A case report of a neonate with Arnold Chiari II malformation.

Chiari malformations are structural defects in the cerebellum that are characterized by the downward displacement of one or both cerebellar tonsils through the foramen magnum. A case report of a female neonate with clinical features of Chiari II malformation is presented. The diagnosis of this condition was made through a combination of clinical and radiological features. In this case, the diagnosis was confirmed by a noncontrast computed tomography (CT) scan of the brain and multiplanar brain magnetic resonance imaging (MRI).

Malformación de Arnold Chiari / Arnold Chiari malformation

La malformación de Arnold Chiari es una enfermedad rara que consiste en una alteración anatómica de la base del cráneo, en la que se produce herniación del cerebelo y del tronco del encéfalo a través del foramen magnum hasta el canal cervical. Muchos niños afectados nunca llegan a tener síntomas. En el caso de que cause síntomas, estos no suelen aparecer hasta la infancia tardía o adolescencia. Presentamos el caso clínico de un paciente de 2 años con malformación de Arnold Chiari tipo I. (AU)

Arnold Chiari malformation is a rare disease that consists of an anatomical alteration of the base of the skull, in which herniation of the cerebellum and brainstem occurs through the foramen magnum to the cervical canal. Many affected children never develop symptoms. If it does cause symptoms, they usually don’t appear until late childhood or adolescence. We present the clinical case of a 2-year-old patient with Arnold Chiari malformation type I. (AU)

Clinical presentation and outcomes of neonates born with neural tube defects- an experience from a level III B NICU in Western India.

PURPOSE: Neural tube defects (NTDs) are one of the most common congenital anomalies and a cause of chronic disability. The study was done to study outcomes of neural tube defects admitted at a tertiary level neonatal intensive care unit (NICU) from 2018 to 2022, a period of 4 years that also coincided with the COVID pandemic. The secondary outcome was to study the clinical presentation, associated anomalies and epidemiological features. METHODS: It was a retrospective observational study; data of infants was obtained from medical records and analysis was done. RESULTS: Thirty-four neonates were enrolled, of which there were 16 (47%) males and 18 (53%) females. History of pre-pregnancy maternal folate intake was present in 4 (11.7%) cases. 33 (97%) babies were diagnosed with meningomyelocele (MMC) and one each had anencephaly, iniencephaly and encephalocele, of which one had frontal and two had occipital encephalocele. The median age of surgery was 16 days of life with primary repair being the most common procedure followed by MMC repair with VP shunt. Twenty babies (58.8%) were discharged successfully, while 9 (26.5%) expired and 5 (14.7%) were discharged against medical advice; which can be attributed to the financial problems of the patients in a developing country. The overall deaths in our series were four (26.5%) which is slightly higher than other studies which may be due to the fact that this study was conducted during the COVID era with lesser rates of folate supplementation, reduced access to prenatal diagnosis coupled with poor follow-up and compliance of patients post-surgical repair. CONCLUSION: This study emphasizes the importance of periconceptional folic acid supplementation, prenatal diagnosis, early surgery and meticulous follow-up as being pivotal to improving outcomes in children with NTDs.

Chiari type III malformation associated with Klippel-Feil syndrome, a case report with a narrative review of the literature.

BACKGROUND: Chiari malformation type III (CM III), a rare hindbrain anomaly, often presents with various concurrent anomalies. This paper reports a unique case of CM III associated with Klippel-Feil syndrome (KFS), a condition previously unreported in Saudi Arabia and documented in only one other case globally in Turkey. This study aims to share insights into the unusual association between CM III and KFS, considering their close embryological development and involvement in the craniocervical junction. METHODOLOGY: The study presents a case of a 2.5-year-old female diagnosed with CM III and KFS. Diagnostic tools such as ultrasound, CT scans, MRI, and physical examinations were used to confirm the patient's condition. Surgical interventions, including decompression and encephalocele repair, were performed. RESULTS: Successful surgical interventions, including encephalocele repair and duraplasty, were carried out. Follow-up visits indicated a stable condition, marked improvement in lower limb strength, and the patient's ability to walk with assistance. CT follow-up affirmed a satisfactory surgical outcome. CONCLUSION: This case study illustrates the potential for an optimistic prognosis in CM III, even when accompanied by complex conditions such as KFS, through early diagnosis and intervention. It underscores the significance of antenatal screening for effective care planning and calls for further research and publications due to the rarity of this association. These findings contribute to our understanding of CM III and its related conditions, emphasizing the need for open-minded consideration of potential embryological associations.

