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1.
PLoS One ; 18(1): e0278665, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36649366

RESUMO

Our knowledge about the underlying pathomechanisms of craniocervical junction abnormalities (CCJA) in dogs mostly derives from measurements based on tomographic imaging. These images are static and the positioning of the dogs' head does not reflect the physiological in vivo position of the craniocervical junction (CCJ). Aberrant motion patterns and ranges of motion (ROM) in sound individuals of CCJA predisposed breeds may be a pathogenetic trigger. To further extend our limited knowledge of physiological motion of the CCJ, this prospective, comparative study investigates the in vivo motion patterns and ROM of the CCJ in walk and trot in sound Cavalier King Charles Spaniels and Chihuahuas. The Labrador retriever is used as a reference breed without predisposition for CCJA. This is the first detailed description of CCJ movement of trotting dogs. Biplanar fluoroscopy images, recorded in walking and trotting dogs, were matched to a virtual reconstruction of the skull and cranial cervical spine utilising Scientific Rotoscoping. Kinematic data reveal the same motion patterns among all breeds and gaits with individual temporal and spatial differences in each dog. A stride cycle-dependent lateral rotation of the cranial cervical spine and axial rotation of the atlantoaxial joint in trot in dogs is described for the first time. The ROM of the atlantoaxial and atlantooccipital joints in walk and trot were not statistically significantly greater in the CCJA-predisposed breeds CKCS and Chihuahua. ROM values of all translational and rotational degrees of freedom were larger in walk than trot, although this is only statistically significant for the atlantoaxial joint. Until proven otherwise, a more species-specific than breed-specific general motion pattern of the CCJ in walking and trotting, clinically sound dogs must be assumed. Species-specific anatomic properties of the CCJ seem to supersede breed-specific anatomical differences in clinically sound dogs.


Assuntos
Doenças do Cão , Siringomielia , Cães , Animais , Estudos Prospectivos , Imageamento por Ressonância Magnética/veterinária , Fenômenos Biomecânicos , Crânio/diagnóstico por imagem , Crânio/patologia , Doenças do Cão/diagnóstico por imagem , Doenças do Cão/patologia
2.
Medicine (Baltimore) ; 101(50): e31394, 2022 Dec 16.
Artigo em Inglês | MEDLINE | ID: mdl-36550873

RESUMO

BACKGROUND: The current surgical management of adult Chiari malformation type I (CM-I) with associated syringomyelia remains controversial. The objective of this study was to explore posterior fossa decompression and duraplasty (PFDD) with and without tonsillar resection in adult patients with CM-I and syringomyelia. METHODS: A total of 116 adult patients suffering from both CM-I and syringomyelia who were scheduled to undergo surgical decompression at our institution between 2012 and 2020 were randomly divided into 2 groups: the PFDD group (n = 64) underwent PFDD without tonsillar resection, while the PFDD-T group (n = 52) underwent PFDD with tonsillar resection. The primary outcome was improvement or resolution of the syrinx. The secondary outcome was an improvement in clinical outcome based on Chicago Chiari Outcome Scale (CCOS) scores. All participants were followed-up to 1-year postoperatively. RESULTS: The proportions of patients who had >20% improvement in syrinx size were 60.9% and 78.8% in the PFDD and PFDD-T groups, respectively (P = .038). The improvement in clinical outcome based on CCOS scores was significantly different in the 2 groups (P = .004). The functionality sub-score was significantly different between the 2 groups (P = .027), but there were no significant differences in the pain symptoms, non-pain symptoms, and complications sub-scores. The total CCOS scores were higher in the PFDD-T group than in the PFDD group (P = .037). CONCLUSION: This study determined the role of tonsillar resection in achieving obvious syrinx improvement following PFDD-T. PFDD with tonsillar resection seems to be a safe and effective surgical option to treat adult CM-I patients with syringomyelia.


