Chiari malformation in a restricted-resource setting and treatment challenges: Case report and literature review

Chiari malformation in a restricted-resource setting and treatment challenges: Case report and literature review | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Chiari malformation in a restricted-resource setting and treatment challenges: Case report and literature review Turyalai Hakimi, Khalid Mohammad Qasem, Zamaryalai Hakimi, Mohammad Anwar Jawed This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-3924230/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Background Chiari malformations encompass many abnormalities in the skull, primarily impacting the cerebellum and typically involving the back part of the skull. It is defined by the downward displacement of one or both cerebellar tonsils via the foramen magnum. The clinical manifestations are a result of impaired function in the brainstem and lower cranial nerves. Decompressive surgery, with or without duraplasty, is the preferred therapeutic option. However, it can be particularly difficult in low-resource settings due to a lack of technical facilities. Case presentation A 40-day-old infant was brought to our pediatric surgery unit with a large cystic mass located in her occipital area. The patient was administered a suitable antibiotic regimen in response to the respiratory illness and prepped for definitive treatment. She underwent tension-free decompressive repair with the preservation of both cerebellar tonsils without duraplasty for the purpose of preventing post-operative complications, mostly hydrocephalus. The surgical procedure and postoperative period were without any notable incidents. Conclusion Chiari malformation is a complex neurosurgical condition with varying levels of surgical outcome, depending on the extent of the problem. A specialized pediatric neurosurgical center and a controlled follow-up system are the essential components of better surgical results and enhancing the quality of life. Chiari malformation encephalocele neural tube defect cerebellum case report Figures Figure 1 Figure 2 Figure 3 Introduction Chiari malformation (CM) refers to a collection of defects in the central nervous system (CNS) that impact the hindbrain and posterior cranial fossa. These abnormalities occur at the typical junction of the skull and spinal canal. The condition is characterized by the downward displacement of the cerebellar tonsils through the foramen magnum and is commonly linked to syringomyelia [ 1 , 2 ]. The typical symptoms that patients experience include pain in the back of the head and neck, as well as headaches that worsen when doing ordinary Valsalva maneuvers such as laughing, sneezing, or coughing [ 3 , 4 ]. The current therapeutic approach for CM involves a synergistic combination of medicinal and surgical interventions. Medical treatment entails providing relief for symptoms with non-steroidal anti-inflammatory drugs and muscle relaxants. Surgical management, on the other hand, involves decompressive intervention at the foramen magnum, with or without duraplasty [ 5 ]. Case presentation Here, we present a 40-day-old infant with a large cystic mass located in the occipital area (Fig. 1 ). The patient was referred to our teaching hospital pediatric surgery department from the western region of the capital due to the mentioned condition. The patient's consanguinity was confirmed, but there were no notable findings in the antenatal history. The patient's remaining two siblings were stillborn. The patient's mother was administered specific drugs during pregnancy (due to certain underlying problems for which she could not present any documents or prescriptions) and had an uncomplicated childbirth. The patient was admitted during the winter season and got suitable supportive care in addition to being scheduled for the surgical procedure. All the routine and biochemical laboratory tests were conducted and yielded normal results. The family could not afford to pay for advanced imaging modalities such as computerized tomography scanning (CT-Scan) and magnetic resonance imaging (MRI) at a private diagnostic clinic. The patient underwent tension-free posterior fossa decompressive surgery, preserving both cerebellar tonsils without duraplasty, for the purpose of preventing subsequent post-operative hydrocephalus. (Fig. 2 ) The patient was monitored for a duration of one year without presentation due to encountering any complications (Fig. 3 at the sixth month of follow-up). Discussion There is a cluster of birth abnormalities affecting the cerebellum, pons, and medulla oblongata in the posterior fossa and