Protracted respiratory failure in a case of global spinal syringomyelia and Chiari malformation following administration of diazepam: illustrative case.

BACKGROUND: Syringomyelia is defined as dilation of the spinal cord's central canal and is often precipitated by skull base herniation disorders. Although respiratory failure (RF) can be associated with skull base abnormalities due to brainstem compression, most cases occur in pediatric patients and quickly resolve. The authors report the case of an adult patient with global spinal syringomyelia and Chiari malformation who developed refractory RF after routine administration of diazepam. OBSERVATIONS: A 31-year-old female presented with malnutrition, a 1-month history of right-sided weakness, and normal respiratory dynamics. After administration of diazepam prior to magnetic resonance imaging (MRI), she suddenly developed hypercapnic RF followed MRI and required intubation. MRI disclosed a Chiari malformation type I and syrinx extending from C1 to the conus medullaris. After decompressive surgery, her respiratory function progressively returned to baseline status, although 22 months after initial benzodiazepine administration, the patient continues to require nocturnal ventilation. LESSONS: Administration of central nervous system depressants should be closely monitored in patients with extensive syrinx formation given the potential to exacerbate diminished central respiratory drive. Early identification of syrinx in the context of Chiari malformation and hemiplegia should prompt clinical suspicion of underlying respiratory compromise and early involvement of intensive care consultants.

Chiari Syndrome: Advances in Epidemiology and Pathogenesis: A Systematic Review.

Arnold Chiari syndrome is a rare congenital disease of unknown prevalence and whose origin is still under study. It is encompassed within the posterior cranial malformations, showing a wide spectrum of symptomatology that can range from severe headache, dizziness, and paresthesia to complete asymptomatology. It is for this reason that early diagnosis of the disease is difficult, and it is usually diagnosed in adolescence. Treatment is based on remodeling and decompression of the malformed posterior cranial fossa, although the risk of residual symptoms after surgery is high. The aim of this review is to update all the existing information on this pathology by means of an exhaustive analysis covering all the scientific literature produced in the last 5 years. In addition, it has been carried out following the PRISMA model and registered in PROSPERO with code CRD42023394490. One of the main conclusions based on the results obtained in this review is that the origin of the syndrome could have a genetic basis and that the treatment of choice is the decompression of the posterior cerebral fossa.

Adenotonsillectomy in Arnold Chiari Malformation: Navigating Surgical Complexities.

Navigating Surgical Complexities associated with a case of Adenotonsillectomy in Arnold Chiari Malformation type 2. Arnold-Chiari or Chiari malformations (ACM) describe a group of deformities of the posterior fossa and hindbrain, which includes the cerebellum, pons, and medulla oblongata. Sleep-disordered breathing is a known but poorly evaluated comorbidity in patients with ACM. Obstructive sleep apnoea (OSA) in children is mainly caused by tonsillar and adenoid hypertrophy, and surgical resection of the palatine tonsils and adenoids is indicated depending on OSA severity. A 4-year-old male child suffering from Arnold Chiari type 2 malformation presented to us in OPD with Severe OSA. Clinical and endoscopic examination revealed presence of Grade 4 adenoids tissue and Grade 4 tonsillar hypertrophy. Patient was planned to undergo Coblation adenoidectomy and Tonsillectomy with Uvulopalatoplasty for the management of OSA. Patient tolerated the procedure well and extubating was un-eventful. Patient was kept in PICU for overnight observation and was discharged on next day without any major complications. Patient of Arnold Chiari malformation type 2 presenting with severe OSA due to peripheral cause like Chronic adenoid and tonsillar hypertrophy present an operative challenge due to nil neck extension and minimal oral cavity space. The key is that ACM 2 along with its complications, such as difficult airway, increased intracranial pressure, and autonomic dysfunction, makes it very challenging and requires well-structured and disciplined management by combined anaesthesiology, neurology, and operative team which emphasize on preoperative, intraoperative, and postoperative complications and its timely management.

