Arnold-Chiari Type Research Articles

Median Suboccipital Craniotomy caused by Arnold Chiari I Malformation

30-year-old female patient’s report diagnosed with Arnold Chiari type I malformation, who was scheduled for a midline suboccipital craniotomy surgery handled with total intravenous anesthesia (TIVA). The patient was treated with Fentanyl and supported with propofol and dexmedetomidine. Brain monitoring was based on Entropy with ranges between 40-60 during all the intraoperative. It was proved the patient’s safety when submitted to a craniotomy with Arnold Chiari type I malformation with (total intravenous anesthesia) TIVA related to an adequate brain monitoring support and hemodynamic stability.

Neurofibromatosis Type 1 with Arnold Chiari Type 1 Malformation in A Child

Neurofibromatosis Type 1 with Arnold Chiari Type 1 Malformation in A Child

1032 Mixed sleep apnea management in a pediatric patient with Arnold Chiari type 1 and septo-optic dysplasia leading to hypopituitarism

Abstract Introduction Mixed Sleep apnea and Central sleep apnea are commonly noted in a patient with Arnold Chiari Malformation and definitive treatment of Arnold Chiari Malformation is surgical decompression. Report of case(s) The patient at the time of diagnosis of Mixed sleep apnea was 7 years old. She had already undergone tonsillectomy and adenoidectomy at the age of 4 years. Due to continued Sleep-disordered breathing, PSG was done in 2016 which showed predominantly mixed and central events. MRI was done which revealed Chiari 1 malformation with cerebellar tonsils extending 6 mm below the foramen magnum. The patient also due to septo-optic dysplasia and hypopituitarism followed with pediatric endocrinology and was on supplementation of Desmopressin, Hydrocortisone, Levothyroxine, and Somatropin. The mother at the time of pregnancy was a substance abuser, was incarcerated and the primary care was assumed by the patient’s legal guardian. The legal guardian was not interested in surgery for Arnold Chiari Malformation as she feared long-term outcomes and requested alternate treatment options. The patient underwent another split study and started on CPAP. The patient did not tolerate the CPAP and was transitioned to BiPAP. During her last clinic visit, the patient’s compliance had improved and she had achieved therapeutic AHI. The patient was also counseled on the effects of GH supplementation and untreated sleep apnea and is regularly followed by Sleep Medicine and Pediatric Endocrinology. The patient after transitioning to BiPAP has responded well to treatment for Mixed sleep apnea. Despite being on GH supplementation, with careful monitoring of hormone levels by Pediatric Endocrinology and symptom control by Sleep Medicine, the patient has shown remarkable improvement. Conclusion Mixed and central sleep apnea secondary to Arnold Chiari Malformation responds well to surgical decompression with improvement in sleep-disordered breathing. In a pediatric patient with complicated medical history with needing stress dose steroids, not a candidate for surgery, and intolerant to CPAP, BiPAP as a treatment modality showed good control of Sleep Apnea. Support (if any)

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.

Hyper‐IgE syndrome presenting with early life craniosynostosis in monozygotic twin sisters

<scp>Hyper‐IgE</scp> syndrome presenting with early life craniosynostosis in monozygotic twin sisters

Chiari Type III Malformation Presenting With Two Huge Encephaloceles in a New Born Infant: Case Report

Arnold-Chiari type III malformation constitutes the rarest type of all the Chiari malformations. This extremely rare congenital anomaly shows poor prognosis and results in either early death or development of various severe neurologic deficits in the surviving patients. It is characterized by the herniation of hindbrain contents into an encephalocele sac through a bony defect located in the upper cervical or lower occipital region. Magnetic resonance imaging (MRI) is the preferred imaging modality in the diagnosis of this rare congenital anomaly, especially due to its multiplanar capability. In this case report, we described the imaging findings of Arnold-Chiari type III malformation in a newborn male infant. He presented with two huge encephalocele sacs containing dysplastic brain tissues in the upper cervical and lower occipital regions together with various findings related to the disease. Following a right-sided shunt insertion for hydrocephalus treatment, he has undergone a surgical procedure ensuring removal of the encephalocele sacs with primary closure of the bony defects.