hindbrain known as Arnold-Chiari or Chiari malformation. A variety of problems can arise, including herniation of the cerebellar tonsils via the foramen magnum or even the complete absence of the cerebellum itself, along with or in addition to hydrocephalus, syrinx, encephalocele, or spinal dysraphism [ 6 ]. CMs are classified based on their severity and anatomic deformities from 0–4 degrees. CM 0 is characterized by syringomyelia without the herniation of the hindbrain. CM I is characterized by the projection of one or both cerebellar tonsils 5mm below the foramen magnum measured by the McRae Line (a radiographic line drawn on a lateral skull radiograph or on a midsagittal section of CT or MRI that connects the anterior and posterior margins of the foramen magnum (basion to opisthion). CM II is represented by the herniation of the brainstem and the towering of the cerebellum, in addition to the herniation of the cerebellar tonsils and vermis due to an open distal spinal dysraphism or myelomeningocele. CM III is characterized by herniation of the hindbrain (cerebellum with or without the brainstem) into a high cervical or low occipital meningoencephalocele, whereas in CM IV, the problem is similar to that of primary cerebellar agenesis and is demonstrated by severe cerebellar hypoplasia [ 7 , 8 ]. The CM is believed to have several etiological components, including molecular, hydrodynamic, and mechanical theories. CM occurs when the cerebellar tonsils protrude through the foramen magnum due to a smaller than normal posterior fossa. Possible causes include congenital hypoplasia, acquired morphologic alterations such as premature suture closure, calvarial dysplasia, or genetic and syndromic factors. CM II and III are characterized by an open neural tube defect called myelomeningocele. This defect causes a change in the flow of cerebrospinal fluid, leading to a fourth ventricle that cannot maintain its normal size. As a result, the posterior fossa becomes underdeveloped, and the cerebellar tonsils herniate due to the ongoing collapse of the fourth ventricle during fetal development. The cause of the remaining CM variations is yet unknown [ 9 , 10 ]. The prevalence of CM I ranges from 0.5 to 3.5%, while CM II occurs in approximately 0.44 per 1000 newborns. The remaining types of CM are far less common [ 11 ]. Suboccipital headaches, coupled with other manifestations such as visual problems, symptoms related to the ear and nervous system (dizziness, hearing impairment, vertigo), an unsteady gait, and overall tiredness, are frequently observed. The typical manifestations of myelopathy include dissociated sensory loss, characterized by the absence of pain and temperature sensation while fine touch and proprioception remain intact, as well as motor weakness. Compression of the cerebellum or medulla at the foramen magnum, whether caused by syringomyelia, syringobulbia, or direct pressure, can lead to impairments in the lower cranial nerves (IX, X, XI, and XII CN) as well as cerebellar symptoms such as ataxia, dysmetria, and nystagmus [ 12 , 13 ]. The most effective method for assessing CMs in both children and adults is MRI, which reveals the downward displacement of the cerebellar tonsils, the reduced size of the posterior fossa, and the presence of a syrinx. If it is not possible to obtain an MRI, myelography is the preferred alternative. A cranial CT scan or X-ray can reveal the craniovertebral junction's osseous abnormality, which is crucial for surgical strategizing, such as addressing basilar invagination. Fetal sonography will reveal the presence of the lemon sign, a distinctive characteristic of CM II and III. This sign is characterized by the abnormal flattening, inward bending, or scalloping of the anterior frontal calvarium, resulting in the loss of its normal convex curvature. This imaging modality can also detect the classic sign of CM II and distal neural tube damage, known as the banana sign. The banana sign is defined by aberrant morphology, namely an anterior curvature of the cerebellum and obliteration of the cisterna magna [ 14 ]. Laboratory tests are not beneficial in assessing CMs, although they are necessary for surgical interventions. Asymptomatic individuals should be treated in a cautious manner, employing analgesics and muscle relaxants. Decompressive surgery is the definitive treatment. Its purpose is to restore the flow of cerebrospinal fluid (CSF) and alleviate the compression on the cerebellum and hindbrain. Surgery is recommended for persistently symptomatic patients with tonsillar herniation, preferably performed within two years of the onset of symptoms. Posterior fossa decompression