Syringomyelia – Rokowania, prognozy i postęp choroby

Syringomyelia
Rokowania, prognozy i postęp choroby

Syringomyelia charakteryzuje się dużą heterogennością kliniczną, co utrudnia prognozowanie rokowania. Kluczowymi czynnikami predykcyjnymi niekorzystnego przebiegu są dysfunkcja autonomiczna, deficyty motoryczne, zaburzenia chodu, ciężkie deficyty neurologiczne oraz zespół centralnego uszkodzenia rdzenia. Z kolei lepsze rokowanie wiąże się z wiekiem poniżej 47 lat w momencie operacji (AUC = 0,71, OR = 5,95), obecnością przepływu płynu mózgowo-rdzeniowego (CSF) na poziomie połączenia czaszkowo-szyjnego (AUC = 0,68, LH+ = 2,1) oraz typem moniliformnym jamistości rdzenia. Radiologicznie średnica jamy syringomielicznej powyżej 5 mm i towarzyszący obrzęk wskazują na gorsze rokowanie. Leczenie operacyjne, zwłaszcza w wadzie Chiari typu I, jest skuteczne w ponad 90% przypadków, a wczesna interwencja poprawia wyniki kliniczne i radiologiczne.

<a href="#prognozy-syringomyelii-wprowadzenie”>Prognozy Syringomyelii – wprowadzenie
Czynniki prognostyczne wyników leczenia

Czynniki kliniczne negatywnie wpływające na rokowanie
Czynniki prognostyczne pomyślnego wyniku leczenia
Czynniki anatomiczne i radiologiczne

Wyniki leczenia operacyjnego

Skuteczność interwencji chirurgicznej
Znaczenie czasu interwencji
Stabilizacja i poprawa po leczeniu

Przewidywanie wyników i algorytmy prognostyczne

Modele predykcyjne
Kliniczne predyktory w diagnostyce

Potencjalne powikłania i historia naturalna
Przyszłe kierunki badań

Kolejne rozdziały

syringomyelii-wprowadzenie”>Prognozy Syringomyelii – wprowadzenie

Syringomyelia (jamistość rdzenia) charakteryzuje się dużą zmiennością pod względem objawów, nasilenia i przyczyn. Ta heterogenność sprawia, że przewidywanie rokowania jest wyzwaniem dla klinicystów. Pacjenci mogą doświadczać różnego tempa progresji choroby lub nie wykazywać progresji wcale, co dodatkowo utrudnia prognozowanie długoterminowych wyników leczenia.¹ Rokowanie w syringomyelii zależy od kilku kluczowych czynników, w tym przyczyny podstawowej, stopnia dysfunkcji neurologicznej oraz lokalizacji i rozległości jamy syringomielicznej.²

Czynniki prognostyczne wyników leczenia

Czynniki kliniczne negatywnie wpływające na rokowanie

W analizie jednowymiarowej zidentyfikowano kilka istotnych czynników predykcyjnych niekorzystnego rokowania neurologicznego i funkcjonalnego. Do najważniejszych należą:³

Dysfunkcja autonomiczna – silny predyktor gorszych wyników neurologicznych i funkcjonalnych
Deficyty motoryczne – powszechnie obserwowane u pacjentów z syringomyelią, stanowią silny predyktor niekorzystnych wyników neurologicznych i funkcjonalnych
Zaburzenia chodu i deficyty ruchowe – niezależnie prognozują gorsze wyniki kliniczne i radiologiczne po operacji dekompresyjnej w przypadku Chiari typu I z zaburzonym przepływem płynu mózgowo-rdzeniowego⁴
Ciężkie początkowe deficyty neurologiczne – pacjenci z umiarkowanymi lub ciężkimi deficytami neurologicznymi mają znacznie gorsze rokowanie niż pacjenci z łagodnymi deficytami⁵
Zespół centralnego uszkodzenia rdzenia – pacjenci z tym zespołem słabo odpowiadają na leczenie⁶

Czynniki prognostyczne pomyślnego wyniku leczenia

Zidentyfikowano również czynniki związane z lepszym rokowaniem:⁷⁸⁹

Wiek w momencie operacji – zabieg przeprowadzony przed 47 rokiem życia jest znaczącym predyktorem dobrego wyniku (AUC = 0,71, 95% CI 0,54-0,89), zwiększając prawdopodobieństwo pomyślnego wyniku około sześciokrotnie (OR = 5,95, IC 95% 1,03-34,4)
Obecność przepływu płynu mózgowo-rdzeniowego (CSF) na poziomie połączenia czaszkowo-szyjnego (CCJ) – przedoperacyjna obecność przepływu CSF na poziomie CCJ pozwala przewidzieć dobry wynik (AUC = 0,68, 95% CI 0,50-0,87 i LH+ = 2,1, IC 95% 1,16-3,07) i jest również istotnie związana z pooperacyjnym ustąpieniem bólu (rho = 0,61, p = 0,0144)
Jamistość rdzenia typu moniliformnego (koralikowego) – sugeruje stosunkowo lepsze rokowanie, a skuteczność zmniejszenia jamy syringomielicznej u pacjentów z tym typem jest znacznie wyższa niż u pacjentów z typem niemoniliformnym (p<0,001 w teście log-rank)¹⁰¹¹
Syringomyelia szyjna – szczególnie posiada potencjał dobrego powrotu funkcji po odpowiedniej interwencji¹²