Functional performance of children with Myelomeningocele submitted to intrauterine repair and conventional surgery

Introduction: Myelomeningocele (MMC) is an embryonic malformation that occurs in the first four weeks of gestation due to a failure in the closure of the neural tube. They have multifactorial etiology such as genetic and environmental factors that affect folic acid metabolism. Several anomalies may be associated with MMC, with the occurrence of hydrocephalus and Arnold Chiari type II malformation being most present. Surgical repair should ideally occur within the first 24 hours after birth to prevent or decrease the risk of infection, preservation of all viable nerve tissue, anatomical reconstitution. However, surgical repair of MMC intrauterine has shown substantial benefit in all babies submitted to this intervention. Objective: To compare the functional performance of children with MMC who underwent intrauterine surgical repair and those who did traditional surgery at birth. Methods: Cross-sectional study carried out at the UCB School-Clinic, where children treated with MMC were evaluated, analyzing their motor status and functional performance using the Pediatric Evaluation of Disability Inventory (PEDI) for a quantitative record of functional capacity and autonomy in daily activities, in the areas of self-care, mobility and social function. Results: There was a statistically significant relevance on the mobility function (p=0.01) for children who underwent intrauterine repair when compared to children who underwent traditional surgery. Conclusion: There is a strong relationship of better functionality of children who underwent intrauterine surgery when compared to children who underwent traditional surgery.

Use of Thrust Cervical Spinal Manipulative Therapy for Complicated Neck Pain: A Cross-Sectional Survey of Asia-Pacific Chiropractors.

Background Chiropractors often use manual thrust cervical spinal manipulative therapy (thrust-cSMT) to treat musculoskeletal neck conditions. We hypothesized <50% of surveyed Asia-Pacific chiropractors would report using thrust-cSMT given potential contraindications, and secondarily explored predictors of thrust-cSMT use. Materials and methods We designed, validated, achieved sufficient reliability, and disseminated a survey to explore thrust-cSMT use. The survey queried chiropractors' characteristics (e.g., years in practice, education level, time with patients, importance of subluxation), and use of thrust-cSMT for uncomplicated neck pain and vignettes describing vertebral artery disorders, Arnold-Chiari malformation, and anterior cervical discectomy and fusion (ACDF). We performed logistic regression for each vignette with thrust-cSMT as the dependent variable and chiropractor characteristics as covariates. Results There were 241 respondents, having 12.8±10.9 years in practice, representing >15 countries. Less than 50% of chiropractors reported the use of thrust-cSMT for each vignette, including vertebral artery insufficiency (14%) and stenosis (17%), Arnold-Chiari type I (18%) and type II (5%), C5/6 ACDF (39%) and C3-6 ACDF (27%). Regressions identified significant predictors of increased or decreased use of thrust-cSMT including time spent with new patients, focus on subluxation, degree, group practice environment, use of thrust-cSMT on a healthy patient, and hours reading scientific literature (P<.05 for each). Conclusions This study was the first to chiropractors' use of thrust-cSMT for complicated neck pain and found that most Asia-Pacific chiropractors reported avoiding this treatment in the presence of a potential treatment contraindication. The use of thrust-cSMT in complicated neck pain may be related to practice characteristics. However, further research is needed to identify specific reasons why chiropractors use or avoid thrust-cSMT.

Ocular manifestations in Arnold Chiari syndrome

AbstractPurpose: Arnold‐Chiari disease is a rare congenital malformation of the cerebellum. Symptoms are dominated by occipital headache, torticollis, and sometimes swallowing disorders. Regarding ophthalmological manifestations, convergence abnormalities, oculomotor paralysis and diplopia are the main clinical signs. We report two cases of ocular manifestations in Arnold Chiari type 1 syndrome.Methods: Two case reports and a literature review.Results:Case 1: A 40‐year‐old patient with no medical history presented an acute loss of vision in both eyes. On examination, the patient had a visual acuity limited to 5/63. Biomicroscopic examination of the anterior segment was without abnormalities. Fundus examination revealed a bilateral papillary edema. Visual field test showed a borderline diffuse loss in both eyes.Case 2: A 17‐year‐old patient with no medical history presented intermittent headaches and a progressive loss of vision in both eyes. On examination, the patient had a visual acuity limited to 5/80. Biomicroscopic examination of the anterior segment was without abnormalities. Fundus examination revealed atrophy not excavated in both eyes. Visual field test showed nasal and temporal scotomas in both eyes. Temporal, superior and inferior RNFL defects were detected in both eyes. A brain MRI revealed a Chiari type 1 malformation without associated hydrocephalus in both patients.Conclusions: Arnold Chiari type 1 malformation is an uncommon pathology. The discovery of ophthalmologic signs requires a neurosurgical intervention as soon as possible to stop progression towards the loss of visual function by optic atrophy.