is the conventional surgical approach for treating CM I. The surgical technique entails the removal of a portion of the skull at the back of the head, known as suboccipital craniectomy, to enlarge the opening at the base of the skull called the foramen magnum. Additionally, it involves the removal of the laminae of the first and second cervical vertebrae, referred to as C1 and C2 laminectomy. The dura may be either opened or left intact, and if there are any arachnoid adhesions, they are carefully separated. The decision to undergo duraplasty depends on the extent of dural enlargement and the size of the posterior fossa. The occipital fascia or tensor fascia lata (TFL) tendon can be utilized as an autograft or artificial dura for duraplasty. If decompression alone proves ineffective in treating syrinx, it is desirable to consider the use of a shunt. An alternative procedure that might be carried out is tonsillar cauterization [ 15 , 16 ]. The surgical treatment for CM II comprises the correction of myelomeningocele, ideally during the initial 48 hours. This can also be accomplished prenatally by means of a hysterotomy. The closure of spinal dysraphism may necessitate primary skin closure, myocutaneous flap, or fasciocutaneous flap, contingent upon the extent of the issue, the affected layers, and the neighboring tissue that is accessible. Most individuals with hydrocephalus will ultimately require a ventricular shunt for the purpose of CSF diversion. If necessary, a further decompression is conducted to facilitate expansion of the suboccipital region [ 17 ]. The surgical remedy for CM III is similar to that of CM II. It includes repairing the encephalocele, removing non-viable herniated contents, closing the dura, and performing a cranioplasty [ 18 ]. The prognosis for CM I and II is favorable in the absence of any neurological impairment. CMs of a severe kind are associated with an unfavorable prognosis [ 19 ]. Pseudomeningocele may occur in certain patients and may necessitate drainage. As a result, it is essential to monitor CSF leakage during the postoperative phase. Post-operatively, patients should be tracked according to the Chicago Chiari Outcome Scale [ 20 ]. Based on the clinical presentation, the index case is considered as a CM II with cervical encephalocele. Despite the prevailing literature advocating for the removal of the cerebellar tonsils [ 15 , 16 ], we chose to retain them due to their role in regulating balance and coordinating voluntary movements in the extremities. The rationale for our implementation of tension-free duraplasty was to mitigate the occurrence of hydrocephalus. Nonetheless, throughout the postoperative period of observation, a minor protrusion was observed in the occipital region, but it did not raise any concerns. This will allow for the maturation of the skull and the accommodation of the minor bulge within the fully formed cranial cavity. Otherwise, the subsequent repair procedure would be scheduled accordingly. Conclusion CM are neurological conditions that primarily affect children and involve various abnormalities in the hindbrain. In societies where the follow-up of these entities is difficult due to a lack of awareness of the condition and a low socioeconomic status, the best treatment modality is tension-free decompressive surgery. This procedure aims to prevent post-operative sequelae, including hydrocephalus. Declarations Acknowledgment Not applicable. Authors’ contributions Turyalai Hakimi (TH) conceptualized the manuscript, reviewed the literature, and wrote the original draft. (TH), and Mohammad Anwar Jawed (MAJ) performed the surgical procedure. TH, Khalid Mohammad Qasem (KMQ) and Zamaryalai Hakimi (ZH) designed and edited the study. TH supervised the entire study process. All authors read and approved the final manuscript. Funding No fund and grant. Availability of data and materials No datasets were generated or analyzed during the current study. Ethics approval and consent to participate Not applicable. Consent for publication Written informed consent was obtained from the patient parents for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request. Competing interest The authors have no conflict of interest. References Hidalgo JA, Tork CA, Varacallo M. Arnold Chiari Malformation. In StatPearls ; StatPearls Publishing LLC.: Treasure Island, FL, USA, 2021. [Google Scholar]. Brugliera L, Iannaccone S, Nocera G, Cimino P, D’angelo G, Mortini P, Capodaglio P, Spina A. Therapeutic cannabis for pain management in a patient with Chiari malformation type I during concomitant SARS-COV-2 infection. J Neurosurg Sci. 2023;67:129–30. [Google Scholar] [CrossRef]. Tubbs RS, Beckman J, Naftel RP, Chern JJ, Wellons JC, Rozzelle CJ, Blount JP, Oakes WJ. Institutional experience with 500 cases of surgically treated pediatric Chiari malformation Type I. J Neurosurg Pediatr. 