Arnold-Chiari Malformation Type I Evident After a Streptococcal Throat Infection in a Young Female: A Rare Case.

Arnold-Chiari malformation (ACM) is a rare neurological developmental disorder that presents at birth. No such cases have been reported in support of microbial infections causing Chiari malformation, yet there is evidence of how microorganisms can lead to brain abscess, brain empyema, and meningoencephalitis. We present a 23-year-old young woman with progressive back and leg pain after a streptococcal throat infection, followed by a single episode of syncope. Radiographs of the spine revealed a mild reversal of cervical spine curvature and minimal levocurvature of the lumbar spine. Magnetic resonance imaging of the brain showed herniation of the cerebral tonsils into the foramen magnum, which suggested the diagnosis of ACM type I. Neurosurgery was recommended for posterior fossa decompression, but the patient was reluctant to undergo the procedure. This is a rare case of ACM symptomology that became evident only after a streptococcal throat infection in a young adult female.

A Critical Update of the Classification of Chiari and Chiari-like Malformations.

Chiari malformations are a group of craniovertebral junction anomalies characterized by the herniation of cerebellar tonsils below the foramen magnum, often accompanied by brainstem descent. The existing classification systems for Chiari malformations have expanded from the original four categories to nine, leading to debates about the need for a more descriptive and etiopathogenic terminology. This review aims to examine the various classification approaches employed and proposes a simplified scheme to differentiate between different types of tonsillar herniations. Furthermore, it explores the most appropriate terminology for acquired herniation of cerebellar tonsils and other secondary Chiari-like malformations. Recent advances in magnetic resonance imaging (MRI) have revealed a higher prevalence and incidence of Chiari malformation Type 1 (CM1) and identified similar cerebellar herniations in individuals unrelated to the classic phenotypes described by Chiari. As we reassess the existing classifications, it becomes crucial to establish a terminology that accurately reflects the diverse presentations and underlying causes of these conditions. This paper contributes to the ongoing discussion by offering insights into the evolving understanding of Chiari malformations and proposing a simplified classification and terminology system to enhance diagnosis and management.

Concurrent Presence of Thoracolumbar Scoliosis and Chiari Malformation: Is Operative Risk Magnified?

STUDY DESIGN: Retrospective review of Kids' Inpatient Database (KID). PURPOSE: Identify the risks and complications associated with surgery in adolescents diagnosed with Chiari and scoliosis. OVERVIEW OF LITERATURE: Scoliosis is frequently associated with Chiari malformation (CM). More specifically, reports have been made about this association with CM type I in the absence of syrinx status. METHODS: The KID was used to identify all pediatric inpatients with CM and scoliosis. The patients were stratified into three groups: those with concomitant CM and scoliosis (CMS group), those with only CM (CM group), and those with only scoliosis (Sc group). Multivariate logistic regressions were used to assess association between surgical characteristics and diagnosis with complication rate. RESULTS: A total of 90,707 spine patients were identified (61.8% Sc, 37% CM, 1.2% CMS). Sc patients were older, had a higher invasiveness score, and higher Charlson comorbidity index (all p<0.001). CMS patients had significantly higher rates of surgical decompression (36.7%). Sc patients had significantly higher rates of fusions (35.3%) and osteotomies (1.2%, all p<0.001). Controlling for age and invasiveness, postoperative complications were significantly associated with spine fusion surgery for Sc patients (odds ratio [OR], 1.8; p<0.05). Specifically, posterior spinal fusion in the thoracolumbar region had a greater risk of complications (OR, 4.9) than an anterior approach (OR, 3.6; all p<0.001). CM patients had a significant risk of complications when an osteotomy was performed as part of their surgery (OR, 2.9) and if a spinal fusion was concurrently performed (OR, 1.8; all p<0.05). Patients in the CMS cohort were significantly likely to develop postoperative complications if they underwent a spinal fusion from both anterior (OR, 2.5) and posterior approach (OR, 2.7; all p<0.001). CONCLUSIONS: Having concurrent scoliosis and CM increases operative risk for fusion surgeries despite approach. Being independently inflicted with scoliosis or Chiari leads to increased complication rate when paired with thoracolumbar fusion and osteotomies; respectively.

