Can GH Therapy Worsen a Clinically Silent Chiari Malformation? A Case Report and Systematic Literature Review.

BACKGROUND: The link between the effects of recombinant human growth hormone (rhGH) therapy in patients with growth hormone deficiency (GHD) and Chiari malformation type I (CM-1) is controversial. SUMMARY: We report the case of a patient with an unusual association of GHD due to ectopic posterior pituitary and CM-1. Our patient developed a headache and worsening of CM-1 after the initiation of rhGH therapy. Following an atlo-occipital decompression surgery, the patient was able to resume therapy with a marked growth improvement. Based on this observation, we provide a systematic review of the current literature about these two pathologies. KEY MESSAGES: A careful follow-up of all patients with CM-1 treated with GH is mandatory, paying particular attention to the appearance of any neurological signs and symptoms.

Resolution of type I Chiari malformation and associated syringomyelia following intrathecal chemotherapy: case report.

Type I Chiari malformation (CM-I) consists of downward herniation of the cerebellar tonsils below the foramen magnum and often requires surgical decompression if symptomatic. Spontaneous resolution of CM-I is rare. We present a case of resolved CM-I without surgery in a 6-year-old boy with B-cell lymphoma who was diagnosed with CM-I during lymphoma staging. Cerebrospinal fluid cytology and brain MRI revealed negative CNS involvement but showed CM-I with tonsillar ectopia 19 mm below the foramen magnum. The patient underwent induction chemotherapy including 5 doses of intrathecal chemotherapy. Follow-up MRI demonstrated marked regression of CM-I to less than 6 mm in 3 months, and complete resolution of CM-I was observed in 2 years. To the best of our knowledge, this is the first case of resolved CM-I and syringomyelia following chemotherapy. In this case report, the authors summarize all of the clinical characteristics, the radiological appearance, and the potential causes of resolution based on a review of the literature and propose the mechanisms through which intrathecal chemotherapy contributed to the CM-I and syringomyelia resolution in the present case.

Effects of intraoperative liposomal bupivacaine on pain control and opioid use after pediatric Chiari I malformation surgery: an initial experience.

OBJECTIVE: Pediatric Chiari I malformation decompression is a common neurosurgical procedure. Liposomal bupivacaine (LB) is a novel formulation that can have an impact on postoperative recovery for particular procedures, but its potential role in pediatric neurosurgery is largely unexplored. The authors sought to describe and assess their initial experience with LB in pediatric Chiari I malformation decompression to better define its potential role as an analgesic agent in a procedure for which the postoperative course is often remarkably painful. METHODS: A retrospective review of all pediatric Chiari procedures performed at the authors' institution between 2018 and 2020 was conducted. Patients were divided into those who were treated with a single intraoperative dose of LB (LB group) and those who were not (control group). Comparisons of total opioid use and pain control were made using chi-square and Wilcoxon rank-sum tests. RESULTS: A total of 18 patients were identified, 9 (50%) in the LB group and 9 (50%) in the control group. Overall, there were 13 (72%) female and 5 (28%) male patients with a mean age of 15.9 years. No surgical complications were observed over a mean length of stay of 2.7 days. Within the first 24 hours after surgery, the LB group had significantly lower total opioid use than the control group (17.5 vs 47.9 morphine milligram equivalents, respectively; p = 0.03) as well as lower mean pain scores reported by patients using a 10-point visual analog scale (3.6 vs 5.5 for the LB vs control groups, p = 0.04). However, from the first 24 postoperative hours to discharge, total opioid use (p = 0.51) and mean pain scores (p = 0.09) were statistically comparable between the two groups. There were 2/9 (22%) LB patients versus 0/9 (0%) control patients who did not require opioid analgesia at any point during hospitalization. CONCLUSIONS: The use of a single intraoperative dose of LB in pediatric Chiari I malformation surgery appears to be safe and has the potential to reduce pain scores and opioid use when administered during the first 24 postoperative hours. From that time period to discharge, however, there may be no significant difference in total opioid use or pain scores.

Effects of growth hormone therapy in pediatric patients with growth hormone deficiency and Chiari I malformation: a retrospective study.