Assuntos
Malformação de Arnold-Chiari , Siringomielia , Humanos , Adulto , Malformação de Arnold-Chiari/complicações , Malformação de Arnold-Chiari/cirurgia , Siringomielia/complicações , Siringomielia/cirurgia , Dura-Máter/cirurgia , Descompressão Cirúrgica , Resultado do Tratamento , Estudos Retrospectivos
3.
Neurocirugía (Soc. Luso-Esp. Neurocir.) ; 33(6): 366-370, nov.-dic. 2022. ilus
Artigo em Inglês | IBECS | ID: ibc-212997

RESUMO

Dorsal arachnoidal webs are condensations of arachnoidal tissue densely adherent to the pial surface of the posterior aspect of the dorsal spinal cord. Infrequently described in literature they disrupt the flow of CSF in the spinal subarachnoid space leading to syringomyelia and myelopathy. While there are several theories on their origin, the “scalpel sign” on magnetic resonance imaging is considered to be pathognomonic of this condition. An illustrative case of a 58 year old man with syringomyelia and dorsal cord indentation who presented with spastic paraparesis, gait instability, parasthesias and bilateral non radicular upper limb pain that resolved following excision of the web is described to highlight the importance of considering this diagnosis when cases of so called “idiopathic” syringomyelia are encountered (AU)


Las membranas aracnoideas dorsales son condensaciones de tejido aracnoideo densamente adherido a la superficie pial de la cara posterior de la médula espinal dorsal. Descritas con poca frecuencia en la literatura, interrumpen el flujo del líquido cefalorraquídeo (LCR) en el espacio subaracnoideo espinal y provocan siringomielia y mielopatía. Si bien existen varias teorías sobre su origen, el «signo del bisturí» en la resonancia magnética se considera patognomónico de esta afección. Se describe un caso ilustrativo de un varón de 58 años con siringomielia e indentación medular dorsal que presentó paraparesia espástica, inestabilidad de la marcha, parestesias y dolor bilateral no radicular en miembro superior que se resolvió tras la exéresis de la telaraña para resaltar la importancia de considerar este diagnóstico cuando se encuentran casos de la llamada siringomielia «idiopática» (AU)


Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Siringomielia/diagnóstico por imagem , Siringomielia/etiologia , Parestesia/etiologia , Telangiectasia/complicações , Imageamento por Ressonância Magnética , Telangiectasia/diagnóstico por imagem , Telangiectasia/cirurgia
4.
BMC Musculoskelet Disord ; 23(1): 1138, 2022 Dec 29.
Artigo em Inglês | MEDLINE | ID: mdl-36581884

RESUMO

BACKGROUND: This study reports on the surgical technique used and clinical outcomes obtained during the treatment of basilar invagination (BI) without atlantoaxial dislocation (AAD) through the correction of the clivus canal angle (CCA) using interfacet distraction and fixation. METHODS: Nineteen cases with BI without AAD treated by the correction of the clivus canal angle were retrospectively analyzed. Pre- and postoperative computed tomography scans and three-dimensional reconstruction views were obtained to measure the size of the CCA, pB-C2 distance, and degree of BI. Chiari malformation and syringomyelia were evaluated by magnetic resonance imaging (MRI). The clinical outcomes for all patients were measured using the Japanese Orthopedic Association (JOA) scale. The CCA was corrected by using interfacet distraction and fixation techniques. The Wilcoxon test was used to compare pre- and postoperative measurements. RESULTS: All the patients were followed up for 24.95 ± 5.22 months (range 12-36 months); no patient suffered intraoperative nerve or vascular injury. Clinical symptoms improved in 17 patients (89.5%). The mean JOA score increased from 12.32 ± 1.89 to 14.37 ± 1.30 (Z = -3.655, P < 0.001). The mean CCA improved from 129.34 ± 8.52° preoperatively to 139.75 ± 8.86° postoperatively (Z = -3.824, P < 0.001). The mean pB-C2 decreased from 7.47 ± 2.21 to 5.68 ± 3.13 (Z = -3.060, P = 0.002). Syringomyelia was significantly reduced in 10 out of 13 patients by the first follow-up year. All patients achieved bony fusion. CONCLUSION: Posterior interfacet distraction and fixation to correct the CCA is a feasible and effective method for treating BI without AAD.