2011;7:248–56. [Google Scholar] [CrossRef]. McClugage SG, Oakes WJ. The Chiari I malformation. J Neurosurg Pediatr. 2019;24:217–26. [Google Scholar] [CrossRef]. Labuda R, Nwotchouang BST, Ibrahimy A, Allen PA, Oshinski JN, Klinge P, Loth F. A new hypothesis for the pathophysiology of symptomatic adult Chiari malformation Type I. Med Hypotheses. 2021;158:110740. [Google Scholar] [CrossRef]. Bhimani AD, Esfahani DR, Denyer S, Chiu RG, Rosenberg D, Barks AL, Arnone GD, Mehta AI. Adult Chiari I Malformations: An Analysis of Surgical Risk Factors and Complications Using an International Database. World Neurosurg. 2018;115:e490–e500. [PubMed]. Arora R. Imaging spectrum of cerebellar pathologies: a pictorial essay. Pol J Radiol. 2015;80:142–50. [PMC free article] [PubMed]. Kim IK, Wang KC, Kim IO, Cho BK. Chiari 1.5 malformation: an advanced form of Chiari I malformation. J Korean Neurosurg Soc. 2010;48(4):375–9. [PMC free article] [PubMed]. Giammattei L, Borsotti F, Parker F, Messerer M. Chiari I malformation: surgical technique, indications and limits. Acta Neurochir (Wien). 2018;160(1):213–7. [PubMed]. Markunas CA, Enterline DS, Dunlap K, Soldano K, Cope H, Stajich J, Grant G, Fuchs H, Gregory SG, Ashley-Koch AE. Genetic evaluation and application of posterior cranial fossa traits as endophenotypes for Chiari type I malformation. Ann Hum Genet. 2014;78(1):1–12. [PMC free article] [PubMed]. Langridge B, Phillips E, Choi D. Chiari Malformation Type 1: A Systematic Review of Natural History and Conservative Management. World Neurosurg. 2017;104:213–9. [PubMed]. Zhang D, Melikian R, Papavassiliou E. Chiari I Malformation Presenting as Shoulder Pain, Weakness, and Muscle Atrophy in a Collegiate Athlete. Curr Sports Med Rep. 2016 Jan-Feb;15(1):10–2. [PubMed]. Jayamanne C, Fernando L, Mettananda S. Chiari malformation type 1 presenting as unilateral progressive foot drop: a case report and review of literature. BMC Pediatr. 2018;18(1):34. [PMC free article] [PubMed]. Klekamp J. How Should Syringomyelia be Defined and Diagnosed? World Neurosurg. 2018;111:e729–45. [PubMed]. Lin W, Duan G, Xie J, Shao J, Wang Z, Jiao B. Comparison of Results Between Posterior Fossa Decompression with and without Duraplasty for the Surgical Treatment of Chiari Malformation Type I: A Systematic Review and Meta-Analysis. World Neurosurg. 2018;110:460–474.e5. [PubMed]. Chen J, Li Y, Wang T, Gao J, Xu J, Lai R, Tan D. Comparison of posterior fossa decompression with and without duraplasty for the surgical treatment of Chiari malformation type I in adult patients: A retrospective analysis of 103 patients. Med (Baltim). 2017;96(4):e5945. [PMC free article] [PubMed]. Messing-Jünger M, Röhrig A. Primary and secondary management of the Chiari II malformation in children with myelomeningocele. Childs Nerv Syst. 2013;29(9):1553–62. [PubMed]. Işik N, Elmaci I, Silav G, Celik M, Kalelioğlu M. Chiari malformation type III and results of surgery: a clinical study: report of eight surgically treated cases and review of the literature. Pediatr Neurosurg. 2009;45(1):19–28. [PubMed]. Goel A, Gore S, Shah A, Dharurkar P, Vutha R, Patil A. Atlantoaxial Fixation for Chiari 1 Formation in Pediatric Age-Group Patients: Report of Treatment in 33 Patients. World Neurosurg. 2018;111:e668–77. [PubMed]. Gilmer HS, Xi M, Young SH. Surgical Decompression for Chiari Malformation Type I: An Age-Based Outcomes Study Based on the Chicago Chiari Outcome Scale. World Neurosurg. 2017;107:285–90. [PubMed]. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-3924230","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":271953913,"identity":"003d6afb-3f32-415e-b529-5c5c6628bab4","order_by":0,"name":"Turyalai Hakimi","email":"data:image/png;base64,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","orcid":"","institution":"Kabul University of Medical Science, Maiwand Teaching Hospital","correspondingAuthor":true,"prefix":"","firstName":"Turyalai","middleName":"","lastName":"Hakimi","suffix":""},{"id":271953914,"identity":"a63e31a3-60ea-4e13-a232-093d9419add1","order_by":1,"name":"Khalid Mohammad Qasem","email":"","orcid":"","institution":"Kabul University of Medical Science, Ali Abad Teaching Hospital","correspondingAuthor":false,"prefix":"","firstName":"Khalid","middleName":"Mohammad","lastName":"Qasem","suffix":""},{"id":271953915,"identity":"17a46fa9-a236-499c-8790-073fd86020d2","order_by":2,"name":"Zamaryalai Hakimi","email":"","orcid":"","institution":"Kabul