Czynniki anatomiczne i radiologiczne

Badania identyfikują istotne czynniki radiologiczne wpływające na rokowanie:¹³¹⁴

Średnica jamy syringomielicznej – średnica przekraczająca 5 mm oraz towarzyszący obrzęk ogólnie wskazują na gorsze rokowanie¹⁵
Różnice między typem moniliformnym a niemoniliformnym obejmują:
- Czas trwania objawów przedoperacyjnych (p=0,029)
- Przestrzeń podpajęczynówkowa (SAS) po stronie brzusznej na poziomie otworu potylicznego (p<0,001)
- Wyprostowana fizjologiczna krzywizna szyi (p<0,001)

Wyniki leczenia operacyjnego

Skuteczność interwencji chirurgicznej

Leczenie operacyjne jest kluczowym elementem postępowania w syringomyelii, szczególnie w przypadkach związanych z wadą Chiari typu I. Dane wskazują na wysoką skuteczność zabiegów chirurgicznych:¹⁶¹⁷

Operacja wiąże się z pozytywnymi wynikami klinicznymi i radiologicznymi w ponad 90% przypadków
Wczesna interwencja chirurgiczna zazwyczaj minimalizuje objawy i daje lepsze wyniki¹⁸
W ogólnej ocenie rokowanie po operacji jest dobre – w jednym z badań stwierdzono korzystne rokowanie u 152 przypadków (75,25%), a jama syringomieliczna została skutecznie zresorbowana w 172 przypadkach (85,15%)¹⁹²⁰

Znaczenie czasu interwencji

Czas podjęcia leczenia operacyjnego ma istotne znaczenie dla wyników:²¹²²

Terminowa operacja dekompresyjna może osiągnąć lepszy wynik u pacjentów z wadą Chiari I z syringomyelią
Czas trwania objawów przedoperacyjnych jest niezależnym czynnikiem wpływającym na rokowanie u dorosłych
Pacjenci bez dysfunkcji autonomicznej lub upośledzenia motorycznego powinni otrzymać terminową interwencję chirurgiczną, aby zapobiec pogorszeniu objawów²³
Operacja powinna być proponowana jak najszybciej u dorosłych z syringomyelią z wadą Chiari I²⁴

Stabilizacja i poprawa po leczeniu

Po leczeniu chirurgicznym można zaobserwować następujące zjawiska:²⁵

Progresja deficytów neurologicznych zwykle stabilizuje się po leczeniu chirurgicznym
Objawy czasami ulegają poprawie, jednak pacjenci często pozostają objawowi

Przewidywanie wyników i algorytmy prognostyczne

Modele predykcyjne

Opracowano algorytmy oparte na punktacji, które mają dobrą wartość predykcyjną dla pooperacyjnych wyników wieloczynnikowych u pacjentów z wadą Chiari I z syringomyelią.²⁶ Modele te uwzględniają różne czynniki prognostyczne i mogą pomóc klinicystom w podejmowaniu decyzji terapeutycznych.

Kliniczne predyktory w diagnostyce

Badania wykazały również, że określone objawy kliniczne mogą służyć jako wiarygodne wskaźniki predykcyjne obecności istotnej klinicznie jamy syringomielicznej, co ma znaczenie dla wczesnej diagnozy i leczenia:²⁷

Obecność specyficznych objawów klinicznych, takich jak drapanie fantomowe, dwustronne drapanie szyi lub barku, awersja przy dotykaniu tego obszaru
Nasilenie objawów klinicznych w sytuacjach pobudzenia lub zdenerwowania
Dodatnia wartość predykcyjna jest jeszcze wyższa przy kombinacji drapania fantomowego, awersji na dotyk głowy, szyi lub barku oraz preferowanej pozycji głowy podczas snu

Potencjalne powikłania i historia naturalna

Naturalna historia syringomyelii wciąż nie jest dobrze poznana, co utrudnia dokładne prognozowanie wyników bez interwencji.²⁸ Potencjalne powikłania nieleczonej lub źle kontrolowanej syringomyelii obejmują:²⁹