Assuntos
Articulação Atlantoaxial , Luxações Articulares , Fusão Vertebral , Siringomielia , Humanos , Estudos Retrospectivos , Siringomielia/diagnóstico por imagem , Siringomielia/cirurgia , Siringomielia/complicações , Articulação Atlantoaxial/diagnóstico por imagem , Articulação Atlantoaxial/cirurgia , Articulação Atlantoaxial/lesões , Luxações Articulares/diagnóstico por imagem , Luxações Articulares/cirurgia , Luxações Articulares/complicações , Descompressão Cirúrgica/métodos , Fusão Vertebral/métodos
5.
Neurocirugia (Astur : Engl Ed) ; 33(6): 366-370, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36333094

RESUMO

Dorsal arachnoidal webs are condensations of arachnoidal tissue densely adherent to the pial surface of the posterior aspect of the dorsal spinal cord. Infrequently described in literature they disrupt the flow of CSF in the spinal subarachnoid space leading to syringomyelia and myelopathy. While there are several theories on their origin, the "scalpel sign" on magnetic resonance imaging is considered to be pathognomonic of this condition. An illustrative case of a 58 year old man with syringomyelia and dorsal cord indentation who presented with spastic paraparesis, gait instability, parasthesias and bilateral non radicular upper limb pain that resolved following excision of the web is described to highlight the importance of considering this diagnosis when cases of so called "idiopathic" syringomyelia are encountered.


Assuntos
Siringomielia , Masculino , Humanos , Pessoa de Meia-Idade , Siringomielia/complicações , Siringomielia/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Internet , Parestesia/etiologia
7.
BMJ Case Rep ; 15(11)2022 Nov 25.
Artigo em Inglês | MEDLINE | ID: mdl-36428034

RESUMO

Syringomyelia is uncommonly revealed by a neuropathic arthropathy (Charcot joint) and characterised by its slow progression with late neurological symptoms. In this particular field, neuropathic arthropathies mainly affect the shoulders and the elbows.We report a new case of neuropathic arthropathy of the wrist caused by syringomyelia, because of the rarity of this condition as well as its uncommon location.Neuropathic arthropathy caused by syringomyelia is poorly published in the literature. This atypical entity should be considered in case of Charcot arthropathy associated with neurological loss, especially when it affects the upper limbs.


Assuntos
Artropatia Neurogênica , Siringomielia , Humanos , Siringomielia/complicações , Siringomielia/diagnóstico por imagem , Artropatia Neurogênica/diagnóstico por imagem , Artropatia Neurogênica/etiologia , Punho , Articulação do Punho/diagnóstico por imagem , Extremidade Superior
8.
Am J Case Rep ; 23: e937826, 2022 Oct 31.
Artigo em Inglês | MEDLINE | ID: mdl-36315459

RESUMO

BACKGROUND Patients with Arnold-Chiari Malformation I (CM-I) treated with foramen magnum decompression (FMD) can have ongoing neck pain, headaches, and other symptoms complicated by persistent syringomyelia, yet there is little research regarding treatment of these symptoms. CASE REPORT A 62-year-old woman with a history of residual syringomyelia following FMD and ventriculoperitoneal shunt for CM-I presented to a chiropractor with progressively worsening neck pain, occipital headache, upper extremity numbness and weakness, and gait abnormality, with a World Health Organization Quality of Life score (WHO-QOL) of 52%. Symptoms were improved by FMD 16 years prior, then progressively worsened, and had resisted other forms of treatment, including exercises, acupuncture, and medications. Examination by the chiropractor revealed upper extremity neurologic deficits, including grip strength. The chiropractor ordered whole spine magnetic resonance imaging, which demonstrated a persistent cervico-thoracic syrinx and findings of cervical spondylosis, and treated the patient using a multimodal approach, with gentle cervical spine mobilization, soft tissue manipulation, and core and finger muscle rehabilitative exercises. The patient responded positively, and at the 6-month follow-up her WHO-QOL score was 80%, her grip strength and forward head position had improved, and she was now able to eat using chopsticks. CONCLUSIONS This case highlights a patient with neck pain, headaches, and persistent syringomyelia after FMD for CM-I who improved following multimodal chiropractic and rehabilitative therapies. Given the limited, low-level evidence for these interventions in patients with persistent symptoms and syringomyelia after FMD, these therapies cannot be broadly recommended, yet could be considered on a case-by-case basis.