Open meningocele associated with a cervical spinal arachnoid cyst and Arnold Chiari malformation type II, a case report

Introduction: Spina bifida is a congenital malformation involving the vertebral spine with different presentations ranging from occult defects to large cutaneous defects exposing tissue and nerves.&#x0D; Case report: Here we describe a patient who had open spina bifida with exposure of the nerve roots, which seemed to be functional on physical examination. A few days after surgery for the closure of the lumbosacral defect, the patient presented with severe respiratory distress and quadriparesis. Magnetic resonance imaging showed a cervical arachnoid cyst and Arnold Chiari type II malformation requiring a second surgical procedure consisting of decompression of the bones and ligaments at the craniospinal junction and microsurgical fenestration of the spinal arachnoid cyst.&#x0D; Discussion: To our knowledge, spinal arachnoid cysts are a rare entity in pediatric&#x0D; patients. On the other hand, spinal dysraphism, of which myelomeningocele is the most frequent congenital form, is common. In a literature search, no previous reports were found of cases similar to ours with open spinal dysraphism with these specific anatomical characteristics associated with an arachnoid cyst in the cervical spine and Arnold Chiari malformation.&#x0D; Conclusion: Spinal dysraphism includes different complex disorders. Full spinal magnetic resonance imaging is recommended for the early detection of associated conditions.

Arnold-Chiari malformation

A case of a 14-year-old girl suffering from intense headaches is presented. At the first pediatrician's examination, the patient had regular findings. Prolonged P100 wave latencies were found by testing the visual evoked potentials. After further examination following repeated headaches, Arnold Chiari type I malformation was diagnosed. Arnold Chiari malformation is classified as downward displacement of one or both cerebellar tonsils through foramen magnum. The most common symptom is occipital or suboccipital headaches that usually happens after hard physical effort. Changes in visual evoked potential latencies can be present before other clinical symptoms and can be a useful tool in the diagnostics of headaches in children and adolescents.

Follow-Up of Newborns with Neural Tube Defects: A Single Center Experience

OBJECTIVES: Neural tube defects, defined as the developmental malformations of the central nervous system, are seen in our country at a rate of three per thousand on average. Our study aims to retrospectively evaluate patients with neural tube defects hospitalized in our intensive care unit for the last decade. STUDY DESIGN: Demographic data, prenatal-maternal characteristics, surgical operation time, intensive care follow-up, and long-term neurological-developmental results of these patients were also evaluated. RESULTS: Eighty-six percent (n=31) of the patients were diagnosed prenatally. Arnold Chiari type 2 and type 3 malformation was 64% (n=23) and 3% (n=1), respectively. Eighty-nine (n=32) percent of these patients had meningomyelocele, whereas 90% (n=27) of them had defects in the lumbosacral region. The patients were operated on the median postnatal 9th day (4-27). Compared patients who were operated on in the 1st week to the patients operated on following the first week, any difference was not detected in terms of neuromotor and cognitive development, and urinary/stool incontinence frequency. Of 28 patients whose long-term follow-up was questioned, 7 (25%), 25 (89%), and 16 (57%) were able to walk, talk, and go to school along with being compatible with their peers, respectively. CONCLUSION: We presented our center's experience to make a significant contribution to the decrease of the neural tube defect frequency, which is commonly seen in our country compared to developed countries.

Assessment of intellectual disability in Klinefelter’s Syndrome associated with other Prenatal and Perinatal risk factors. A case report