University of Medical Science","correspondingAuthor":false,"prefix":"","firstName":"Zamaryalai","middleName":"","lastName":"Hakimi","suffix":""},{"id":271953916,"identity":"b40689b5-8264-4053-92d8-3fc42193b3e7","order_by":3,"name":"Mohammad Anwar Jawed","email":"","orcid":"","institution":"Kabul University of Medical Science, Maiwand Teaching Hospital","correspondingAuthor":false,"prefix":"","firstName":"Mohammad","middleName":"Anwar","lastName":"Jawed","suffix":""}],"badges":[],"createdAt":"2024-02-03 14:19:17","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-3924230/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-3924230/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":51018239,"identity":"a0cc869e-0a95-4719-89d3-1c86daa2d250","added_by":"auto","created_at":"2024-02-12 19:21:14","extension":"jpg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":45269,"visible":true,"origin":"","legend":"\u003cp\u003eCystic mass in the occipital region\u003c/p\u003e","description":"","filename":"Fig.1.jpg","url":"https://assets-eu.researchsquare.com/files/rs-3924230/v1/4e54f2f54fc655ebf9cad291.jpg"},{"id":51018235,"identity":"d3643883-953c-4120-bc5a-a7ca7f6dd4d2","added_by":"auto","created_at":"2024-02-12 19:21:11","extension":"jpg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":49015,"visible":true,"origin":"","legend":"\u003cp\u003eshowing intraoperative view\u003c/p\u003e","description":"","filename":"Fig.2.jpg","url":"https://assets-eu.researchsquare.com/files/rs-3924230/v1/9ddd304ba34cdb41e6eb372d.jpg"},{"id":51018238,"identity":"cc435f97-4caa-4886-b92e-e3acf35ce9c7","added_by":"auto","created_at":"2024-02-12 19:21:13","extension":"jpg","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":32602,"visible":true,"origin":"","legend":"\u003cp\u003ePost-operative view\u003c/p\u003e","description":"","filename":"Fig.3.jpg","url":"https://assets-eu.researchsquare.com/files/rs-3924230/v1/7456bbe223035033d5cea6ed.jpg"},{"id":51333981,"identity":"0ab0510f-1b62-41e0-9eb0-90fa39a47e83","added_by":"auto","created_at":"2024-02-19 18:25:10","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":405046,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-3924230/v1/0979315d-4dc0-4524-88d3-a9a4b82dd3b0.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Chiari malformation in a restricted-resource setting and treatment challenges: Case report and literature review","fulltext":[{"header":"Introduction","content":"\u003cp\u003eChiari malformation (CM) refers to a collection of defects in the central nervous system (CNS) that impact the hindbrain and posterior cranial fossa. These abnormalities occur at the typical junction of the skull and spinal canal. The condition is characterized by the downward displacement of the cerebellar tonsils through the foramen magnum and is commonly linked to syringomyelia [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. The typical symptoms that patients experience include pain in the back of the head and neck, as well as headaches that worsen when doing ordinary Valsalva maneuvers such as laughing, sneezing, or coughing [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. The current therapeutic approach for CM involves a synergistic combination of medicinal and surgical interventions. Medical treatment entails providing relief for symptoms with non-steroidal anti-inflammatory drugs and muscle relaxants. Surgical management, on the other hand, involves decompressive intervention at the foramen magnum, with or without duraplasty [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e].\u003c/p\u003e"},{"header":"Case presentation","content":"\u003cp\u003eHere, we present a 40-day-old infant with a large cystic mass located in the occipital area (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). The patient was referred to our teaching hospital pediatric surgery department from the western region of the capital due to the mentioned condition. The patient's consanguinity was confirmed, but there were no notable findings in the antenatal history. The patient's remaining two siblings were stillborn. The patient's mother was administered specific drugs during pregnancy (due to certain underlying problems for which she could not present any documents or prescriptions) and had an uncomplicated childbirth. The patient was admitted during the winter season and got suitable supportive care in addition to being scheduled for the surgical procedure. All the routine and biochemical laboratory tests were conducted and yielded normal results. The family could not afford to pay for advanced imaging modalities such as computerized tomography scanning (CT-Scan) and magnetic resonance imaging (MRI) at a private diagnostic clinic. The patient underwent tension-free posterior fossa decompressive surgery, preserving both cerebellar tonsils without duraplasty, for the purpose of preventing subsequent post-operative hydrocephalus. (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e) The patient was monitored for a duration of one year without presentation due to encountering any complications (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003e at the sixth month of follow-up).