Case Of Chiari Research Articles

Radiometric and Morphologic Analysis of Arnold Chiari Type-I Malformation and Idiopathic Syringomyelia: A Case Series from Pakistan

Background and Objective: Chiari I Malformation-associated syringomyelia (CM) and idiopathic syringomyelia (IS) are often confused together. They require different diagnostic approach and treatment modalities; it is important to distinguish between the two. We aimed to evaluate the radiological and morphologic characteristics of CM and IS in adult and pediatric patients in Pakistani population. Methods: Our retrospective case series was conducted at the Department of Radiology, Punjab Institute of Neurosciences assessing the operated cases of CM and IS cases from January 2022 to December 2023. Preoperative morphologic and radiological data was collected for adult and pediatric patients. Results: Out of 16 patients, 75% were adults and four were children. Twelve patients were male. The mean age of adult patients at presentation was 30.50 ± 9.20 years. There were nine cases of Chiari I Malformation and seven cases of idiopathic syringomyelia. Craniocervical junction (CCJ) was the most common site of syrinx formation in adults (41.66%) in both Chiari I and IS groups. The mean size of syrinx in adults in IS was 7.842 ± 1.93 and in CM was 4.740 ± 0.45. Mean size of syrinx in children was 4.84 ± 0.48 mm. Moniliform shape was the most common form of syrinx in patients in both CM and IS groups. Conclusion: In the Pakistani population, this study focuses on the radiological characteristics of idiopathic syringomyelia (IS) and Chiari I Malformation (CM), both of which commonly exhibit a moniliform syrinx. The craniocervical junction was frequently involved in CM, but IS exhibited a wider, bigger syrinx. Accurate diagnosis as well as treatment depend on imaging. doi: https://doi.org/10.12669/pjms.40.12(PINS).11095 How to cite this: Khan MT, Kaleem M, Qadri HM, Manzoor H, Shoaib M. Radiometric and Morphologic Analysis of Arnold Chiari Type-I Malformation and Idiopathic Syringomyelia: A Case Series from Pakistan. Pak J Med Sci. 2024;40(12):S69-S74. doi: https://doi.org/10.12669/pjms.40.12(PINS).11095 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Clinical Relevance of Preoperative MRI in Adolescent Idiopathic Scoliosis: Is Hydromyelia a Predictive Factor of Intraoperative Electrophysiological Monitoring Alterations?

This was a prospective cohort study. The main objectives of this study were to evaluate the prevalence and clinical relevance of neuroaxial anomalies in adolescent idiopathic scoliosis (AIS) patients as well as to evaluate different clinical and radiologic variables as potential predictors of the presence of a magnetic resonance imaging (MRI) abnormality. The usefulness of preoperative magnetic resonance imaging in AIS is still debated in the literature as well as the clinical relevance of the neuroaxial anomalies detected. We performed an analysis on 88 patients affected by AIS with normal neurological examination undergoing a posterior arthrodesis intervention. Patients were stratified according to the presence and type of neuroaxial abnormalities and were compared by age, sex, Risser grading, Lenke curve type, coronal and sagittal curve parameters, presence of alterations at intraoperative neuromonitoring, and "pain" and "function" scores at the SRS-22 test. Neuroaxial abnormalities were reported in 23 patients (26.14%): 14 hydromyelias, 7 syringomyelias, 1 case of Chiari I syndrome, and 1 case of spinal ependymoma. Age, sex, Risser grading, curve type, and coronal and sagittal curve parameters did not differ between normal patients and patients with any neuroaxial abnormality. Alterations of motor-evoked potential/somatosensory-evoked potential monitoring during surgery were reported in 23 patients; the difference in their incidence between normal (20%) and hydromyelia (64%) patients was significant (P<0.01). In evaluating the subareas of the SRS-22 test, we found that the "function" scores differed significantly by comparing normal (21.5±1.6) and hydromyelia (20.4±1.8) patients (P<0.05). The prevalence of neuroaxial abnormalities in AIS patients is relatively high, but we could not identify any definitive clinical or radiologic predictor of their presence. Hydromyelia was correlated with intraoperative motor-evoked potential/somatosensory-evoked potential electrophysiological alterations.