Assuntos
Malformação de Arnold-Chiari , Quiroprática , Siringomielia , Feminino , Humanos , Pessoa de Meia-Idade , Siringomielia/complicações , Siringomielia/terapia , Malformação de Arnold-Chiari/complicações , Malformação de Arnold-Chiari/terapia , Malformação de Arnold-Chiari/diagnóstico , Forame Magno/cirurgia , Qualidade de Vida , Cervicalgia/etiologia , Cervicalgia/terapia , Descompressão Cirúrgica/efeitos adversos , Descompressão Cirúrgica/métodos , Imageamento por Ressonância Magnética , Cefaleia/etiologia , Cefaleia/terapia , Resultado do Tratamento
9.
Biomed Res Int ; 2022: 9770323, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36277900

RESUMO

Objective: Combining the dynamics of cerebrospinal fluid, our study investigates the clinical effects of syringomyelia after the combination of fourth ventricle-subarachnoid shunt (FVSS) for recurrent Chiari (type I) malformations after cranial fossa decompression (foramen magnum decompression (FMD)). Methods: From December 2018 to December 2020, 15 patients with recurrent syringomyelia following posterior fossa decompression had FVSS surgery. Before and after the procedure, the clinical and imaging data of these individuals were retrospectively examined. Results: Following FVSS, none of the 15 patients experienced infection, nerve injury, shunt loss, or obstruction. 13 patients improved dramatically after surgery, while 2 patients improved significantly in the early postoperative period, but the primary symptoms returned 2 months later. The Japanese Orthopedic Association (JOA) score was 12.67 ± 3.95, which was considerably better than preoperatively (t = 3.69, P0.001). The MRI results revealed that the cavities in 13 patients were reduced by at least 50% compared to the cavities measured preoperatively. The shrinkage rate of syringomyelia was 86.67% (13/15). One patient's cavities nearly vanished following syringomyelia. The size of the cavity in the patient remain unchanged, and the cavity's maximal diameter was significantly smaller than the size measured preoperatively (P < 0.001) PC-MRI results indicated that the peak flow rate of cerebrospinal fluid at the central segment of the midbrain aqueduct and the foramen magnum in patients during systole and diastole were significantly reduced after surgery (P < 0.05). Conclusion: After posterior fossa decompression, FVSS can effectively restore the smooth circulation of cerebrospinal fluid and alleviate clinical symptoms in patients with recurrent Chiari (type I) malformation and syringomyelia. It is a highly effective way of treatment.


Assuntos
Malformação de Arnold-Chiari , Siringomielia , Humanos , Siringomielia/diagnóstico por imagem , Siringomielia/cirurgia , Malformação de Arnold-Chiari/diagnóstico por imagem , Malformação de Arnold-Chiari/cirurgia , Estudos Retrospectivos , Forame Magno/diagnóstico por imagem , Forame Magno/cirurgia , Espaço Subaracnóideo , Descompressão Cirúrgica/métodos , Imageamento por Ressonância Magnética/métodos , Resultado do Tratamento
12.
Neurosurg Rev ; 45(6): 3537-3550, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36180807

RESUMO

Several complications have been reported after the use of grafts for duraplasty following posterior fossa decompression for the treatment of Chiari malformation type I. This study aims to investigate the rate of meningitis after posterior fossa decompression using different types of grafts in patients with Chiari malformation type I and associated syringomyelia. The search was conducted using multiple databases, including PubMed, Scopus, Web of Science, and Embase. Data on the rate of meningitis, syrinx change, and rate of reoperation were extracted and investigated. Quality of evidence was assessed using the Newcastle-Ottawa scale. Nineteen studies were included in the final meta-analysis, encompassing 1404 patients and investigating autografts, synthetic grafts, allografts, and xenografts (bovine collagen, bovine pericardium, and pig pericardium). Autografts were associated with the lowest rate of meningitis (1%) compared to allografts, synthetic grafts, and xenografts (2%, 5%, and 8% respectively). Autografts were also associated with the lowest rate of reoperation followed by xenografts, allografts, and synthetic grafts (4%, 5%, 9%, and 10% respectively). On the other hand, allografts were associated with the highest rate of syrinx improvement (83%) in comparison to autografts and synthetic grafts (77%, and 79% respectively). Autografts were associated with the lowest meningitis, reoperation, and syrinx improvement rates. Furthermore, synthetic grafts were associated with the highest reoperation and xenografts with the highest rate of meningitis, whereas allografts were associated with the best syrinx improvement rate and second-best meningitis rate. Future studies comparing autografts and allografts are warranted to determine which carries the best clinical outcome.


