JOURNAL OF MILITARY PHARMACO MEDICINE N05 2022 246 CLINICAL CHARACTERISTICS OF CHIARI MALFORMATION TYPE I IN ADULTS Huynh Thanh Binh1, Huynh Le Phuong2, Bui Quang Tuyen2 Nguyen Thanh Bac3, Tong Duc Mi[.]
JOURNAL OF MILITARY PHARMACO - MEDICINE N05 - 2022 CLINICAL CHARACTERISTICS OF CHIARI MALFORMATION TYPE I IN ADULTS Huynh Thanh Binh1, Huynh Le Phuong2, Bui Quang Tuyen2 Nguyen Thanh Bac3, Tong Duc Minh3 SUMMARY Objectives: To describe some clinical characteristics of Chiari malformation type I in adults Subjects and methods: A retrospective, prospective, descriptive, and cross-sectional study on 45 adult patients (≥ 18 years of age) diagnosed Chiari I malformation and surgically treated at the Departments of Neurosurgery, Cho Ray Hospital from January 2016 to December 2019 Results: Mean age was 35.84 ± 9.35 years old The average time from symptom onset to surgery was 16.42 ± 37.34 months Clinical symptoms of Chiari I malformation varied, suboccipital headache and limb numbness were still the most common symptoms (48.89% and 64.44%, respectively) Tremor in limbs was 13.33% Dizziness and nausea were encountered in 6.67%; 4.44% had balance disorder Shortness of breath accounted for 2.22%; facial numbness pain 6.67% and hoarse voice 2.22% In the group of symptoms of spinal cord compression, 2.22% had weakness and paralysis of the limbs, 2.22% of sensory disturbances, and 11.11% of muscle atrophy Valsalva test was positive (2.22%) Conclusion: Clinical symptoms of Chiari I malformation varied, suboccipital headache and limb numbness were still the most common symptoms Tremor, dizziness, nausea, balance disorder, shortness of breath, facial pain, hoarseness, weakness in limbs, sensory disturbances, muscle atrophy, and Valsalva were less commonly seen in our study * Keywords: Chiari I malformation; Clinical characteristics; Adults Military Hospital 175 Cho Ray Hospital Vietnam Military Medical University Corresponding author: Huynh Thanh Binh (Drbinhv175@gmail.com) Date received: 02/5/2022 Date accepted: 30/5/2022 246 JOURNAL OF MILITARY PHARMACO - MEDICINE N05 - 2022 INTRODUCTION Chiari malformation belongs to a group of pathologies, which are mostly congenital, but sometimes acquired It is an abnormal development of the posterior brain, herniation of the cerebellum amygdala, and posterior brain inferior to the occipital foramen, which is not caused by mass or ventricular dilatation This condition may be accompanied by myelosuppression, hydrocephalus, and posterior fossa stenosis, Chiari malformation type I associated with myelosuppression accounts for 30 - 70% of patients [3] The clinical characteristics are obscure unpresentation, easily misdiagnosed with some diseases of the brain or cervical spinal cord The diagnosis is mainly based on magnetic resonance imaging Currently, there have not been many clinical studies of this disease in Vietnam Therefore, we carry out this study: To describe some clinical characteristics of Chiari malformation type I in adults SUBJECTS AND METHODS Subjects 45 adult patients (≥ 18 years of age) were diagnosed as Chiari I malformation and surgically treated at the Department of Neurosurgery, Cho Ray Hospital from January 2016 to December 2019 * Selection criteria: - Patients were 18 years old and presented with clinical symptoms of Chiari I malformation - Amygdala of the cerebellum, with ≥ mm herniating through the occipital foramen - The image of the large tank of cerebrospinal fluid in the back fossa was pinched - There may be cervical or thoracic myelosuppression, kyphosis, or dilated ventricles The confirmed criterion was that during surgery, there was a sign of the cerebellar amygdala descending through the occipital foramen in the cervical canal, with obstruction of cerebrospinal fluid flow at the position of the occipital foramen * Exclusion criteria: - Patients and their families