Mielopatię (uszkodzenie rdzenia kręgowego z powodu ciężkiego ucisku) – główne powikłanie i obawa w syringomyelii
Paraplegia/tetraplegia – poważne deficyty neurologiczne mogące rozwinąć się z czasem
Nawracające zapalenie płuc – jako konsekwencja postępujących deficytów neurologicznych
Dysfunkcję jelit i pęcherza moczowego – częste powikłanie w zaawansowanych przypadkach

Chociaż starsze badania sugerowały, że 20% pacjentów umierało w średnim wieku 47 lat, współczesne wskaźniki śmiertelności są prawdopodobnie niższe dzięki interwencjom chirurgicznym i lepszemu leczeniu powikłań związanych ze znacznym niedowładem.³⁰

Przyszłe kierunki badań

Mimo znacznych postępów w zrozumieniu czynników prognostycznych syringomyelii, nadal istnieje potrzeba dalszych badań:³¹

Potrzebne są dalsze doświadczenia na większych kohortach, aby lepiej zdefiniować wartość prognostyczną markerów radiologicznych, takich jak przepływ CSF na poziomie CCJ
Konieczne jest lepsze zrozumienie naturalnej historii syringomyelii, zwłaszcza w przypadkach bez interwencji chirurgicznej
Badania nad nowymi biomarkerami i technikami obrazowania mogą poprawić zdolność przewidywania wyników leczenia

