treatment. Several new medical and surgical therapies are now available for all epilepsy patients, some of which may be helpful for the older epilepsy patient.     

Eating disorders: National Institute of Mental Health's perspective.

The mission of the National Institute of Mental Health (NIMH) is to reduce the burden of mental and behavioral disorders through research, and eating disorders embody an important fraction of this burden. Although past and current research has provided important knowledge regarding the etiology, classification, pathophysiology, and treatment of the eating disorders, there are still significant challenges that need to be addressed. This article briefly describes some of these challenges, recent NIMH-supported research and research-related activities directed at addressing these challenges, and approaches and areas of research that hold promise for furthering the understanding and treatment of eating disorders. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Hydrodynamics of syringomyelia

Abnormality of CSF hydrodynamics is an important factor of the pathogenesis of syringomyelia. Recent development of cine-MRI visualized real time CSF movement in the syrinx or subarachnoid space. In the present study, twenty cases of syringomyelia associated with Chiari I malformation, 10 cases of syringomyelia associated with spina bifida aperta, and 10 cases of syringomyelia associated with spina bifida occulta were studied with MRI and cine-MRI. The result demonstrated that hydrodynamics of these three groups were different to each other. Chiari I malformation showed CSF hydrodynamic abnormality at the foramen magnum and marked flow void in the syrinx. On the other hand, myelomeningocele showed hydrocephalus and less prominent flow void in the syrinx. Spinal lipoma which is the majority of spina bifida occulta showed no abnormality of CSF hydrodynamics. In conclusion, the selection of treatments should be individualized depending on the hydrodynamical abnormality of each patient.     

Congenital diaphragmatic hernia. I. Simple defect of the diaphragm or anomaly of the pulmonary mesenchyme?

Described for the first time in 1848 by Bochdalek, congenital diaphragmatic hernia is still a hot topic. How can it be that a simple defect of the diaphragm still has a mortality rate reaching 50% in 1997, and this despite continuous progress in neonatal intensive care? If some problems remain unsolved, experimental studies over the past 30 years have raised some questions concerning the pathogenesis, and have shed some light into the pathophysiology of congenital diaphragmatic hernia. This article reviews the recent knowledge about the aetiology, pathogenesis and pathophysiology of this complex malformation.     

Surgical complications of open spinal dysraphism

The embryogenesis, closure technique, and preoperative preparation of open myelomeningocele are described in this article. Both early and late complications of myelomeningocele closure are discussed with respect to predisposing factors, diagnosis, treatment, and prevention. These complications include worsened neurological level, wound dehiscence, wound infection, cerebrospinal fluid leak, postoperative ileus, symptomatic Chiari malformation, shunt infection, necrotizing enterocolitis, and problems related to kyphectomy.     

Presentation and management of Chiari I malformation in children

To determine the efficacy of operative treatment for children with Chiari I malformation, the medical records and magnetic resonance imaging (MRI) studies of 68 consecutive patients cared for at The Children's Hospital, Boston, Mass., USA, from December, 1988 to November, 1996 were retrospectively reviewed. All patients underwent suboccipital craniectomy, C1 laminectomy, and dural grafting. Bipolar coagulation to shrink and reduce the volume of the cerebellar tonsils was carried out in 40 patients. In 32 of 40 patients with associated syringomyelia, the procedure included placement of a IVth ventricle to cervical subarachnoid space shunt. Twenty-three patients with syringomyelia also had plugging of the obex. There was no operative mortality. Morbidity included a 22% incidence of nausea/vomiting and a 10% incidence of headache, both limited to the immediate postoperative period. Within the first postoperative month, all patients or their parents reported clear improvement in their presenting symptoms and 93% were found to have clear improvement in their presenting signs. In follow-up periods of 6-70 months, all patients had continued unequivocal symptom improvement and all patients were found on examination to have clear improvement in neurological signs. In patients with syringomyelia, MRI studies carried out at least 6 months postoperatively revealed near total or total syrinx resolution in 80% of the cases. This study demonstrates that a standard bony and dural decompression of the foramen magnum region with modifications designed to maximize the restoration of CSF circulation across the foramen magnum is a safe, effective operative treatment for Chiari I malformation in children.     