refused surgical treatment - The patient was diagnosed accidentally during screening with a head CT scan or cervical spine - The patient had accompanying lesions in the brain such as brain tumor, brain vascular malformation - Patients with Chiari malformation of other types Methods * Study design: A retrospective, prospective, descriptive, and crosssectional study * Research variables: - Age (18 - 40, 41 - 60, > 60 years old) - Gender - Medical history: Obstetric trauma, old craniocerebral surgery (ventricular 247 JOURNAL OF MILITARY PHARMACO - MEDICINE N05 - 2022 drainage, posterior fossa tumour ), brain trauma, cervical spine - Time from symptom detection to surgery (< 3; - 6; > months) - Clinical manifestation: Headache in the suboccipital region spreads to the parietal region, eye sockets, or the shoulder, pain increases with coughing or exertion, dizziness, vertigo, and hand pain; numbness in limbs, shortness of breath, glare; muscle atrophy, weakness of limbs Headache in the lower occipital region spreads to the parietal region or down the nape, shoulder one or two sides Valsalva test Posterior fossa compression syndrome (dizziness, tinnitus, unsteady walking, balance disorder) Brainstem compression syndrome Syndrome of spinal cord compression (limb numbness, sensory dissociation, limb weakness, muscle atrophy) Signs of pain in the cranial nerves (V, XI, XII ) Myelosuppression syndrome: sensory dissociation, nutritional disorders - Determining the relationship between the clinical symptoms of Chiari I malformation group with and without accompanying Myelosuppression * Statistical analysis: The research data were processed by a computer using SPSS 22.0 and Excel 2016 software based on the biomedical statistics method Comparison of mean value by testing quantitative variables on two independent samples (IndependentSample T-Test and Mann-Whitney U); quantitative test variables on paired samples (Pair-Sample T-Test) Comparison of the rates using the χ2 test or Fisher’s Exact test The difference was statistically significant, with p < 0.05 RESULTS Table 1: Age and gender characteristics of the study group Age group Male [n (%)] Female [n (%)] Total [n (%)] 18 - 39 16 (84.21) 16 (61.54) 32 (71.11) 40 - 60 (15.79) 10 (38.46) 13 (28.89) Total 19 (42.22) 26 (57.78) 45 (100) 33.63 ± 9.00 37.46 ± 9.44 35.84 ± 9.35 ± SD p 0.097 0.178 In the study group, there were 19 male patients (42.22%) and 26 female patients (57.78%) The female/male ratio was approximately 3/2 The youngest 248 JOURNAL OF MILITARY PHARMACO - MEDICINE N05 - 2022 patient was 19 years, and the oldest was 58 years, average age: 35.84 ± 9.35 years Most of the patients (71.11%) were in 18 - 39 age group There were 16 male patients (84.21%) aged 18 to 39 and 16 female patients (61.54%) Table 2: Medical history Medical history Number (n = 45) Rate (%) Previous ventricular dilatation surgery 2.22 Trauma 2.22 There were two patients (4.44%) with a medical history, of which a history of trauma was identified in one patient (2.22%); one patient had a history of previous ventricular dilatation factors Table 3: Prevalence of diseases diagnosed and treated before surgery Diagnosis Number (n = 45) Rate (%) Unexplained headache 23 51.11 Other diagnoses 22 48.89 45 100 Total There were 23 patients (51.11%) with headache of unknown cause and prolonged treatment before surgery Table Onset-to-diagnosis duration (months) Time of symptom onset (months) Number (n = 45) Rate % ≤ 12 36 80.00 13 - 60 15.56 61 - 120 2.22 > 120 2.22 Total 45 100 Among 45 patients, there were 36 patients (80%) with a disease duration of less than year, patients (15.56%) from to years, patient (2.22%) from - 10 years, and patient (2.22%) over 10 years 249 JOURNAL OF MILITARY PHARMACO - MEDICINE N05 - 2022 The average time from symptom onset to surgery was 16.42 ± 37.34 months, the earliest