First Reported Case of Gabriele-de Vries Syndrome with Spinal Dysraphism.

Gabriele-de Vries syndrome is a rare autosomal dominant genetic disease caused by de novo pathogenic variants in the Yin Yang 1 (YY1) gene. Individuals with this syndrome present with multiple congenital anomalies, as well as a delay in development and intellectual disability. Herein, we report the case of a newborn male patient with a novel de novo pathogenic variant in the Guanine Nucleotide-Binding Protein, Alpha Stimulating (GNAS) gene, which was identified by whole-exome sequencing. Our patient suffered from a large open spinal dysraphism which was treated surgically immediately after birth. During the follow-up, facial dysmorphism, bladder and bowel incontinence, and mildly delayed motor and speech development were observed. Congenital central nervous system disorders were also confirmed radiologically. In this case report, we present our diagnostic and treatment approaches to this patient. To our knowledge, this is the first reported case of Gabriele-de Vries syndrome presenting with spinal dysraphism. Extensive genetic evaluation is the cornerstone in treatment of patients with suspected Gabriele-de Vries syndrome. However, in cases with potentially life-threatening conditions, surgery should be strongly considered.

Neurological pathologies in acute acquired comitant esotropia.

BACKGROUND: Acute acquired comitant esotropia (AACE) is an uncommon subtype of esotropia characterized by sudden and usually late onset of a relatively large angle of comitant esotropia with diplopia in older children and adults. METHODS: A literature survey regarding neurological pathologies in AACE was conducted using databases (PubMed, MEDLINE, EMBASE, BioMed Central, the Cochrane Library, and Web of Science) in order to collect data for a narrative review of published reports and available literature. RESULTS: The results of the literature survey were analyzed to provide an overview of the current knowledge of neurological pathologies in AACE. The results revealed that AACE with unclear etiologies can occur in many cases in both children and adults. Functional etiological factors for AACE were found to be due to many reasons, such as functional accommodative spasm, the excessive near work use of mobile phones/smartphones, and other digital screens. In addition, AACE was found to be associated with neurological disorders, such as astrocytoma of the corpus callosum, medulloblastoma, tumors of the brain stem or cerebellum, Arnold-Chiari malformation, cerebellar astrocytoma, Chiari 1 malformation, idiopathic intracranial hypertension, pontine glioma, cerebellar ataxia, thalamic lesions, myasthenia gravis, certain types of seizures, and hydrocephalus. CONCLUSIONS: Previously reported cases of AACE with unknown etiologies have been reported in both children and adults. However, AACE can be associated with neurological disorders that require neuroimaging probes. The author recommends that clinicians should perform comprehensive neurological assessments to rule out neurological pathologies in AACE, especially in the presence of nystagmus or abnormal ocular and neurological indications (e.g., headache, cerebellar imbalance, weakness, nystagmus, papilloedema, clumsiness, and poor motor coordination).

Arnold Chiari malformation classified as Chiari 1.5 malformation in a Japanese black calf.

A 2-day-old male black calf presented with neurological symptoms, including opisthotonus. It was unable to stand due to hindquarter paresis. At 5 days old, the calf was able to stand, but exhibited a crossed forelimb gait. Computed tomography and magnetic resonance imaging revealed the expansion of the sutures between the squamous-lateral part of the occipital bone and between the occipital-temporal bone, cerebellar tonsillar herniation, posterior displacement of the brainstem, and cervical syringomyelia at 12 days old. This is the first case report of a live calf diagnosed with Arnold Chiari malformation classified as Chiari type 1.5 malformation in humans.

