Presentation
The patients' records were retrospectively reviewed for demographic data, chief complaint and presenting symptoms, neurological and radiographic findings, treatment, outcome, and complications. [ncbi.nlm.nih.gov]
The clinical presentation depends on the site of the cavity and the involved structures [6] which ranges from asymptomatic presentation to cranial nerve palsy [7], vocal cord paralysis [8] and facial myokymia [9]. [beat-journal.com]
When present, it is seen in continuity with a long syrinx that demonstrates syringomyelia 1,2. [radiopaedia.org]
Presentation on theme: "Syringobulbia Mark R. Lee, MD, PhD Pediatric Neurosurgery"— Presentation transcript: 1 Syringobulbia Mark R. [slideplayer.com]
Entire Body System
- Pain
[…] heat and pain. [quintpub.com]
It makes perfect sense for those who suffer with relentless pain. [pinterest.se]
Because of the loss of pain and temperature sensation in the hands and inner forearms, painless ulcer may follow a trauma. [nmihi.com]
The case occurred in a 3.5-year-old girl who presented with a history of frequent bouts of severe pain in the lower neck and the shoulder blades. Simple decompression of the syrinx by open myelotomy resulted in prompt relief and reversal of symptoms. [ncbi.nlm.nih.gov]
The disorder generally leads to a cape-like loss of pain and temperature sensation along the back and arms. Each patient experiences a different combination of symptoms. [icd.codes]
Respiratoric
- Stridor
A 10-year, 11-month-old boy who had had chronic stridor since birth died suddenly following the development of slowly progressive symptoms of respiratory obstruction due to vocal cord paralysis. [ncbi.nlm.nih.gov]
Children may rarely present with stridor even. [1] Paraesthasia of the face Due to the involvement of trigeminal nerve there could be bilateral or unilateral sensory impairment for pain and temperature on face. [1][6] Dysphagia Due to the palatal and [explainmedicine.com]
Psychiatrical
- Impulsivity
Syringobulbia clefts due to a dissection of the CSF under pressure from the fourth ventricle should be differentiated from the ascending syringobulbia which may occur from upward impulsive fluid movements in a syringomyelia. [ncbi.nlm.nih.gov]
Neurologic
- Nystagmus
[…] malformation with syringomyelia But very rare… 3-5% of patients with Chiari malformation 4 Symptoms Head and neck pain Snoring Diplopia Sensory disturbances Unsteady gait Dysphagia Paresthesias and numbness 5 Clinical Findings Cranial nerve deficits Nystagmus [slideplayer.com]
Oscillopsia, nystagmus, and Horner syndrome are frequent manifestations of syringobulbia. Oscillopsia may be a disturbing symptom for the patient, whereas Horner syndrome is usually an asymptomatic finding. [ncbi.nlm.nih.gov]
The Disease Database lists the following medical conditions that Syringobulbia may cause: * Bulbar palsy * Cerebellar syndrome * Dizzyness * Nystagmus Other Possible Causes of these Symptoms * Rapid involuntary eye movements * Speaking difficulty * Vertigo [checkorphan.org]
In syringobulbia, the individual frequently complains of dizziness and a sensation of spinning ( vertigo ), jerky eye movements ( nystagmus ), tongue wasting, uni- or bilateral facial sensory impairment, and speech impairment (dysphagia). [nmihi.com]
Syringobulbia may cause vertigo, nystagmus, unilateral or bilateral loss of facial sensation, lingual atrophy and weakness, dysarthria, dysphagia, hoarseness, and sometimes peripheral sensory or motor deficits due to medullary compression. [merckmanuals.com]
- Cranial Nerve Involvement
Spastic paraparesis: Legs > Arms Skeletal Scoliosis Cranial nerve involvement: With syringobulbia Most often unilateral XII: Tongue weakness & hemiatrophy IX - X: Dysphagia; Dysarthria XI: Weakness & wasting of sternomastoid & trapezius VII: Facial paresis [neuromuscular.wustl.edu]
Interestingly, despite the syrinxes in their brainstems, neither patient had symptoms or neurological signs, such as breathing issues or cranial nerve involvement, directly attributable to those syrinxes. [conquerchiari.org]
- Oscillopsia
Oscillopsia, nystagmus, and Horner syndrome are frequent manifestations of syringobulbia. Oscillopsia may be a disturbing symptom for the patient, whereas Horner syndrome is usually an asymptomatic finding. [ncbi.nlm.nih.gov]
Workup
If no focal arachnopathy can be demonstrated after intensive neuroradiological workup (MRI with FLAIR, T1/T2 ± gadolinium-weighted images, cardiac-gated CINE-MRI for revealing CSF pulsation with additional 3D-CISS sequences) [ 22 ] but the syringomyelia [neupsykey.com]
Treatment