\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eThere is a cluster of birth abnormalities affecting the cerebellum, pons, and medulla oblongata in the posterior fossa and hindbrain known as Arnold-Chiari or Chiari malformation. A variety of problems can arise, including herniation of the cerebellar tonsils via the foramen magnum or even the complete absence of the cerebellum itself, along with or in addition to hydrocephalus, syrinx, encephalocele, or spinal dysraphism [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eCMs are classified based on their severity and anatomic deformities from 0\u0026ndash;4 degrees. CM 0 is characterized by syringomyelia without the herniation of the hindbrain. CM I is characterized by the projection of one or both cerebellar tonsils 5mm below the foramen magnum measured by the McRae Line (a radiographic line drawn on a lateral skull radiograph or on a midsagittal section of CT or MRI that connects the anterior and posterior margins of the foramen magnum (basion to opisthion). CM II is represented by the herniation of the brainstem and the towering of the cerebellum, in addition to the herniation of the cerebellar tonsils and vermis due to an open distal spinal dysraphism or myelomeningocele. CM III is characterized by herniation of the hindbrain (cerebellum with or without the brainstem) into a high cervical or low occipital meningoencephalocele, whereas in CM IV, the problem is similar to that of primary cerebellar agenesis and is demonstrated by severe cerebellar hypoplasia [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e, \u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe CM is believed to have several etiological components, including molecular, hydrodynamic, and mechanical theories. CM occurs when the cerebellar tonsils protrude through the foramen magnum due to a smaller than normal posterior fossa. Possible causes include congenital hypoplasia, acquired morphologic alterations such as premature suture closure, calvarial dysplasia, or genetic and syndromic factors. CM II and III are characterized by an open neural tube defect called myelomeningocele. This defect causes a change in the flow of cerebrospinal fluid, leading to a fourth ventricle that cannot maintain its normal size. As a result, the posterior fossa becomes underdeveloped, and the cerebellar tonsils herniate due to the ongoing collapse of the fourth ventricle during fetal development. The cause of the remaining CM variations is yet unknown [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e, \u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe prevalence of CM I ranges from 0.5 to 3.5%, while CM II occurs in approximately 0.44 per 1000 newborns. The remaining types of CM are far less common [\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. Suboccipital headaches, coupled with other manifestations such as visual problems, symptoms related to the ear and nervous system (dizziness, hearing impairment, vertigo), an unsteady gait, and overall tiredness, are frequently observed. The typical manifestations of myelopathy include dissociated sensory loss, characterized by the absence of pain and temperature sensation while fine touch and proprioception remain intact, as well as motor weakness. Compression of the cerebellum or medulla at the foramen magnum, whether caused by syringomyelia, syringobulbia, or direct pressure, can lead to impairments in the lower cranial nerves (IX, X, XI, and XII CN) as well as cerebellar symptoms such as ataxia, dysmetria, and nystagmus [\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e, \u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe most effective method for assessing CMs in both children and adults is MRI, which reveals the downward displacement of the cerebellar tonsils, the reduced size of the posterior fossa, and the presence of a syrinx. If it is not possible to obtain an MRI, myelography is the preferred alternative. A cranial CT scan or X-ray can reveal the craniovertebral junction's osseous abnormality, which is crucial for surgical strategizing, such as addressing basilar invagination. Fetal sonography will reveal the presence of the lemon sign, a distinctive characteristic of CM II and III. This sign is characterized by the abnormal flattening, inward bending, or scalloping of the anterior frontal calvarium, resulting in the loss of its normal convex curvature. This imaging modality can also detect the classic sign of CM II and distal neural tube damage, known as the banana sign. The banana sign is defined by aberrant morphology, namely an anterior curvature of the cerebellum and obliteration of the cisterna magna [\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e]. Laboratory tests are not beneficial in assessing CMs, although they are necessary for surgical interventions.