Assuntos
Malformação de Arnold-Chiari , Meningite , Siringomielia , Humanos , Animais , Bovinos , Suínos , Malformação de Arnold-Chiari/cirurgia , Malformação de Arnold-Chiari/complicações , Siringomielia/cirurgia , Siringomielia/complicações , Descompressão Cirúrgica/efeitos adversos , Dura-Máter/cirurgia , Resultado do Tratamento , Meningite/epidemiologia , Meningite/etiologia , Meningite/cirurgia , Estudos Retrospectivos
13.
Clin Neurol Neurosurg ; 222: 107413, 2022 11.
Artigo em Inglês | MEDLINE | ID: mdl-36049402

RESUMO

BACKGROUND: Spontaneous resolution of syringomyelia has rarely been reported in the literature. Rarer still are cases wherein this process is associated with pregnancy and parturition. We review theories on syringomyelia development and spontaneous resolution to better understand the role pregnancy and parturition may play in both processes. METHODS: We present a 30-year-old female with MRI-confirmed spontaneous syrinx regression following caesarean delivery of a full-term pregnancy. We additionally review the literature to identify previously reported cases of spontaneous syrinx regression both independent of and associated with pregnancy. RESULTS: Including the present case, 39 cases describing spontaneous regression of syringomyelia have been reported in the literature, of which only four are associated with pregnancy and parturition. 75% of all reported cases were associated with type I Chiari malformation, though several disorders of the craniocervical junction and spinal canal were implicated. Complete syrinx regression was achieved in 33.3% of cases and 5% of cases described recurrence of syringomyelia following the spontaneous resolution. CONCLUSION: Syringomyelia likely develops due to disturbance of the physiologic flow of cerebrospinal fluid around the craniocervical junction and the obex. Several mechanisms including fissuring of the spinal cord parenchyma and reduction of subarachnoid scarring are likely involved in this process. In the setting of pregnancy, additional mechanisms surrounding the increased intraabdominal forces imparted by a growing fetus, Valsalva-like strain experienced during labor, and hemodynamic changes that occur to accommodate gestation are likely implicated. Nevertheless, patients should continue to be monitored periodically for syrinx recurrence.


Assuntos
Malformação de Arnold-Chiari , Siringomielia , Gravidez , Feminino , Humanos , Adulto , Siringomielia/complicações , Siringomielia/diagnóstico por imagem , Malformação de Arnold-Chiari/complicações , Malformação de Arnold-Chiari/diagnóstico por imagem , Espaço Subaracnóideo , Imageamento por Ressonância Magnética , Parto
14.
Childs Nerv Syst ; 38(11): 2119-2128, 2022 11.
Artigo em Inglês | MEDLINE | ID: mdl-35962220

RESUMO

PURPOSE: Multiple imaging parameters have been examined to estimate the presence of syrinx and the need for surgery in Chiari I patients (CM1); however, no consistent or definitive criteria have been proposed. The objective of this study was to review existing and identify novel radiological and clinical characteristics of CM1 patients that associate syrinx development and surgical intervention. METHODS: Patients with Chiari I malformation diagnosed on imaging between 0 and 18 years were retrospectively reviewed from January 1, 2007 to February 12, 2020. Participants were included if they had a baseline MRI of the head and spine prior to surgical intervention if required. Forty age-matched controls with cranial imaging were identified for comparison. Imaging parameters and clinical symptoms were recorded. RESULTS: A total of 122 CM1 patients were included in this study. Of the 122 patients, 28 (23%) had syrinx, and 27 (22%) had surgery. The following imaging parameters associated with syrinx and surgical intervention were identified: midbrain length (P < 0.001; P = 0.032), the obex position (P = 0.002; P < 0.001) and medullary kinking (P = 0.041; P < 0.001). Among the clinical features, the presence of overall pain (P = 0.017; P = 0.042), neck pain (P = 0.005; P = 0.027), and sensory dysfunction (P < 0.001) were found to be strongly associated with syrinx and surgery. CONCLUSION: While further investigation is needed, these specific radiological and clinical parameters should be considered when evaluating CM1 patients and may be used to guide further management.