PURPOSE: The safety and efficacy of growth hormone replacement therapy (GHRT) on pediatric patients with growth hormone deficiency (GHD) and Chiari I malformation (CIM) are not well investigated within the current body of literature. With no clear indication of the effects of GHRT on CIM disease progression, we sought to determine the effect of GHRT on tonsillar herniation and progression of CIM symptomatology. METHODS: From a previously established database of 465 patients with radiologically confirmed CIM defined as > 5 mm of tonsillar descent on head magnetic resonance imaging (MRI), we identified 20 patients who also had GHD. Using the imaging analysis software package, ANALYZE, the degree of change in tonsillar herniation was documented between initial and final MRI measurements. The radiologic and clinical changes over time were examined via a proportional odds model, Student's t test, Mann-Whitney test, or a mixed model corresponding to the outcomes measured either on an ordinal scale or on a quantitative scale. RESULTS: Incidence of GHD in our CIM population was 4.3%. There was no significant effect of GHRT on the degree of tonsillar herniation in patients with GHD and CIM. No patient became symptomatic, developed syringomyelia, or required surgical intervention for CIM. CONCLUSION: Based on our findings with a larger sample size, along with recent reports, the incidence of patients with CIM and GHD we reported (0.86-5%) is likely more indicative of the actual incidence of GHD and CIM than the prior findings within the literature (9.1-20%). We also suggest that GHRT does not significantly affect CIM morphology or symptomatology. Therefore, neurosurgeons should have no hesitation clearing these patients for GHRT.

Pregabalin for the treatment of syringomyelia-associated neuropathic pain in dogs: A randomised, placebo-controlled, double-masked clinical trial.

Pregabalin is the first-line treatment for neuropathic pain (NeP) in humans. Dogs with Chiari-like malformation and syringomyelia (CM/SM) associated with NeP could benefit from pregabalin. The aim of this study was to evaluate the efficacy of pregabalin for NeP in dogs with CM/SM. Eight dogs with symptomatic CM/SM were included in a double-masked, randomised, crossover placebo-controlled clinical trial. All dogs received anti-inflammatory drugs as base-line treatment during placebo or pregabalin phase of 14±4 days each. Analgesic efficacy was assessed with a daily numerical rating scale (NRS) recorded by dog owners (0-10, 10=worst pain) and quantitative sensory testing at baseline, placebo and pregabalin phases. Blood samples were collected to report pregabalin exposure and to assess renal function. Daily NRS scores recorded by dog owners in the pregabalin group were lower than in the placebo group (P=0.006). Mechanical thresholds were higher with pregabalin compared to baseline or placebo (P=0.037, P<0.001). Cold latency at 15°C was prolonged on the neck and humeri with pregabalin compared to baseline (P<0.001 for both) or placebo (P=0.02, P=0.0001). Cold latency at 0°C was longer on pregabalin compared to baseline and placebo (P=0.001, P=0.004). There was no pregabalin accumulation between first and last dose. This study demonstrates the efficacy of pregabalin for the treatment of NeP due to CM/SM on daily pain scores recorded by dog owners. Pregabalin significantly reduced mechanical hyperalgesia, cold hyperalgesia (0°C) and allodynia (15°C) compared to placebo. Pregabalin was non-cumulative and well tolerated with occasional mild sedation.

A rare combination: congenital factor VII deficiency with Chiari malformation.

Congenital factor (VII) deficiency is a rare bleeding disorder. We present a patient with congenital FVII deficiency and congenital hydrocephalus who underwent a ventriculoperitoneal shunt operation and needed no prophylaxis after the procedure.

Comparison of gabapentin versus topiramate on clinically affected dogs with Chiari-like malformation and syringomyelia.

To date there is no evidence-based data for efficacious treatment of neuropathic pain in dogs with Chiari-like malformation (CM) and syringomyelia (SM). The objective of this prospective cross-over study was to compare the effect of gabapentin versus topiramate, as an add-on treatment to carprofen, on quality of life (QoL) of dogs experiencing signs of neuropathic pain due to CM/SM. A visual analogue scale (VAS) was used to assess the QoL: (1) on day 0; (2) after 1 week of carprofen only; (3) after 2 weeks on carprofen and gabapentin; and (4) after 2 weeks on carprofen and topiramate. No significant difference was observed between VAS after gabapentin or topiramate (P=0.91). However, an improvement in QoL was observed when gabapentin was compared with baseline (P=0.009), but not for topiramate. In conclusion, the addition of gabapentin was more effective in improving QoL than carprofen alone, but the study failed to identify that gabapentin was more efficacious than topiramate. Perhaps the more favourable side effect profile of the former makes it more suitable for the treatment of neuropathic pain associated with CM/SM but further placebo-controlled trials are required to assess the efficacy of these drugs.