Comparison Study between Posterior Fossa Decompression with Duroplasty and Posterior Fossa Decompression without Duroplasty, in 20 Cases of Chiari I Malformation

Introduction: Chiari malformation Type I (CM-I) is typically defined as descent of the caudal tip of cerebellar tonsils at least 5 mm below the foramen magnum. The incidence of the malformation is not exactly known. Treatment of Chiari I malformation is debatable. Some advocate posterior fossa decompression (PFD) with duroplasty and others advocate posterior fossa decompression only without duroplasty. Aim of the Study: To compare the outcomes of patients who undergoing PFD with duroplasty and PFD without duroplasty, in Chiari I malformation. Patients and Methods: In the period between “January 2015-June 2016”, a prospective study was conducted involving 20 patients complaining of headache, motor and/or sensory affection secondary to Chiari I malformation. These patients were randomly divided into 2 groups. The first one had PFD with duroplasty and the other will have PFD without duroplasty. All patients had chiari one malformation in form of tonsillar herniation and cervical or cervico-dorsal syrinx. The average follow-up period was 9 months. Results: Over 90% of patients had a good clinical outcome, with improvement or resolution of their symptoms at last follow-up. There were no major complications. The mean length of hospital stay was 2.0 days. There was no perioperative death or neurological deterioration. The use of duroplasty was significantly associated with presence of complications and longer duration of hospital stay. Conclusion: PFD without duroplasty in cases of chiari I malformation carries a good results as well as PFD with duroplasty with lower risk of complications.

Pediatric encephaloceles: A series of 20 cases over a period of 3 years.

Background:Encephalocele is the protrusion of the cranial contents beyond the normal confines of the skull through a defect in the calvarium and is far less common than spinal dysraphism. The exact worldwide frequency is not known.Aims and Objectives:To determine the epidemiological features, patterns of encephalocele, and its postsurgical results.Materials and Methods:The study was carried from year July 2012 to June 2015. Patients with encephalocele were evaluated for epidemiological characteristics, clinical features, imaging characteristics, and surgical results.Results:20 encephaloceles patients were treated during the study period. Out of these 12 (60%) were male and 8 (40%) female. Age range was 1 day to 6 years. The most common type of encephalocele was occipital 12 (60%), occipito-cervical 4 (20%), parietal 2 (10%), fronto-nasal 1 (5%), and fronto-naso-ethmoidal 1 (5%). One patient had a double encephalocele (one atretic and other was occipital) with dermal sinus tract and limited dermal myeloschisis. Other associations: Chiari 3 malformation (2), meningomyeloceles (4), and syrinx (4). Three patients presented with rupture two of whom succumbed to meningitis and shock. Seventeen patients treated surgically did well with no immediate surgical mortality (except a case of Chiari 3 malformation who succumbed 6 months postsurgery to unrelated causes). Shunt was performed in 4 cases.Conclusion:The most common type of encephalocele is occipital in our set up. Early surgical management of encephalocele is not only for cosmetic reasons but also to prevent tethering, rupture, and future neurological deficits.

An Atypical Case of Chiari II Malformation Mimicking Partial Rhombencephalosynapsis

Partial rhombencephalosynapsis in the presence of Chiari II malformation has been proposed as a "new abnormality of the hindbrain and spine". We describe a case of Chiari II malformation with imaging features mimicking partial rhombencephalosynapsis. Our case demonstrates how the imaging findings of Chiari II malformation can be confused with the above entity and highlights the differentiating features to help radiologists make an accurate diagnosis.

Chiari III Malformation: A Rare Case with Review of Literature

The Chiari Malformation is a neural tube defect involving the posterior cranial fossa and the hind brain, characterized by herniation of the posterior fossa contents below the level of the foramen magnum. Four types were described by Hans Chiari in the late 19th century. Of these, Type III is exceedingly rare. We report the case of Chiari III malformation in a 24-week fetus by the integration of radiologic and autopsy findings which was conspicuous by the absence of high cervical spina bifida and the presence of occipital squama defect.

A single-center experience with eccentric syringomyelia found with pediatric Chiari I malformation

Eccentric syringes associated with Chiari I malformation have received scant attention in the medical literature. Herein, we describe our experience and long-term outcome in patients with this finding. A retrospective analysis of a Chiari I database was performed. Patients known to have an associated syringomyelia were then further analyzed for the type of syrinx present. When an eccentric syrinx was noted, the symptoms and postoperative course of these patients were analyzed. Of well over 500 operative cases of Chiari I malformation, roughly 70 % (pre-syrinx and minimally dilated central canals were excluded) were found to have an associated syringomyelia. Of these, four patients were found to have an eccentrically positioned syrinx. Three of these cases presented with symptoms referable to the side of the eccentric syrinx. Postoperatively, cases with both a central and eccentrically located syrinx were found to have a greater decrease in the size of the central portion of their syrinx compared to the eccentrically located portion. Symptoms decreased in all patients. The minority of our patients with hindbrain-induced syringomyelia were found to have an eccentrically located syrinx. Of these, most will have symptoms localized to the abnormal fluid-filled cavity, and these may not decrease in size as much as centrally located syringes following posterior fossa decompression. However, all symptoms decreased in those operated. Based on the literature, non-hindbrain-induced syringomyelia is more likely to result in an eccentrically placed syrinx. The mechanism for this is yet to be elucidated.