Cranial Versus Cervical Spine Magnetic Resonance Imaging in Adult Chiari Malformation Type I Diagnostics: Is There a Difference in Tonsil Ectopia Length?

BACKGROUND: Chiari malformation type 1 (CM-1) diagnosis is based on measurement of the cerebellar tonsils on cranial or cervical spine magnetic resonance imaging (MRI). However, imaging parameters of cranial and cervical spine MRI could differ because spine MRI has greater resolution. METHODS: We conducted a retrospective chart review of 161 patients of a single neurosurgeon for adult CM-I consultation between February 2006 and March 2019. Patients were selected based on receiving both cranial and cervical spine MRI within a month of each other to determine tonsillar ectopia length for CM-1. Ectopias were measured to determine if differences in values were statistically significant. RESULTS: From the 161 total patients, 81 had cranial and cervical spine MRI for a total of 162 tonsil ectopia measurements (81 cranial and 81 spinal). Average ectopia length on a cranial MRI was 9.1 mm (±5.2 mm); average ectopia length on a spinal MRI was 8.9 mm (±5.3 mm). Average cranial and spinal MRI values were found to be <1 standard deviation apart. Two-tailed, nonequal variances t test determined that differences between the cranial and spinal ectopia measurements were insignificant (P = 0.2403). CONCLUSIONS: This study confirmed that the added resolution offered by spine MRI did not make better or more refined measurements over cranial MRI that could lead to measurement differences, which can instead be attributed to chance. Cranial and cervical spine MRI can be used to determine the degree of the tonsil ectopia.

Surgical outcomes of calvaria reconstruction in cranial pansynostosis associated with Arnold-Chiari type 1.5 malformation, a case report.

A 6-year-old girl with persistent headaches and the visual problem was diagnosed as a delayed onset cranial pansynostosis with concurrent type 1.5 Arnold-Chiari malformation. She underwent multi-sutural reconstructive surgery and followed. The headache was greatly decreased and tonsillar-brain stem herniation and syrinx were resolved.

Malformación de Arnold-Chiari tipo I con siringomielia

Introducción: Las malformaciones de Arnold-Chiari, son un grupo heterogéneo de desórdenes neurológicos caracterizados por alteraciones dentro de las regiones del cerebelo, tallo cerebral y unión cráneo-cervical; todas resultan en un desplazamiento inferior del cerebelo hacia el canal espinal por el foramen magno, sea en conjunto con la médula inferior o no. Objetivo: Describir los hallazgos clínicos, estudios complementarios, criterios diagnósticos, conducta terapéutica y evolución, en un caso con malformación de Chiari tipo I con siringomielia. Presentación de caso: Se presenta a una paciente con cefalea de curso insidioso a la que se le diagnostica malformación de Chiari tipo I con siringomielia, que requirió tratamiento neuroquirúrgico. La evolución posoperatoria fue favorable. Conclusiones: La malformación de Arnold-Chiari es una entidad rara; la aparición de la resonancia magnética nuclear ha permitido que su diagnóstico aumente y por tanto, su incidencia. El caso presentado tuvo un curso clínico clásico.

Introduction: Arnold-Chiari malformations are a heterogeneous group of neurological disorders characterized by alterations within the regions of the cerebellum, brain stem and craniocervical junction; all result in inferior displacement of the cerebellum into the spinal canal through the foramen magnum, whether in conjunction with the inferior cord or not. Objective: To describe the clinical findings, complementary studies, diagnostic criteria, therapeutic approach and evolution, in a case with type I Chiari malformation with syringomyelia. Case report: A patient with an insidious course of headache is reported. She was diagnosed with type I Chiari malformation with syringomyelia, which required neurosurgical treatment. The postoperative evolution was favorable. Conclusions: Conclusions: Arnold-Chiari malformation is a rare entity. the appearance of nuclear magnetic resonance has allowed it to be diagnosed and therefore its incidence has increased. The reported case had a classic clinical course.