Kolejne rozdziały

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Materiały źródłowe

#1 Syringomyelia: What It Is, Symptoms, Causes & Treatment
https://my.clevelandclinic.org/health/diseases/6126-syringomyelia
Its important to remember that syringomyelia has a wide range of symptoms, severity and causes. In addition, people can have different rates of progression of the condition or no progression at all. These factors make it difficult for healthcare providers to predict the prognosis (outlook) for syringomyelia. […] Early surgery usually minimizes symptoms and has better outcomes. Although the progression of neurologic deficits usually stabilizes after surgical treatment, and the symptoms sometimes improve, people often remain symptomatic. […] In general, a syrinx diameter of more than five millimeters (mm) and associated swelling (edema) generally indicate a worse prognosis. […] Myelopathy (injury to your spinal cord due to severe compression) is a major complication and concern of syringomyelia. It can eventually lead to paraplegia/quadriplegia, recurrent pneumonia and bowel and bladder dysfunction.
#2 Syringomyelia: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/1151685-overview
Prognosis depends on the underlying cause, the magnitude of neurological dysfunction, and the location and extension of the syrinx. […] Patients presenting with moderate or severe neurological deficits fare much worse than those patients with mild deficits. […] Patients with central cord syndrome have poor response to treatment. […] Natural history of syringomyelia still is not well understood. […] Although older studies had suggested that 20% of patients died at an average age of 47 years, mortality rates are likely lower in today’s patients as a result of surgical interventions and better treatment of complications associated with significant paresis, such as pulmonary embolism.
#3 Outcome predictors and clinical presentation of syringomyelia – PubMed
https://pubmed.ncbi.nlm.nih.gov/35868963/
Background: The prognosis of syringomyelia is not yet established. Syringomyelia derived from different etiologies contributes to similar symptoms. […] In univariate analysis, autonomic dysfunction and motor impairment were strong predictors of poor neurological and functional outcomes. […] Motor impairment, which is commonly seen in patients with syringomyelia in Taiwan, is a strong predictor to poor neurological and functional outcomes. […] Our study indicates that patients without autonomic dysfunction or motor impairment should receive timely surgical intervention to prevent symptomatic deterioration. […] We also found that cervical syringomyelia in particular has the potential for good functional recovery after adequate intervention.
#4 A points-based algorithm for prognosticating clinical outcome of Chiari malformation Type I with syringomyelia: results from a predictive model analysis of 82 surgically managed adult patients in: Journal of Neurosurgery: Spine Volume 28 Issue 1 (2017) Jo
https://thejns.org/spine/view/journals/j-neurosurg-spine/28/1/article-p23.xml
The presence of gait imbalance and motor deficits independently predict worse clinical and radiological outcomes, respectively, after decompressive surgery for CMI with altered hindbrain CSF flow. […] The proposed points-based algorithm has good predictive value for postoperative multifactorial outcome in these patients.
#5 Syringomyelia: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/1151685-overview
Prognosis depends on the underlying cause, the magnitude of neurological dysfunction, and the location and extension of the syrinx. […] Patients presenting with moderate or severe neurological deficits fare much worse than those patients with mild deficits. […] Patients with central cord syndrome have poor response to treatment. […] Natural history of syringomyelia still is not well understood. […] Although older studies had suggested that 20% of patients died at an average age of 47 years, mortality rates are likely lower in today’s patients as a result of surgical interventions and better treatment of complications associated with significant paresis, such as pulmonary embolism.
#6 Syringomyelia: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/1151685-overview
Prognosis depends on the underlying cause, the magnitude of neurological dysfunction, and the location and extension of the syrinx. […] Patients presenting with moderate or severe neurological deficits fare much worse than those patients with mild deficits. […] Patients with central cord syndrome have poor response to treatment. […] Natural history of syringomyelia still is not well understood. […] Although older studies had suggested that 20% of patients died at an average age of 47 years, mortality rates are likely lower in today’s patients as a result of surgical interventions and better treatment of complications associated with significant paresis, such as pulmonary embolism.
#7 Syringomyelia Associated with Chiari 1 Malformation in Adults: Positive Outcome Predictors after Posterior Fossa Decompression with Duraplasty
https://pmc.ncbi.nlm.nih.gov/articles/PMC10143011/
Surgery was associated with positive clinical and radiological outcomes in more than 90% of cases. […] The presurgical presence of CSF flow at the CCJ was able to predict a good outcome (AUC = 0.68, 95% CI 0.500.87 and LH+ = 2.1, IC 95% 1.163.07) and was also significantly associated with post-surgical pain relief (rho = 0.61 and p = 0.0144). […] Presurgery CSF flow at the CCJ is proposed as a radiological marker with the ability to predict a positive outcome after PFDD in adults with syringomyelia and CM1. […] Among the demographic variables, age at surgery was able to predict a good outcome. In particular, undergoing surgery before 47 years of age was found to be a significant predictor of a good outcome (AUC = 0.71, 95% CI 0.540.89), increasing the probability of a good outcome by about six times (OR = 5.95, IC 95% 1.0334.4).