\u003c/p\u003e \u003cp\u003eAsymptomatic individuals should be treated in a cautious manner, employing analgesics and muscle relaxants. Decompressive surgery is the definitive treatment. Its purpose is to restore the flow of cerebrospinal fluid (CSF) and alleviate the compression on the cerebellum and hindbrain. Surgery is recommended for persistently symptomatic patients with tonsillar herniation, preferably performed within two years of the onset of symptoms. Posterior fossa decompression is the conventional surgical approach for treating CM I. The surgical technique entails the removal of a portion of the skull at the back of the head, known as suboccipital craniectomy, to enlarge the opening at the base of the skull called the foramen magnum. Additionally, it involves the removal of the laminae of the first and second cervical vertebrae, referred to as C1 and C2 laminectomy. The dura may be either opened or left intact, and if there are any arachnoid adhesions, they are carefully separated. The decision to undergo duraplasty depends on the extent of dural enlargement and the size of the posterior fossa. The occipital fascia or tensor fascia lata (TFL) tendon can be utilized as an autograft or artificial dura for duraplasty. If decompression alone proves ineffective in treating syrinx, it is desirable to consider the use of a shunt. An alternative procedure that might be carried out is tonsillar cauterization [\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e, \u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe surgical treatment for CM II comprises the correction of myelomeningocele, ideally during the initial 48 hours. This can also be accomplished prenatally by means of a hysterotomy. The closure of spinal dysraphism may necessitate primary skin closure, myocutaneous flap, or fasciocutaneous flap, contingent upon the extent of the issue, the affected layers, and the neighboring tissue that is accessible. Most individuals with hydrocephalus will ultimately require a ventricular shunt for the purpose of CSF diversion. If necessary, a further decompression is conducted to facilitate expansion of the suboccipital region [\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe surgical remedy for CM III is similar to that of CM II. It includes repairing the encephalocele, removing non-viable herniated contents, closing the dura, and performing a cranioplasty [\u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e]. The prognosis for CM I and II is favorable in the absence of any neurological impairment. CMs of a severe kind are associated with an unfavorable prognosis [\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e]. Pseudomeningocele may occur in certain patients and may necessitate drainage. As a result, it is essential to monitor CSF leakage during the postoperative phase. Post-operatively, patients should be tracked according to the Chicago Chiari Outcome Scale [\u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eBased on the clinical presentation, the index case is considered as a CM II with cervical encephalocele. Despite the prevailing literature advocating for the removal of the cerebellar tonsils [\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e, \u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e], we chose to retain them due to their role in regulating balance and coordinating voluntary movements in the extremities. The rationale for our implementation of tension-free duraplasty was to mitigate the occurrence of hydrocephalus. Nonetheless, throughout the postoperative period of observation, a minor protrusion was observed in the occipital region, but it did not raise any concerns. This will allow for the maturation of the skull and the accommodation of the minor bulge within the fully formed cranial cavity. Otherwise, the subsequent repair procedure would be scheduled accordingly.