Neurological pathologies associated with acute acquired comitant esotropia.

Previously reported cases of acute acquired comitant esotropia (AACE) with unknown etiologies have been reported in both children and adults. However, AACE can be associated with neurological disorders that require neuroimaging probes. The author recommends that clinicians should perform comprehensive neurological assessments to rule out neurological pathologies in AACE, especially in the presence of A or V pattern esotropia, nystagmus, or abnormal ocular and neurological indications (e.g. headache, cerebellar imbalance, weakness, nystagmus, papilloedema, clumsiness, and poor motor coordination).

Mosaic variegated aneuploidy syndrome 2 with biallelic novel CEP57 splice site variation in Indian siblings: Expanding the clinical and molecular spectrum.

Mosaic variegated aneuploidy syndrome 2 (MVA2) (MIM# 614114) is a rare autosomal recessive condition caused by biallelic loss of function variants in the CEP57 gene. MVA2 is characterized by a variable phenotype ranging from poor growth to facial dysmorphism, short stature and congenital heart defects. Only 11 families and 5 pathogenic variants of MVA2 have been described so far. Intragenic duplication of 11 nucleotides (c.915_925dup11) in homozygous or compound heterozygous state is the commonest genetic aberration (10/13). We describe the first Indian family with two siblings with a novel homozygous splice site variant (c.382+2T>C) in CEP57. Molecular characterization demonstrated skipping of exon 3 due to the variant with protein modeling predicting subsequent complete loss of function. This is the first report of a splice site variation in CEP57 leading to MVA2.

Practical Algorithm for the Management of Multisutural Craniosynostosis with Associated Chiari Malformation and/or Hydrocephalus.

INTRODUCTION: The association between multisutural craniosynostosis with Chiari malformation (CM), venous hypertension, and hydrocephalus is widely described in the literature, especially in children with paediatric craniofacial syndromes. Some efforts have been done in the last years to understand the complex pathogenetic mechanisms underlying this association, and several theories have been proposed. In particular, it is now accepted that the hypothesis of the overcrowding of the posterior fossa due to precocious suture fusion is the cause of the cerebellar herniation in syndromic and non-syndromic patients, against the theory of intrinsic cerebellar anomalies, ventriculomegaly, and venous hypertension. However, whatever the pathophysiological mechanism, it is still unclear what the best management and treatment of CM and hydrocephalus are in multisutural craniosynostosis patients. The aim of this study was to report our 25 years' experience in treating paediatric patients affected by these rare pathologies in order to propose a simple and effective therapeutic flow chart for their management. MATERIALS AND METHODS: We retrospectively collected data of each patient who underwent a cranial vault remodelling (CVR) for complex multisutural craniosynostosis in our institution in the last 25 years, while monosutural craniosynostosis was excluded. We recorded data concerning type of craniosynostosis and craniofacial syndromes, presence of ventriculomegaly, and CM at presentation and clinical and radiological follow-up. Therefore, we evaluated the final outcomes (improved, stable, deteriorated) of these patients and created a practical flow chart that could help physicians choose the best surgical treatment when different pathological conditions, as Chiari malformation I (CMI) or hydrocephalus, affect complex craniosynostosis children. RESULTS: Thirty-nine patients (39 out of 55; 70.9%), with an isolated multisutural craniosynostosis at presentation, underwent a two-step CVR as first surgery; 36 patients (92.3%) had an improved outcome, 2 patients (5.1%) had a stable outcome, and 1 patient (2.56%) had a deteriorated outcome. Other eight children (8 out of 55; 14.5%) had a radiological evidence of asymptomatic CMI at presentation. In this group, we performed CVR as first surgery. As for the final outcome, 7 patients had an improved outcome (87.5%) with good aesthetic result and stability or resolution of CMI. Finally, 7 patients (7 out of 55; 12.7%) presented a various combination of CMI and ventriculomegaly or hydrocephalus at presentation. Among them, 3 patients had an improved outcome (42.8%), and 4 patients had a deteriorated outcome (57.1%). DISCUSSION: The prevalence of one pathological condition with associated symptoms over the others was the key factor leading our therapeutic strategy. When craniosynostosis is associated with a radiological CM, the assessment of clinical symptoms is of capital importance. When asymptomatic or pauci-symptomatic, we suggest a CVR as first step, for its efficacy in reducing tonsillar herniation and solving CM symptoms. When craniosynostosis is associated with ventricular enlargement, the presence of intracranial hypertension signs and symptoms forces physicians to first treat hydrocephalus with a ventriculo-peritoneal shunt or endoscopic third ventriculostomy. For patients with various degrees and severity of ventriculomegaly and associated CM, the outcomes were very heterogeneous, even when the same therapeutic strategy was applied to patients with similar starting conditions and symptoms. This is maybe the most unexpected and least clear part of our results. Despite the proposed algorithm comes from a clinical experience on 85% successfully treated patients with multiple craniosynostosis, more extensive and deep studies are needed to better understand CM and hydrocephalus development in such conditions.