#8 Syringomyelia Associated with Chiari 1 Malformation in Adults: Positive Outcome Predictors after Posterior Fossa Decompression with Duraplasty
https://www.mdpi.com/2077-0383/12/8/3019
Syringomyelia (Syr) in patients with Chiari 1 malformation (CM1) may be attributable to abnormal dynamics of cerebrospinal fluid (CSF) in the upper cervical segment; fourth ventricle enlargement has been reported in association with a worse clinical and radiological presentation, independently of the posterior fossa volume. […] As a primary endpoint, we aimed to correlate improvement in the fourth ventricle area with positive clinical outcomes. […] Surgery was associated with positive clinical and radiological outcomes in more than 90% of cases. […] The presurgical presence of CSF flow at the CCJ was able to predict a good outcome (AUC = 0.68, 95% CI 0.50â0.87 and LH+ = 2.1, IC 95% 1.16â3.07) and was also significantly associated with post-surgical pain relief (rho = 0.61 and p = 0.0144).
#9 Syringomyelia Associated with Chiari 1 Malformation in Adults: Positive Outcome Predictors after Posterior Fossa Decompression with Duraplasty
https://www.mdpi.com/2077-0383/12/8/3019
Presurgery CSF flow at the CCJ is proposed as a radiological marker with the ability to predict a positive outcome after PFDD in adults with syringomyelia and CM1. […] A young age at surgery (<47 years) was a significant good outcome predictor; according to these results, as also proposed by Aghakani et al., surgery should be proposed as soon as possible in adults affected by syringomyelia with CM1.
#10 Phenotypes and Prognostic Factors of Syringomyelia in Single-Center Patients With Chiari I Malformation: Moniliform Type as a Special Configuration
https://www.e-neurospine.org/journal/view.php?year=2022&vol=19&no=3&startpage=816
Timely decompression surgery could achieve a better outcome in CM-I patients with syringomyelia. Moniliform syringomyelia may suggest a relatively better prognosis. […] The overall prognosis after surgery was good in our series, among them 152 cases (75.25%) with a favorable prognosis, and syrinx was resolved effectively in 172 cases (85.15%). […] According to the univariate and multivariate analyses, the preoperative symptom duration, observation time, and with/without moniliform type were independent factors affecting the prognosis in adults. […] The effectiveness of syrinx resolution in patients with moniliform type was significantly higher than that with the nonmoniliform type (p0.001 by log-rank test) in the adult group. […] The most obvious difference between moniliform type and nonmoniliform type lies in the preoperative symptom duration (p=0.029), ventral SAS at the FM (p0.001), and the patients with straightened cervical physio-curve or not (p0.001).
#11 Phenotypes and Prognostic Factors of Syringomyelia in Single-Center Patients With Chiari I Malformation: Moniliform Type as a Special Configuration
https://www.e-neurospine.org/journal/view.php?number=1305
Timely decompression surgery could achieve a better outcome in CM-I patients with syringomyelia. […] Moniliform syringomyelia may suggest a relatively better prognosis. […] The overall prognosis after surgery was good in our series, among them 152 cases (75.25%) with a favorable prognosis, and syrinx was resolved effectively in 172 cases (85.15%). […] According to the univariate and multivariate analyses, the preoperative symptom duration, observation time, and with/without moniliform type were independent factors affecting the prognosis in adults. […] The effectiveness of syrinx resolution in patients with moniliform type was significantly higher than that with the nonmoniliform type (p0.001 by log-rank test) in the adult group. […] The most obvious difference between moniliform type and nonmoniliform type lies in the preoperative symptom duration (p=0.029), ventral SAS at the FM (p0.001), and the patients with straightened cervical physio-curve or not (p0.001).
#12 Outcome predictors and clinical presentation of syringomyelia – PubMed
https://pubmed.ncbi.nlm.nih.gov/35868963/
Background: The prognosis of syringomyelia is not yet established. Syringomyelia derived from different etiologies contributes to similar symptoms. […] In univariate analysis, autonomic dysfunction and motor impairment were strong predictors of poor neurological and functional outcomes. […] Motor impairment, which is commonly seen in patients with syringomyelia in Taiwan, is a strong predictor to poor neurological and functional outcomes. […] Our study indicates that patients without autonomic dysfunction or motor impairment should receive timely surgical intervention to prevent symptomatic deterioration. […] We also found that cervical syringomyelia in particular has the potential for good functional recovery after adequate intervention.
#13 Phenotypes and Prognostic Factors of Syringomyelia in Single-Center Patients With Chiari I Malformation: Moniliform Type as a Special Configuration
https://www.e-neurospine.org/journal/view.php?year=2022&vol=19&no=3&startpage=816
Timely decompression surgery could achieve a better outcome in CM-I patients with syringomyelia. Moniliform syringomyelia may suggest a relatively better prognosis. […] The overall prognosis after surgery was good in our series, among them 152 cases (75.25%) with a favorable prognosis, and syrinx was resolved effectively in 172 cases (85.15%). […] According to the univariate and multivariate analyses, the preoperative symptom duration, observation time, and with/without moniliform type were independent factors affecting the prognosis in adults. […] The effectiveness of syrinx resolution in patients with moniliform type was significantly higher than that with the nonmoniliform type (p0.001 by log-rank test) in the adult group. […] The most obvious difference between moniliform type and nonmoniliform type lies in the preoperative symptom duration (p=0.029), ventral SAS at the FM (p0.001), and the patients with straightened cervical physio-curve or not (p0.001).