Primary craniovertebral anomalies and the hindbrain herniation syndrome (Chiari I): data base analysis

This prospective study analyzes 100 patients with Chiari malformation and primary craniovertebral junction (CVJ) anomalies (3-66 years). Neurodiagnostic investigations employed tomography, gas myelography, CT and CT myelography, and MRI. Factors considered were reducibility, mode of encroachment, cerebrospinal fluid (CSF) dynamics and syringohydromyelia. Sixty-six patients with irreducible pathology underwent ventral or ventrolateral decompression and dorsal stabilization. Dorsal occipitocervical fixation was performed in reducible lesions that also required dorsal decompression (n = 34). Proatlas remnants were identified in 8 and atlas assimilation in 92 patients. Paramesial invagination was present in 20, syringohydromyelia in 46, and vertebral segmentation defects in 66 others. Completely reducible abnormalities were identified in 16 of 20 patients aged 2-14 years, and partially reducible abnormalities in 4 of 16 patients aged 14-20 years, 8 of 48 patients aged 20-40 years and 6 of 16 patients aged 40-60 years. The critical sagittal canal diameter at the foramen magnum was 19 mm. Twenty-two patients had previous posterior decompression and 27 had previous syrinx to subarachnoid shunt with delayed deterioration. Improvement occurred in all after ventral or ventrolateral decompression with resolution of the syringohydromyelia and normalization of CSF flow. We conclude that: (1) hindbrain herniation syndrome is frequently seen with fourth occipital sclerotome abnormalities; (2) Chiari malformation with craniovertebral abnormalities become symptomatic with a canal diameter of < 19 mm; (3) abnormal ventral bony pathology is reducible in children wit atlas assimilation and later becomes irreducible invagination, therefore early operation with fusion is recommended; (4) ventral decompression relieves brain stem, cerebellar symptoms and syringohydromyelia; (5) CSF studies with cine MRI shows reversal of craniospinal CSF dissociation after ventral CVJ decompression and; (6) craniovertebral anomalies associated with Chiari malformations must be addressed early and appropriately.     

Treatment of sexual disorders in the 1990s: An integrated approach.

Marked changes have occurred in the formulation and treatment of sexual disorders in the past 2 decades. Emphasis has shifted to the role of biomedical and organic factors in the etiology of sexual dysfunction, along with the growing use of medical and surgical treatment interventions. Multidimensional assessment models are widely used, particularly in the evaluation of male erectile dysfunction and sexual pain disorders. Integrated treatment approaches have also been developed, as cognitive-behavioral and couples' therapy procedures are increasingly combined with traditional sex therapy techniques. This article reviews existing data regarding the etiology and treatment of male and female sexual dysfunctions. Despite the conceptual and technological sophistication of current approaches, treatment outcome is less than satisfactory in several areas. Further research is needed on the etiology and treatment of sexual disorders. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Simplified hepatic resection utilizing absorbable polyglycolic acid-based tape and other ligature apparatus

Central nervous system malformations have been reported in a number of inherited enzyme defects. Ethylmalonic encephalopathy, an organic aciduria of unknown pathogenesis, has not been reported previously in association with brain or spinal cord malformations. We report on 2 sibs with confirmed ethylmalonic encephalopathy and malformations of the central nervous system; one with tethered cord, the other with cerebellar tonsillar ectopia (Chiari I malformation).     

Developmental expression of a DNA repair gene in Arabidopsis

As the use of electroconvulsive therapy (ECT) increases, the chance of a practitioner's encountering a patient with significant heart failure, ventricular dysfunction, or valvular heart disease also increases. This article reviews the epidemiology, pathophysiology, and available data on the risk of ECT in these patients. Recommendations are made regarding evaluation and treatment of such patients. Some special situations are identified that may require a modification of routine procedures. Overall, ECT can be performed safely in most patients with underlying cardiac conditions, as long as appropriate precautions are taken to identify these patients ahead of time.