Assuntos
Malformação de Arnold-Chiari , Siringomielia , Humanos , Criança , Estudos Retrospectivos , Siringomielia/diagnóstico por imagem , Siringomielia/cirurgia , Siringomielia/complicações , Malformação de Arnold-Chiari/complicações , Malformação de Arnold-Chiari/diagnóstico por imagem , Malformação de Arnold-Chiari/cirurgia , Coluna Vertebral/cirurgia , Descompressão Cirúrgica/métodos , Imageamento por Ressonância Magnética , Cervicalgia
15.
Adv Tech Stand Neurosurg ; 45: 317-338, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35976455

RESUMO

Idiopathic syringomyelia (IS) and refractory syringomyelia (RS) are types of syringomyelia that often pose a management challenge and are associated with long-term clinical sequela. They are usually an epiphenomenon reflecting an underlying pathology where the treatment of the primary cause should be the aim for any surgical intervention. In the case of IS, the initial step is agreeing on the definition of the terms idiopathic and syringomyelia. After a rigorous exhaustive clinic-radiological workup, only IS patients with progressive neurology are treated, usually unblocking subarachnoid cerebrospinal fluid (CSF) pathway obstruction somewhere in the thoracic spinal canal and reserving shunting techniques to nonresponsive cases. Similar to IS, also RS is multifactorial, and its management varies based on the initial pathology, strongly supported by radiological and clinical features. We aim to address this topic focusing on the etiopathology, investigation paradigm, and surgical pathway, formulating algorithms of management with available evidence in literature. Surgical techniques are discussed in detail.


Assuntos
Malformação de Arnold-Chiari , Siringomielia , Malformação de Arnold-Chiari/complicações , Derivações do Líquido Cefalorraquidiano/efeitos adversos , Humanos , Imageamento por Ressonância Magnética/efeitos adversos , Espaço Subaracnóideo/diagnóstico por imagem , Siringomielia/diagnóstico por imagem
17.
J Rehabil Med ; 54: jrm00322, 2022 Aug 18.
Artigo em Inglês | MEDLINE | ID: mdl-35924332

RESUMO

OBJECTIVE: This scoping review aimed to identify and synthesize existing research on active conservative management of primary spinal syringomyelia and associated symptoms and to discuss perspectives for clinical application using an activity-based approach. METHODS: PubMed, Embase, Scopus, and Web of Science were systematically searched for empirical studies of conservative management or therapies of adults with primary spinal syringomyelia from inception to April 2021. In addition, abstracts from relevant conferences were searched. Study characteristics and key findings were extracted, and findings descriptively synthesized. RESULTS: Of 1,186 studies screened, 7 studies met the eligibility criteria (4 single case studies and 3 cohort studies, a total of 90 individuals). The interventions were primarily physiotherapeutic, mostly by posture correction and exercises, and effects were alleviation of pain, improved physical function, improved activities of daily living and quality of life. Analysis of factors triggering symptoms and rationale for choice of intervention based upon these was limited. CONCLUSION: Evidence of active conservative management of primary spinal syringomyelia and associated symptoms is limited. Many variations and limitations in the existing research limit the conclusions. High-quality research is needed to enable healthcare professionals to apply evidencebased active conservative interventions.


Assuntos
Qualidade de Vida , Siringomielia , Atividades Cotidianas , Adulto , Tratamento Conservador , Terapia por Exercício , Humanos , Siringomielia/terapia
18.
P R Health Sci J ; 41(3): 168-171, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-36018748

RESUMO

Currarino syndrome (CS) is characterized by a triad of anomalies consisting of: a sacral bone defect, anorectal malformations and a pre-sacral mass. We present the case of an adult patient with a medical history of CS who presented with septic shock and was subjected to an emergency laparotomy due to severe abdominal distension. In this particular case, we underline the importance of immediate surgery on the patient's outcome as well as the considerable role of landiolol in controlling the heart rate with no further deterioration of blood pressure in this patient presenting with atrial fibrillation and sepsis.