#14 Phenotypes and Prognostic Factors of Syringomyelia in Single-Center Patients With Chiari I Malformation: Moniliform Type as a Special Configuration
https://www.e-neurospine.org/journal/view.php?number=1305
Timely decompression surgery could achieve a better outcome in CM-I patients with syringomyelia. […] Moniliform syringomyelia may suggest a relatively better prognosis. […] The overall prognosis after surgery was good in our series, among them 152 cases (75.25%) with a favorable prognosis, and syrinx was resolved effectively in 172 cases (85.15%). […] According to the univariate and multivariate analyses, the preoperative symptom duration, observation time, and with/without moniliform type were independent factors affecting the prognosis in adults. […] The effectiveness of syrinx resolution in patients with moniliform type was significantly higher than that with the nonmoniliform type (p0.001 by log-rank test) in the adult group. […] The most obvious difference between moniliform type and nonmoniliform type lies in the preoperative symptom duration (p=0.029), ventral SAS at the FM (p0.001), and the patients with straightened cervical physio-curve or not (p0.001).
#15 Syringomyelia: What It Is, Symptoms, Causes & Treatment
https://my.clevelandclinic.org/health/diseases/6126-syringomyelia
Its important to remember that syringomyelia has a wide range of symptoms, severity and causes. In addition, people can have different rates of progression of the condition or no progression at all. These factors make it difficult for healthcare providers to predict the prognosis (outlook) for syringomyelia. […] Early surgery usually minimizes symptoms and has better outcomes. Although the progression of neurologic deficits usually stabilizes after surgical treatment, and the symptoms sometimes improve, people often remain symptomatic. […] In general, a syrinx diameter of more than five millimeters (mm) and associated swelling (edema) generally indicate a worse prognosis. […] Myelopathy (injury to your spinal cord due to severe compression) is a major complication and concern of syringomyelia. It can eventually lead to paraplegia/quadriplegia, recurrent pneumonia and bowel and bladder dysfunction.
#16 Syringomyelia Associated with Chiari 1 Malformation in Adults: Positive Outcome Predictors after Posterior Fossa Decompression with Duraplasty
https://pmc.ncbi.nlm.nih.gov/articles/PMC10143011/
Surgery was associated with positive clinical and radiological outcomes in more than 90% of cases. […] The presurgical presence of CSF flow at the CCJ was able to predict a good outcome (AUC = 0.68, 95% CI 0.500.87 and LH+ = 2.1, IC 95% 1.163.07) and was also significantly associated with post-surgical pain relief (rho = 0.61 and p = 0.0144). […] Presurgery CSF flow at the CCJ is proposed as a radiological marker with the ability to predict a positive outcome after PFDD in adults with syringomyelia and CM1. […] Among the demographic variables, age at surgery was able to predict a good outcome. In particular, undergoing surgery before 47 years of age was found to be a significant predictor of a good outcome (AUC = 0.71, 95% CI 0.540.89), increasing the probability of a good outcome by about six times (OR = 5.95, IC 95% 1.0334.4).
#17 Syringomyelia Associated with Chiari 1 Malformation in Adults: Positive Outcome Predictors after Posterior Fossa Decompression with Duraplasty
https://www.mdpi.com/2077-0383/12/8/3019
Syringomyelia (Syr) in patients with Chiari 1 malformation (CM1) may be attributable to abnormal dynamics of cerebrospinal fluid (CSF) in the upper cervical segment; fourth ventricle enlargement has been reported in association with a worse clinical and radiological presentation, independently of the posterior fossa volume. […] As a primary endpoint, we aimed to correlate improvement in the fourth ventricle area with positive clinical outcomes. […] Surgery was associated with positive clinical and radiological outcomes in more than 90% of cases. […] The presurgical presence of CSF flow at the CCJ was able to predict a good outcome (AUC = 0.68, 95% CI 0.50â0.87 and LH+ = 2.1, IC 95% 1.16â3.07) and was also significantly associated with post-surgical pain relief (rho = 0.61 and p = 0.0144).
#18 Syringomyelia: What It Is, Symptoms, Causes & Treatment
https://my.clevelandclinic.org/health/diseases/6126-syringomyelia
Its important to remember that syringomyelia has a wide range of symptoms, severity and causes. In addition, people can have different rates of progression of the condition or no progression at all. These factors make it difficult for healthcare providers to predict the prognosis (outlook) for syringomyelia. […] Early surgery usually minimizes symptoms and has better outcomes. Although the progression of neurologic deficits usually stabilizes after surgical treatment, and the symptoms sometimes improve, people often remain symptomatic. […] In general, a syrinx diameter of more than five millimeters (mm) and associated swelling (edema) generally indicate a worse prognosis. […] Myelopathy (injury to your spinal cord due to severe compression) is a major complication and concern of syringomyelia. It can eventually lead to paraplegia/quadriplegia, recurrent pneumonia and bowel and bladder dysfunction.
#19 Phenotypes and Prognostic Factors of Syringomyelia in Single-Center Patients With Chiari I Malformation: Moniliform Type as a Special Configuration
https://www.e-neurospine.org/journal/view.php?year=2022&vol=19&no=3&startpage=816
Timely decompression surgery could achieve a better outcome in CM-I patients with syringomyelia. Moniliform syringomyelia may suggest a relatively better prognosis. […] The overall prognosis after surgery was good in our series, among them 152 cases (75.25%) with a favorable prognosis, and syrinx was resolved effectively in 172 cases (85.15%). […] According to the univariate and multivariate analyses, the preoperative symptom duration, observation time, and with/without moniliform type were independent factors affecting the prognosis in adults. […] The effectiveness of syrinx resolution in patients with moniliform type was significantly higher than that with the nonmoniliform type (p0.001 by log-rank test) in the adult group. […] The most obvious difference between moniliform type and nonmoniliform type lies in the preoperative symptom duration (p=0.029), ventral SAS at the FM (p0.001), and the patients with straightened cervical physio-curve or not (p0.001).