Posterior fossa decompression is a safe and effective treatment. [ncbi.nlm.nih.gov]
Conservative treatment primarily includes physical therapy and the symptomatic treatment of spasticity as well as pain management. [neupsykey.com]
As the data is scarce regarding the treatment of isolated syringobulbia, the results are variable and the treatment option should be chosen based on underlying etiology, to achieve the best outcome. [beat-journal.com]
Surgery is the main treatment. Some people also need to have the syrinx drained. Medicines can help ease pain. In some cases, there are no symptoms, so you may not need treatment. [ Read More ] [icdlist.com]
Prognosis
Treatment and prognosis When symptomatic, neurosurgical intervention may be required 1. Promoted articles (advertising) [radiopaedia.org]
Prognosis - Syringobulbia Not supplied. Treatment - Syringobulbia Not supplied. Resources - Syringobulbia Not supplied. [checkorphan.org]
Syringomyelia Prognosis and Outcomes It is generally accepted that if the causative pathology is identified and treated, the long-term improvement and resolution of the syrinx is good. 13 Prognosis is also largely dependent on the prognosis of the primary [csfinfo.org]
The prognosis depends on the course of the underlying lesion. [books.google.ro]
The prognosis depends on the course of the underlying lesion. [books.google.com]
Etiology
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
Syringomyelia Treatment is Based on Etiology Asymptomatic syringomyelia patients with small syrinx cavities and no obvious etiology are best managed with watchful waiting and serial imaging exams. [csfinfo.org]
Although post-traumatic syringomyelia has been wieldy described previously, traumatic brain injury has not been reported as the mechanism and etiology of isolated syringobulbia. [beat-journal.com]
Neuroradiological findings and etiology of the syrinx are discussed, and a causal relationship to the tuberculoma is proposed. [archive-ouverte.unige.ch]
[…] in both adult and pediatric patients is Chiari I malformation, representing 48-50% of adult cases 4,5 and 43.2% of pediatric cases. 6 In adults, the next most common etiologies include idiopathic (15.7%), Chiari II malformation (8.1%), post-traumatic [now.aapmr.org]
Epidemiology
Epidemiology References: [1] [2] Epidemiological data refers to the US, unless otherwise specified. Etiology Syringomyelia is an abnormal fluid-filled dilation of the central canal of the spinal cord occurring as a result of impaired CSF flow. [amboss.com]
General Epidemiology: Varied frequency 1.9 per 100,000 in Japan 8.4 per 100,000 in Western countries Pathology Cavitation: Fluid filled Surrounding tissue: Gliosis Location Spinal cord Usually cervical or thoracic: C2 to T9 Medulla Course: Chronic Children [neuromuscular.wustl.edu]
Associations Scoliosis Spina bifida Klippel-Feil syndrome Epidemiology There is a paucity of data on the prevalence of the condition in the West. [patient.info]
[…] meningeal inflammation Arachnoid scarring related to surgical trauma Subarachnoid space stenosis due to spinal neoplasm or vascular malformation Subarachnoid space stenosis, with possible scarring, related to disk and osteophytic disease Idiopathic Epidemiology [emedicine.medscape.com]
Pathophysiology
The pathophysiology is discussed. [ncbi.nlm.nih.gov]
Includes separate chapters focusing on individual neurologic disorders with detailed descriptions from pathophysiology to in-depth analysis. [books.google.ro]
Pathophysiology Although many mechanisms for syrinx formation have been postulated, the exact pathogenesis is still unknown. Frequently cited theories are those of Gardner, William, and Oldfield. [emedicine.medscape.com]
Prevention
MRI detection of syringomyelia has led to earlier treatment of syringomyelia and prevention of upward extension of the cavity. This probably explains why syringobulbia is less frequently encountered at present. [ncbi.nlm.nih.gov]
Next, an allogeneic expansion duraplasty is performed to prevent recurrent adhesions in this area. [neupsykey.com]
[…] following medical conditions that Syringobulbia may cause: * Bulbar palsy * Cerebellar syndrome * Dizzyness * Nystagmus Other Possible Causes of these Symptoms * Rapid involuntary eye movements * Speaking difficulty * Vertigo * Vocal cord paralysis Prevention [checkorphan.org]
Prevention of ascariasis requires adequate sanitation. [merck.com]
Builds a knowledge base of required skills for the necessary close observation and monitoring of the neuroscience patient to prevent complications and achieve the best outcomes. [books.google.ro]