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eCM are neurological conditions that primarily affect children and involve various abnormalities in the hindbrain. In societies where the follow-up of these entities is difficult due to a lack of awareness of the condition and a low socioeconomic status, the best treatment modality is tension-free decompressive surgery. This procedure aims to prevent post-operative sequelae, including hydrocephalus.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eAcknowledgment\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors\u0026rsquo; contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eTuryalai Hakimi (TH) conceptualized the manuscript, reviewed the literature, and wrote the original draft. (TH), and Mohammad Anwar Jawed (MAJ) performed the surgical procedure. TH, Khalid Mohammad Qasem (KMQ) and Zamaryalai Hakimi (ZH) designed and edited the study. TH supervised the entire study process. All authors read and approved the final manuscript.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eNo fund and grant.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and materials\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNo datasets were generated or analyzed during the current study.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWritten informed consent was obtained from the patient parents for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interest\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors have no conflict of interest.\u0026nbsp;\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eHidalgo JA, Tork CA, Varacallo M. Arnold Chiari Malformation. In \u003cem\u003eStatPearls\u003c/em\u003e; StatPearls Publishing LLC.: Treasure Island, FL, USA, 2021. [Google Scholar].\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eBrugliera L, Iannaccone S, Nocera G, Cimino P, D\u0026rsquo;angelo G, Mortini P, Capodaglio P, Spina A. Therapeutic cannabis for pain management in a patient with Chiari malformation type I during concomitant SARS-COV-2 infection. J Neurosurg Sci. 2023;67:129\u0026ndash;30. 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[PubMed].\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Chiari malformation, encephalocele, neural tube, defect, cerebellum, case report","lastPublishedDoi":"10.21203/rs.3.rs-3924230/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-3924230/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eBackground\u003c/h2\u003e \u003cp\u003eChiari malformations encompass many abnormalities in the skull, primarily impacting the cerebellum and typically involving the back part of the skull. It is defined by the downward displacement of one or both cerebellar tonsils via the foramen magnum. The clinical manifestations are a result of impaired function in the brainstem and lower cranial nerves. Decompressive surgery, with or without duraplasty, is the preferred therapeutic option. However, it can be particularly difficult in low-resource settings due to a lack of technical facilities.\u003c/p\u003e\u003ch2\u003eCase presentation\u003c/h2\u003e \u003cp\u003eA 40-day-old infant was brought to our pediatric surgery unit with a large cystic mass located in her occipital area. The patient was administered a suitable antibiotic regimen in response to the respiratory illness and prepped for definitive treatment. She underwent tension-free decompressive repair with the preservation of both cerebellar tonsils without duraplasty for the purpose of preventing post-operative complications, mostly hydrocephalus. The surgical procedure and postoperative period were without any notable incidents.\u003c/p\u003e\u003ch2\u003eConclusion\u003c/h2\u003e \u003cp\u003eChiari malformation is a complex neurosurgical condition with varying levels of surgical outcome, depending on the extent of the problem. A specialized pediatric neurosurgical center and a controlled follow-up system are the essential components of better surgical results and enhancing the quality of life.\u003c/p\u003e","manuscriptTitle":"Chiari malformation in a restricted-resource setting and treatment challenges: Case report and literature review","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-02-12 19:20:29","doi":"10.21203/rs.3.rs-3924230/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"fd1be2de-d237-4fc0-ae16-19da9e05f659","owner":[],"postedDate":"February 12th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2024-02-19T18:17:03+00:00","versionOfRecord":[],"versionCreatedAt":"2024-02-12 19:20:29","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-3924230","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-3924230","identity":"rs-3924230","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}