#20 Phenotypes and Prognostic Factors of Syringomyelia in Single-Center Patients With Chiari I Malformation: Moniliform Type as a Special Configuration
https://www.e-neurospine.org/journal/view.php?number=1305
Timely decompression surgery could achieve a better outcome in CM-I patients with syringomyelia. […] Moniliform syringomyelia may suggest a relatively better prognosis. […] The overall prognosis after surgery was good in our series, among them 152 cases (75.25%) with a favorable prognosis, and syrinx was resolved effectively in 172 cases (85.15%). […] According to the univariate and multivariate analyses, the preoperative symptom duration, observation time, and with/without moniliform type were independent factors affecting the prognosis in adults. […] The effectiveness of syrinx resolution in patients with moniliform type was significantly higher than that with the nonmoniliform type (p0.001 by log-rank test) in the adult group. […] The most obvious difference between moniliform type and nonmoniliform type lies in the preoperative symptom duration (p=0.029), ventral SAS at the FM (p0.001), and the patients with straightened cervical physio-curve or not (p0.001).
#21 Phenotypes and Prognostic Factors of Syringomyelia in Single-Center Patients With Chiari I Malformation: Moniliform Type as a Special Configuration
https://www.e-neurospine.org/journal/view.php?year=2022&vol=19&no=3&startpage=816
Timely decompression surgery could achieve a better outcome in CM-I patients with syringomyelia. Moniliform syringomyelia may suggest a relatively better prognosis. […] The overall prognosis after surgery was good in our series, among them 152 cases (75.25%) with a favorable prognosis, and syrinx was resolved effectively in 172 cases (85.15%). […] According to the univariate and multivariate analyses, the preoperative symptom duration, observation time, and with/without moniliform type were independent factors affecting the prognosis in adults. […] The effectiveness of syrinx resolution in patients with moniliform type was significantly higher than that with the nonmoniliform type (p0.001 by log-rank test) in the adult group. […] The most obvious difference between moniliform type and nonmoniliform type lies in the preoperative symptom duration (p=0.029), ventral SAS at the FM (p0.001), and the patients with straightened cervical physio-curve or not (p0.001).
#22 Phenotypes and Prognostic Factors of Syringomyelia in Single-Center Patients With Chiari I Malformation: Moniliform Type as a Special Configuration
https://www.e-neurospine.org/journal/view.php?number=1305
Timely decompression surgery could achieve a better outcome in CM-I patients with syringomyelia. […] Moniliform syringomyelia may suggest a relatively better prognosis. […] The overall prognosis after surgery was good in our series, among them 152 cases (75.25%) with a favorable prognosis, and syrinx was resolved effectively in 172 cases (85.15%). […] According to the univariate and multivariate analyses, the preoperative symptom duration, observation time, and with/without moniliform type were independent factors affecting the prognosis in adults. […] The effectiveness of syrinx resolution in patients with moniliform type was significantly higher than that with the nonmoniliform type (p0.001 by log-rank test) in the adult group. […] The most obvious difference between moniliform type and nonmoniliform type lies in the preoperative symptom duration (p=0.029), ventral SAS at the FM (p0.001), and the patients with straightened cervical physio-curve or not (p0.001).
#23 Outcome predictors and clinical presentation of syringomyelia – PubMed
https://pubmed.ncbi.nlm.nih.gov/35868963/
Background: The prognosis of syringomyelia is not yet established. Syringomyelia derived from different etiologies contributes to similar symptoms. […] In univariate analysis, autonomic dysfunction and motor impairment were strong predictors of poor neurological and functional outcomes. […] Motor impairment, which is commonly seen in patients with syringomyelia in Taiwan, is a strong predictor to poor neurological and functional outcomes. […] Our study indicates that patients without autonomic dysfunction or motor impairment should receive timely surgical intervention to prevent symptomatic deterioration. […] We also found that cervical syringomyelia in particular has the potential for good functional recovery after adequate intervention.
#24 Syringomyelia Associated with Chiari 1 Malformation in Adults: Positive Outcome Predictors after Posterior Fossa Decompression with Duraplasty
https://www.mdpi.com/2077-0383/12/8/3019
Presurgery CSF flow at the CCJ is proposed as a radiological marker with the ability to predict a positive outcome after PFDD in adults with syringomyelia and CM1. […] A young age at surgery (<47 years) was a significant good outcome predictor; according to these results, as also proposed by Aghakani et al., surgery should be proposed as soon as possible in adults affected by syringomyelia with CM1.
#25 Syringomyelia: What It Is, Symptoms, Causes & Treatment
https://my.clevelandclinic.org/health/diseases/6126-syringomyelia
Its important to remember that syringomyelia has a wide range of symptoms, severity and causes. In addition, people can have different rates of progression of the condition or no progression at all. These factors make it difficult for healthcare providers to predict the prognosis (outlook) for syringomyelia. […] Early surgery usually minimizes symptoms and has better outcomes. Although the progression of neurologic deficits usually stabilizes after surgical treatment, and the symptoms sometimes improve, people often remain symptomatic. […] In general, a syrinx diameter of more than five millimeters (mm) and associated swelling (edema) generally indicate a worse prognosis. […] Myelopathy (injury to your spinal cord due to severe compression) is a major complication and concern of syringomyelia. It can eventually lead to paraplegia/quadriplegia, recurrent pneumonia and bowel and bladder dysfunction.