IntroductionKlinefelter’s syndrome (KS) is considered as a genetic risk factor for intellectual disability and specifically for impairment on verbal skills. Prenatal and perinatal morbidity concurrent with this diagnosis determine the entity and typology of neurodevelopmental deficits.ObjectivesTo describe the intellectual and adaptive functioning of a patient with KS and prenatal and perinatal difficulties, assessed in the Service of Psychiatry, Clinical Psychology and Mental Health at La Paz University Hospital (Madrid).MethodsA descriptive study was conducted of a 6 year and 7-month-old boy with diagnosis of KS (karyotype XXY), extremely low weight at birth (752 gr.), Arnold Chiari type I malformation with post-hemorrhagic hydrocephalus and periventricular leukomalacia, bronchopulmonary dysplasia, intra-atrial septal defect, left kidney agenesis with iatrogenic adrenal insufficiency, and acute ventriculitis due to E. Coli. Neuropsychological evaluation was carried out in October 2021. Leiter-3 scale was selected due to the absence of expressive language; and his parents completed the questionnaire Adaptive Behavior Assessment System-II.ResultsThe nonverbal intelligence quotient was observed in the very low range (full-scale IQ = 69). The result of general adaptive behavior (z = -2.83), showed very high functional disability both in the conceptual, social, and practical domains.ConclusionsThe high number of causes of disability in this patient is consistent with a high degree of functional disability. Efficient evaluation sessions of intellectual performance, adaptive functioning, and necessary supports, due to the absence of expressive language and limited receptive language, are required. A specific neuropsychological evaluation profile should be established for KS.DisclosureNo significant relationships.

Prevalencia de malformaciones congénitas en la Unidad de Perinatología del Hospital Universitario de Caracas

Objective: To determine the frequency of congenital malformations diagnosed by prenatal ultrasound in the “Dr. Freddy Guevara Zuloaga” Perinatology Unit of the University Hospital of Caracas, Venezuela, in the period between January 1, 2015 and December 31, 2020. Methods: Retrospective, descriptive, cross-sectional study. The statistical records of the Unit were reviewed, with a population of 56,712 and 1844 patients were included in accordance with the inclusion criteria. Results: A general prevalence of 3.3% was obtained. The largest number of studies was conducted in 2016 (12,781). The highest prevalence of congenital malformations was 4.6%, in 2018. In order of frequency, the affected systems were: central nervous system, cardiovascular, genitourinary, gastrointestinal and abdominal wall. Less frequently, abnormalities of the face and neck, chest and lung, musculoskeletal system, and tumors were diagnosed. The most frequent malformations were: pyelic dilation (152 / 8.2%), ventriculomegaly (143 / 7.6%), gastroschisis (114 / 6.1%), Arnold Chiari type II (71 / 3.8%), acraniaexencephaly sequence (66 / 3.5%) and ventricular septal defect (64 / 3.4%). Conclusion: The frequency of congenital malformations diagnosed by ultrasound was 3.3%. The most frequently affected areas were the central nervous, cardiovascular and genitourinary systems.

ФЕТОСКОПИЧЕСКАЯ КОРРЕКЦИЯ МИЕЛОМЕНИНГОЦЕЛЕ – ПЕРВЫЙ РОССИЙСКИЙ ОПЫТ

This study presents the first successful experience of fetoscopic repaire of myelomeningocele (MMC) in the Russian Federation, performed in the fetus at the 27th week of gestational age (GA). The article presents a clinical case of fetoscopic treatment of MMC in a fetus at 27 weeks' gestation. At 18–19 weeks. gestation, an ultrasound examination revealed a congenital malformation of the fetal CNS – spina bifida, MMC of the lumbosacral spine (L2–S1), Arnold-Chiari anomaly type II, hindbrain hernia, occlusive hydrocephalus. Basic endotracheal anesthesia was used in the mother and additional anesthesia in the fetus, which was carried out immediately after insertion of instrumental ports into the uterus. The spinal cord was released by making an incision along the edge of the skin-epithelial zone existing between the placode and the skin, allowing the neural plate to literally fall to the bottom of the spinal canal. Then, a Durepair® prosthesis (Medtronic, USA) was implanted into the edges of the defect. The skin flaps were brought together along the midline using a continuous suture made from the innovative Stratafix® notched suture material (Ethicon, USA). After birth, the results of the control ultrasound and magnetic resonance imaging, as well as motor function in the lower extremities and the state of the sphincters of the bladder neck and rectum were evaluated. A girl was born with a body weight of 2200 g, GA – 36 weeks. The APGAR score was 8–9 points. The results of the primary medical examination revealed a slight decrease in the tone of the muscles of the lower extremities, however, spontaneous movements were noted in both lower extremities. The newborn had a dosed discharge of urine and meconium, she did not need bladder catheterization and enemas. Examination of the surgical site revealed a well-formed scar with no signs of CSF leak and no pseudomeningocele formation. Spontaneous, symmetrical movements were noted in all limbs, including movements of the hips, knees, and ankles. Magnetic resonance imaging revealed moderate ventriculomegaly, reversal of the hindbrain hernia, and no signs of spinal cord fixation to the dura mater prosthesis. By the 1st year of life, the child walked independently and did not need assistance with walking, as well as additional neurosurgical interventions that are required to correct hydrocephalus, including correction of urinary and stool diversion disorders, orthopedic deformities. Fetoscopic surgery is a promising alternative to open prenatal treatment of MMC, it significantly reduces the risks for the pregnant woman and the fetus, but is a technically difficult operation.