Experiencia en patología cervical alta en el Hospital Guillermo Almenara Irigoyen 2016 - 2021

Introducción : El complejo C0-C1-C2 es responsable de la transición de la carga axial, con función biomecánica única, siendo afectada por múltiples patologías, que por lo general la literatura no las considera como un solo ítem, sino que lo desarrolla según su etiología, pero en nuestro estudio se ha considerado en 5 grupos: traumática, congénita, inflamatoria reumática, neoplásica y degenerativa. Objetivo : Determinar las características epidemiológicas, clínicas y del tratamiento en la patología cervical alta. Materiales y métodos : Se incluyeron a todos los pacientes con diagnóstico clínico radiológico de alguna patología cervical alta que hayan sido sometidos a tratamiento quirúrgico entre 2016 y 2021 en el Hospital Almenara. Se usó el test "t" de student y de chi cuadrado. Se dividió a los pacientes en alguno de los 5 grupos antes mencionados. Resultados : Se consideraron 31 pacientes, con una edad media de 51.16 años. La patología cervical alta más frecuente fue la traumática con el 35.48%. El déficit motor se presentó en el 51.61% y el déficit sensitivo se presentó en el 54.84%. La cirugía más frecuente fue la fijación cervical alta con el 43.89%. La tasa de complicaciones fue del 16.13% con una mortalidad del 0%. Conclusiones : La patología cervical alta es rara, siendo la del tipo traumática la más frecuente, pero un manejo oportuno y adecuado permite un mejor pronóstico funcional del paciente.

Introduction : The C0-C1-C2 complex is responsible of axial load transition, and its biomechanical function is unique, it is affected by multiple pathological conditions; and generally speaking, the literature does not consider these conditions as a single item, it describes them according to etiology. For our study we considered five groups: trauma-related, congenital, rheumatic-inflammatory, neoplastic, and degenerative. Objective : To determine epidemiological, clinical, and therapy-related characteristics in upper cervical pathological conditions. Materials and methods : All patients with a clinical-radiological diagnosis of any upper cervical pathological condition that had undergone surgery between 2016 and 2021 in Guillermo Almenara Hospital were included. Student's t test and chi square methods were used. patients were divided into one of the five aforementioned groups. Results : Thirty-one patients were included in the study; their mean age was 51.16 years. The most frequent upper cervical pathological condition was trauma-related, with 35.48%. Motor deficit occurred in 51.61% of all patients, and sensitive deficit occurred in 54.84%. The most frequently surgical procedure performed was upper cervical fixation, in 43.89% of all patients. Complication rate was 16.13%, and mortality was 0%. Conclusions : Upper cervical pathological conditions are rare, trauma-related conditions are most frequent, but timely and adequate management allow us to achieve better functional prognosis for these patients.