#26 A points-based algorithm for prognosticating clinical outcome of Chiari malformation Type I with syringomyelia: results from a predictive model analysis of 82 surgically managed adult patients in: Journal of Neurosurgery: Spine Volume 28 Issue 1 (2017) Jo
https://thejns.org/spine/view/journals/j-neurosurg-spine/28/1/article-p23.xml
The presence of gait imbalance and motor deficits independently predict worse clinical and radiological outcomes, respectively, after decompressive surgery for CMI with altered hindbrain CSF flow. […] The proposed points-based algorithm has good predictive value for postoperative multifactorial outcome in these patients.
#27 Clinical predictors of syringomyelia in Cavalier King Charles Spaniels with chiari-like malformation based on ownersâ observations | Acta Veterinaria Scandinavica | Full Text
https://actavetscand.biomedcentral.com/articles/10.1186/s13028-024-00725-1
Specific clinical signs can be used individually and in combination as clinical predictors of a large clinical syrinx in CKCSs with CM and SM. […] The presence of one of the following clinical signs; phantom scratching, bilateral scratching of the neck or shoulder, aversion when that area is touched, or exacerbation of clinical signs when the dog is excited or nervous, each serves as a reliable predictive indicator of CM and a large and clinically relevant syrinx in CKCSs. […] The positive predictive value is even higher when a combination of phantom scratching, aversion to touch to the head, neck or shoulder, and a preferred head posture during sleep are all present in the same dog. […] The information coming from our study is particularly valuable for general practitioners who can use the specific clinical signs, individually or in combination, as a clinical predictor of a large clinically relevant syrinx.
#28 Syringomyelia: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/1151685-overview
Prognosis depends on the underlying cause, the magnitude of neurological dysfunction, and the location and extension of the syrinx. […] Patients presenting with moderate or severe neurological deficits fare much worse than those patients with mild deficits. […] Patients with central cord syndrome have poor response to treatment. […] Natural history of syringomyelia still is not well understood. […] Although older studies had suggested that 20% of patients died at an average age of 47 years, mortality rates are likely lower in today’s patients as a result of surgical interventions and better treatment of complications associated with significant paresis, such as pulmonary embolism.
#29 Syringomyelia: What It Is, Symptoms, Causes & Treatment
https://my.clevelandclinic.org/health/diseases/6126-syringomyelia
Its important to remember that syringomyelia has a wide range of symptoms, severity and causes. In addition, people can have different rates of progression of the condition or no progression at all. These factors make it difficult for healthcare providers to predict the prognosis (outlook) for syringomyelia. […] Early surgery usually minimizes symptoms and has better outcomes. Although the progression of neurologic deficits usually stabilizes after surgical treatment, and the symptoms sometimes improve, people often remain symptomatic. […] In general, a syrinx diameter of more than five millimeters (mm) and associated swelling (edema) generally indicate a worse prognosis. […] Myelopathy (injury to your spinal cord due to severe compression) is a major complication and concern of syringomyelia. It can eventually lead to paraplegia/quadriplegia, recurrent pneumonia and bowel and bladder dysfunction.
#30 Syringomyelia: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/1151685-overview
Prognosis depends on the underlying cause, the magnitude of neurological dysfunction, and the location and extension of the syrinx. […] Patients presenting with moderate or severe neurological deficits fare much worse than those patients with mild deficits. […] Patients with central cord syndrome have poor response to treatment. […] Natural history of syringomyelia still is not well understood. […] Although older studies had suggested that 20% of patients died at an average age of 47 years, mortality rates are likely lower in today’s patients as a result of surgical interventions and better treatment of complications associated with significant paresis, such as pulmonary embolism.
#31 [ë¼ë¬¸]Syringomyelia Associated with Chiari 1 Malformation in Adults: Positive Outcome Predictors after Posterior Fossa Decompression with Duraplasty
https://scienceon.kisti.re.kr/srch/selectPORSrchArticle.do?cn=NART124451589
Syringomyelia (Syr) in patients with Chiari 1 malformation (CM1) may be attributable to abnormal dynamics of cerebrospinal fluid (CSF) in the upper cervical segment; fourth ventricle enlargement has been reported in association with a worse clinical and radiological presentation, independently of the posterior fossa volume. […] Surgery was associated with positive clinical and radiological outcomes in more than 90% of cases. […] The presurgical presence of CSF flow at the CCJ was able to predict a good outcome (AUC = 0.68, 95% CI 0.500.87 and LH+ = 2.1, IC 95% 1.163.07) and was also significantly associated with post-surgical pain relief (rho = 0.61 and p = 0.0144). […] Presurgery CSF flow at the CCJ is proposed as a radiological marker with the ability to predict a positive outcome after PFDD in adults with syringomyelia and CM1. […] Further experience on larger cohorts is required to better define the prognostic yield of this radiological parameter.