Congenital Central Nervous System and Associated Systemic Anomalies in Foetal and Perinatal Autopsy- A Retrospective Study

Introduction: Congenital malformations in India accounts for 10-15% of perinatal deaths. Congenital Heart Defects (CHD) is most common congenital anomaly followed by Central Nervous System (CNS) anomalies, among which Neural Tube Defects (NTD) are most common. Folic acid deficiency can lead to NTDs and can be corrected by its supplementation in subsequent pregnancies, the authors analysed neurological anomalies in foetal and perinatal autopsies to ascertain distribution of various congenital neurological malformations and to classify those according to WHO classification system and their association with other systemic malformation. Aim: To study CNS anomalies in foetal autopsies. Materials and Methods: The present retrospective study conducted in Department of Pathology, Karnataka Institute of Medical Sciences, Hubli, Karnataka, India from July 2015 to June 2020 analysed 500 foetal autopsies retrospectively. Data regarding maternal age, parity, history of previous abortion, foetal birth weight, gestational age, gender and CNS and other systemic anomalies detected at autopsy were included from departmental autopsy database. Data was presented as frequencies and percentages using Microsoft Excel 2017. Results: In the study, 500 foetal autopsies were analysed, out of which 70 (14%) foetuses had CNS anomalies were included in study. Among 70 foetuses with CNS anomalies, anencephaly was most common 25 (35%) followed by meningomyelocele 19 (27%), Arnold chiari type 2 malformation 8 (11.4%), congenital hydrocephalus and encephalocele 5 (7%). Multiple neurological anomalies were observed in various combinations. Neurological with musculoskeletal anomalies were also observed in the present study. Conclusion: Foetal autopsy plays an important role in arriving at final diagnosis and detecting cause of death. Findings of autopsy are of practical significance to clinicians in the form of estimating risk of recurrence and genetic counselling.

Dynamic CSF MRI Findings in the Diagnosis and Follow-up of Arnold Chiari Type 1

Aim:&#x0D; Chiari malformation is a spectrum of diseases that includes different subtypes, has a wide clinical presentation, and therefore has different follow-up and treatment methods. We aimed to compare clinical scoring systems and cerebrospinal fluid (CSF) dynamics obtained by magnetic resonance imaging (MRI) in Arnold Chiari Malformation Type-1 (CM-1) patients in the preoperative and postoperative periods.&#x0D; Material and Method:&#x0D; Twenty-one patients with a diagnosis of CM-1 who underwent CSF MRI between January 2016 and June 2020 were included. In addition, 21 healthy volunteers were evaluated. All the patients had undergone decompression surgery due to CM-1 related symptoms. The relationship between preoperative MRI findings and clinical findings of 21 patients was evaluated. Changes in clinical and imaging findings of 21 patients who underwent surgery were compared. &#x0D; Results:&#x0D; Measurements were made just below the level of the cerebellar tonsil. CM-1 patients had higher preoperative peak positive velocity anteriorly (PPV: 6.26±2.72 vs 3.89±1.74 cm/s; p=0.001) and peak negative velocity anteriorly (PNV: -7.45±3.36 vs -3.61±2.65 cm/s; p=0.001) than healthy volunteers. Preoperative net flow in the posterior subarachnoid space of CM-1 patients was lower than in healthy volunteers (p=0.017).&#x0D; Aliasing of the reverse flow was seen in 13(62%) patients.&#x0D; PPV and PNV were decreased after surgery (PPV: 6.26±2.72 vs 4.73±1.94 cm/s, p=0.017; PNV: -7.45±3.36 vs -4.97±1.97, p=0.005). Net Flow was increased posteriorly (0.32 vs 2.21 ml/min; p=0.053).&#x0D; Postoperative response was inversely proportional to the degree of tonsillar herniation. As the Asgari score of the cases increases, the Peak Positive and Negative Velocity Anterior values also increase with a statistically significant correlation (p=0.003; p=0,032 respectively)&#x0D; Conclusions:&#x0D; Our findings suggest that the determination of flow dynamics and morphology using MR imaging correlates with clinical findings and may be a useful tool in determining the need and timing of surgery.