CHERI

Chiari I malformations are congenital abnormalities that are etiologically heterogeneous. Some studies recognize a probable association between Chiari 1 malformation, delayed language or motor development, and mental retardation with or without epilepsy. The present patient appeared to have an isolated cleft palate and Chiari 1 malformation with co-existing functional and behavioral disorders (i.e., speech delay, mental retardation, and deviant electroencephalography [EEG]). In consideration of the cleft palate population, the implication of the present case study is that more attention should be paid to the developmental milestones in children with cleft palate. Because several cases of Chiari I anomaly co-occurring with mental retardation and deviant EEG or epilepsy have been recognized, it appears justified to identify a syndrome, which is here called CHERI (Chiari 1 malformation with or without cleft palate, deviant EEG or epilepsy, and retarded intelligence with delayed language development). It will be important in the future to describe those cases identified to delineate the clinical picture and to estimate the frequency of the occurrence of various characteristics.

Comparison of syringopleural and syringosubarachnoid shunting in the treatment of syringomyelia in children

Case records from the Montreal Children's Hospital containing the diagnosis of shunted syringomyelia were retrospectively reviewed. From 1984 to 1994, 31 patients had their syrinx treated by either syringopleural (19 cases, Group A) or syringosubarachnoid (13 cases, Group B) shunting. One patient was included in both groups. Associated diagnoses included: in Group A, two cases of Chiari I and 14 of Chiari II malformations, 14 cases of shunted hydrocephalus, 13 cases of spina bifida aperta, and three cases of spina bifida occulta; Group B, four cases of Chiari I and two of Chiari II malformations, four cases of shunted hydrocephalus, two cases of spina bifida aperta, and five cases of spina bifida occulta. Eight Group A and six Group B patients had undergone prior posterior fossa decompression. Motor deficits predominated in both groups and arachnoiditis was a uniform operative finding. Neurological follow-up examinations showed 11 Group A patients improved and eight stabilized, whereas on magnetic resonance imaging, 12 cavities appeared to have collapsed, five were markedly reduced, and one had increased. One patient underwent reoperation for pleural effusions and one for shunt displacement. In Group B, one patient improved, eight stabilized, three worsened neurologically, and one was lost to follow-up review. Radiologically, one cavity appeared to have collapsed, six were significantly reduced, two were unchanged, and three had enlarged. The authors conclude that syringopleural shunting is a valuable option for controlling syringomyelia in patients without Chiari malformation or in patients who have previously undergone a craniovertebral decompression or are otherwise asymptomatic from their Chiari malformation.

脊髄内空洞性病変の臨床的検討

A retrospective study of 21 patients with syrinx was carried out about the clinical symptoms, signs, sizes of syrinx and surgical results. There were 5 cases of Chiari I malformation, 5 cases of intramedullary tumor, 4 cases of adhesive arachnoiditis, 4 cases of post-traumatic and 3 cases of idiopathic pathogenesis. MRI was useful in differential diagnosis. Better results were obtained in Chiari I malformation managed by craniovertebral decompression.

Chiari's network

1. 1. A case of Chiari's network is presented. In this case potentially fatal embolization of the lung was prevented by the embolus becoming lodged in the fibers of the net. 2. 2. The suggested clinical implications of this anomaly, both as applied to the presently reported case and to those in the literature, are reviewed.

Chiari's syndrome—Obliterative endophlebitis of the hepatic veins

A case of Chiari's syndrome, idiopathic obliterative endophlebitis of the hepatic veins, is presented with the clinical and pathologic findings. A distinction is made between obliterative endophlebitis of the hepatic veins as seen in this syndrome and occlusion due to other intrahepatic or extrahepatic diseases in which the hepatic veins are secondarily involved.

THROMBOSIS OF THE VENA CAVA AND HEPATIC VEINS (CHIARI'S SYNDROME) IN A PATIENT WITH NEPHROSIS

A CASE of Chiari's syndrome is reported, with autopsy and observations on the serum phosphatase level and the prothrombin time of the plasma. <h3>REPORT OF CASE</h3> M. T., a 31/2 year old white boy, was admitted to the Children's Hospital on June 2, 1945, with a history of a "cold" followed by swelling of the face and extremities, oliguria and anorexia. Examination revealed generalized edema and a moderate amount of fluid in the peritoneal cavity. The blood pressure was 98 mm. of mercury systolic and 60 mm. diastolic. The nonprotein nitrogen of the serum measured 34 mg., the total protein 3.8 Gm. and the albumin 1.5 Gm., per hundred cubic centimeters. The serum cholesterol was 572 mg. per hundred cubic centimeters one month after admission and 1,088 mg. two weeks later. His urine contained albumin, a few white blood cells and granular casts. A diagnosis of lipid nephrosis (nephrotic syndrome)