time was month and the longest time was 20 years Table 5: Time to diagnosis of groups with and without myelosuppression (months) Groups Number Mean Min Max Myelosuppression 25 16.64 240 Without myelosuppression 20 16.15 84 Total 45 16.42 240 p 0.966 The average time from the onset to the diagnosis of myelosuppression was 16.64 months, longer than that of patients without myelosuppression (16.15 months) This difference was not statistically significant, with p = 0.966 Figure 1: Clinical signs upon admission Headache in the lower occipital region, in the nape of the neck was the most common (19 patients accounting for 42.22%), followed by muscle contraction, weakness in limbs (16 patients accounting for 35.56%), and dizziness, nausea (4.44%); in which patients (4.44%) had to be hospitalized because of facial numbness 250 JOURNAL OF MILITARY PHARMACO - MEDICINE N05 - 2022 Table 6: Clinical symptoms of Chiari I malformation Clinical presentations Patient (n = 45) Percentage % Suboccipital headache 22 48.89 Valsalva test (+) 2.22 Eyeache 2.22 Photophobia 2.22 Dizziness 2.22 Vertigo and nausea 6.67 Balance disorder 4.44 Tinnitus 2.22 Earache 2.22 Dysphagia 2.22 Shortness of breath 2.22 Hoarseness 2.22 Tremor in limbs 13.33 Facial pain 6.67 Sensory disturbances 2.22 Weakness and paralysis of the limbs 2.22 Muscle atrophy 11.11 Teres disorder 2.22 Numbness of limbs 29 64.44 Clinical symptoms of Chiari I malformation were diverse and abundant, but suboccipital headache and numbness of limbs were still the most common symptoms (48.89% and 64.44%, respectively), tremor in limbs: 13.33% Symptoms of posterior fossa compression, including dizziness and nausea were found in 6.67%; 4.44% had a balance disorder Symptoms of compression of the brain stem and cranial nerves, the shortness of breath accounted for 2.22%, followed by facial pain (6.67%) and 2.22% hoarseness In the group of symptoms of spinal cord compression, 2.22% had weakness and paralysis of the limbs, 2.22% of sensory disturbances, and 11.11% of muscle atrophy Valsalva test met 2.22% 251 JOURNAL OF MILITARY PHARMACO - MEDICINE N05 - 2022 Table 7: Clinical symptoms of Chiari I malformation by age group Groups < 40 years old > 40 years old (n = 32) (n = 13) 13 (40.63) (69.23) Valsalva test (0.0) (7.69) Eyeache (0.0) (7.69) Photophobia (0.0) (7.69) Dizziness (0.0) (7.69) Vertigo and nausea (23.08) Balance disorder (15.38) Tinnitus (0.0) (7.69) Earache (0.0) (7.69) Dysphagia (0.0) (7.69) Shortness of breath (0.0) (7.69) Hoarseness (0.0) (7.69) Tremor in limbs (25.63) (7.69) Facial pain (3.13) (15.38) Sensory disturbances (0.0) (7.69) Weakness and paralysis of the limbs (0.0) (7.69) Muscle atrophy (12.50) (7.69) Teres disorder (3.13) (0.0) 19 (59.38) 10 (76.92) Clinical symptoms Suboccipital headache Numbness of limbs Symptoms of headache in the suboccipital region in groups under 40 years old and over 40 years old were 40.63% and 69.23%, respectively Tremor and muscle atrophy in the group under 40 years old were more common (15.63% and 12.50%, respectively); only 7.69% were found in the group over 40 years old In contrast, symptoms of dizziness, nausea and balance disorder, and facial pain in the group over 40 years old were more common than 23.08; 15.38 and 15.38%, respectively 252 ... have not been many clinical studies of this disease in Vietnam Therefore, we carry out this study: To describe some clinical characteristics of Chiari malformation type I in adults SUBJECTS AND... (V, XI, XII ) Myelosuppression syndrome: sensory dissociation, nutritional disorders - Determining the relationship between the clinical symptoms of Chiari I malformation group with and without... (4.44%); in which patients (4.44%) had to be hospitalized because of facial numbness 250 JOURNAL OF MILITARY PHARMACO - MEDICINE N05 - 2022 Table 6: Clinical symptoms of Chiari I malformation Clinical