Neuropathic Arthropathy of The Hand Joints in Patient with Arnold-Chiari Malformation Type I And Syringomyelia Caused by Cerebellopontine Angle Arachnoid Cyst: A Case Report

Neuropathic arthropathy (NA), is a progressive degenerative arthropathy associated with an underlying central or peripheral neurologic disorder. Neuropathic arthropathy has been observed in a variety of conditions including syringomyelia, diabetes mellitus and peripheral nerve disorders. The shoulder and elbow joints are the most frequently affected while the hand joints are quite rarely seen in the cases of NA caused by syringomyelia. In this article, we report the case of neuropathic arthropathy in a patient with Arnold-Chiari malformation type I and syringomyelia probably caused by a cerebellopontine angle (CPA) arachnoid cyst. Case : A 39-years old man came with slight weakness in both upper and lower extremities and spastic-related deformities in both hands accompanied with a history of headache. Cranial nerves examinations showed rotatoar nystagmus. Plain radiograph examinations of both hands demonstrated destructive osteoarthritic changes and multiple ossified intraarticular bodies consistent with intra-articular osteophytes. He was investigated with magnetic resonance imaging (MRI) of the brain and the cervicothoracic spine and they revealed arachnoid cyst at cerebelopontine cisterna causing the cerebellar tonsils downward displacement across foramen magnum (Arnold-Chiari type I malformation) with communican hydrochepalus and a syringomyelic cavity extending from C2 to Th5 vertebral levels. Bed rest and lowering-intracaranial pressure therapy with acetazolamide was given to the patient. Conclusion: Development of neuropathic atrophy following the diagnosis and surgery of syringomyelia should be considered especially in destructive cases without evident pain complaint and the underlying neurological problem should be surmounted.

Spontaneous Pneumocephalus with Meningomyelocele: A Case Report

Spontaneous pneumocephalus is one of the complications of open myelomeningocele with or without clinical signs, reported very rarely in the literature. Pneumocephalus It can associated with several neurosurgical procedures, lumbar puncture, or cranial trauma; But very rarely, the open neural tube defects may lead to spontaneous pneumocephalus. The radiographic appearance of intraspinal air on CT was not described previously. The following report presents a case of spontaneous appearance of internal pneumocephalus in a newborn infant with open myelomeningocele which environmental air could gain access to the cerebrum associated with bi-ventricular hydrocephalus and an Arnold-Chiari type II malformation, revealed on CT images. Spontaneous pneumocephalus should be kept in mind as a rare complication of open meningomyelocele. Any patient with open meningomyelocele should be early evaluated for pneumocephalus with a cranial imaging.

First occurrence of Arnold Chiari type II malformation and associated abnormalities in a Gir calf produced in vitro from Brazil - case report

ABSTRACT This study characterized the clinical, radiological, ultrasound, and necroscopic findings of a case of Arnold-Chiari type II malformation in a Gir breed calf from Brazil. The animal was hospitalized at sixty days of age, in permanent sternal recumbency, cutaneous appendix at the 4th lumbar vertebra and kyphoscoliosis of the caudal and lumbosacral thoracic spine. Radiographic examination of the spine and skull revealed spina bifida and suspected occipital hypoplasia. Upon examination of myelography with an injection of lumbar and atlantooccipital contrast, it was possible to visualize the meningocele at the 4th lumbar vertebra region and findings at the rhombencephalon level of increased regional pressure with failure to fill the contrast in the posterior fossa, in the presence of clear demarcation of the circumvolutions of the cerebral cortex and the subarachnoid space of the cervical spinal cord. Ultrasonographic examination of the cerebellum showed an insinuation of the cerebellar worm through the foramen magnum. The animal did not show changes in complete blood count, biochemical series, and cerebrospinal fluid and was negative for Pestivirus. There was a worsening of the clinical conditions and the animal died. This malformation of unknown etiology must be studied as a differential diagnosis of the nervous system disorders.