Assuntos
Anormalidades do Sistema Digestório , Sepse , Siringomielia , Adulto , Canal Anal/anormalidades , Humanos , Reto/anormalidades , Sacro/anormalidades
19.
BMC Nephrol ; 23(1): 248, 2022 07 14.
Artigo em Inglês | MEDLINE | ID: mdl-35836135

RESUMO

BACKGROUND: Distal renal tubular acidosis (dRTA) is the most common type of renal tubular acidosis (RTA) in children. Pediatric dRTA is usually genetic and rarely occurs due to acquired issues such as obstructive uropathies, recurrent urinary tract infections (UTIs), and chronic kidney disease (CKD). Although persistent hypokalemia frequently occurs with dRTA, acute hypokalemic paralysis is not frequently reported, especially in older children. CASE PRESENTATION: An eight-year-old girl presented with an acute first episode of paralysis. A physical examination revealed normal vital signs, short stature consistent with her genetic potential, and decreased muscle strength of her upper and lower extremities. Preexisting conditions included stage 4 CKD due to recurrent UTIs, severe vesicoureteral reflux and bilateral hydronephrosis, neurogenic bladder, and multisegment thoracic syringomyelia. Her laboratory work-up revealed hypokalemic, hyperchloremic metabolic acidosis with a normal anion gap. She also had a urine osmolal gap of 1.9 mOsmol/kg with a high urine pH. Intravenous potassium replacement resulted in a complete resolution of her paralysis. She was diagnosed with dRTA and discharged with oral bicarbonate and slow-release potassium supplementation. CONCLUSIONS: This case report highlights the importance of considering dRTA in the differential diagnosis of hypokalemic acute paralysis in children. Additionally, in children with neurogenic lower urinary tract dysfunction and recurrent UTIs, early diagnosis of spinal cord etiology is crucial to treat promptly, slow the progression of CKD, and prevent long-term complications such as RTA.


Assuntos
Acidose Tubular Renal , Hipopotassemia , Insuficiência Renal Crônica , Siringomielia , Infecções Urinárias , Refluxo Vesicoureteral , Acidose Tubular Renal/complicações , Acidose Tubular Renal/diagnóstico , Adolescente , Criança , Feminino , Humanos , Hipopotassemia/complicações , Hipopotassemia/diagnóstico , Paralisia/complicações , Potássio , Insuficiência Renal Crônica/complicações , Siringomielia/complicações , Siringomielia/diagnóstico , Infecções Urinárias/complicações , Refluxo Vesicoureteral/complicações , Refluxo Vesicoureteral/diagnóstico
20.
Spinal Cord Ser Cases ; 8(1): 69, 2022 07 26.
Artigo em Inglês | MEDLINE | ID: mdl-35882848

RESUMO

INTRODUCTION: After spinal cord injury, further neurological deterioration up to one to two neurological levels is not uncommon. Late neurological deterioration can occur after two months, mainly due to the syrinx formation. In a rare case like in sub-acute post-traumatic ascending myelopathy, the neurological level may ascend more than four levels from the initial level of injury and it usually starts within a few weeks after injury. CASE PRESENTATION: Our case was diagnosed as a case of traumatic spinal cord injury having a lower thoracic neurological level of injury initially, which rapidly progressed over a few weeks into a higher thoracic neurological level. He was operated with pedicle screw fixation of the spine before admission to rehabilitation unit. He was having progressive ascending neurological deterioration, starting a few days after surgery, which was evident by the progression of neurological level by more than four segments clinically. Cerebrospinal fluid(CSF) study showed no significant abnormality. Magnetic resonance imaging (MRI) study showed involvement of the spinal cord at the upper thoracic region. Patient was monitored to note any further worsening. Rehabilitation and supportive measures were provided according to standard protocol. DISCUSSION: Very few cases of ascending paralysis of more than four levels have been reported globally. It results in increased morbidity and mortality in spinal cord injury patients. In our case few possible reasons are ruled out but the actual underlying reason was not clear. Various hypotheses have been proposed as the cause in previous published literatures. Management is mostly supportive.


Assuntos
Traumatismos da Medula Espinal , Traumatismos da Coluna Vertebral , Siringomielia , Humanos , Incidência , Masculino , Paralisia , Traumatismos da Medula Espinal/complicações , Traumatismos da Medula Espinal/cirurgia , Traumatismos da Coluna Vertebral/complicações , Siringomielia/etiologia
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