Acquired Chiari malformation type I associated with a supratentorial fistulous arteriovenous malformation: a case report.

A case of acquired Chiari malformation type I with frontal fistulous arteriovenous malformation (AVM) is presented, and the pathophysiology is discussed. The tonsillar herniation and hydrocephalus both resolved after AVM was excised. This case provides some insight into the complex hemodynamic change exerted by the fistulous AVM and the mechanism of the development of acquired Chiari malformation type I.

Malformación de Arnold Chiari tipo I con siringomegalia y epilepsia analgesia neuroaxial en el trabajo de parto / No disponible

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Malformación de Arnold Chiari tipo I: presentación de un caso / Arnold-Chiari malformation Type I: presentation of a case

La malformación de Arnold Chiari es una rara enfermedad incapacitante que afecta al 0,5 por ciento de la población, siendo el 80 por ciento mujeres. La hidrocefalia resultante es causada por el exceso de líquido cefalo raquídeo en el sistema ventricular, o por una falta de equilibrio entre la formación y su absorción, lo que da lugar a un progresivo aumento de las cavidades ventriculares. Se presenta el caso de una paciente con malformación de Arnold Chiari tipo I, en la cual hay un descenso anormal en grado variable de la porción inferior del cerebelo, del bulbo y el cuarto ventrículo hacia el conducto raquídeo a través del agujero occipital. El objetivo de este trabajo es describir el cuadro clínico y compararlo con la literatura médica revisada, del caso en cuestión...(AU)

Malformación de Arnold Chiari tipo I: presentación de un caso / Arnold-Chiari malformation Type I: presentation of a case

La malformación de Arnold Chiari es una rara enfermedad incapacitante que afecta al 0,5 por ciento de la población, siendo el 80 por ciento mujeres. La hidrocefalia resultante es causada por el exceso de líquido cefalo raquídeo en el sistema ventricular, o por una falta de equilibrio entre la formación y su absorción, lo que da lugar a un progresivo aumento de las cavidades ventriculares. Se presenta el caso de una paciente con malformación de Arnold Chiari tipo I, en la cual hay un descenso anormal en grado variable de la porción inferior del cerebelo, del bulbo y el cuarto ventrículo hacia el conducto raquídeo a través del agujero occipital. El objetivo de este trabajo es describir el cuadro clínico y compararlo con la literatura médica revisada, del caso en cuestión...

Effects of birth advancement in Chiari malformation in a surgical myelomeningocele model in rabbits.

BACKGROUND/PURPOSE: In myelomeningocele (MMC), Chiari II malformation progresses during gestation because of the continuous loss of cerebrospinal fluid at the site of the defect. Our purpose was to assess the impact of birth advancement (BA) and prenatal corticosteroid treatment (PCT) on Chiari malformation in a surgical MMC model in rabbits. METHODS: A surgical MMC-like defect was created in 75 fetal rabbits. Animals were distributed into 4 groups depending on the treatment received: not treated and those undergoing BA + PCT, BA, or PCT. The degree of Chiari malformation in newborn rabbits was defined as the percentage of downward protrusion of the hindbrain between the end of the occipital bone and the beginning of the first vertebral arch. RESULTS: The degree of hindbrain herniation was 80% (8.15) in the not treated group, 36.8% (10.57) in BA + PCT, 41.8% (8.27) in BA, and 44.4% (8.32) in PCT. The BA + PCT, BA, and PCT groups showed less severe hindbrain herniation than not treated animals (mean decrease, 39.86%; SD, 10.57; P = .000). There were no significant differences between the BA + PCT, BA, and PCT groups (P = .311). CONCLUSIONS: Birth advancement and prenatal administration of corticosteroids decrease the severity of the hindbrain herniation component of Chiari II malformation in surgical MMC in fetal rabbits.

Resolution of syringomyelia and Chiari malformation after growth hormone therapy.

INTRODUCTION: The association between hypopituitarism, Chiari I malformation, and syringomyelia has been recently recognized. Most of the reported patients suffered perinatal injury or asphyxia. MATERIALS AND METHODS: We present the case of a premature child without identifiable perinatal injury, who was recognized to have growth hormone (GH) deficiency, Chiari I malformation, and syringohydromyelia. CONCLUSION: There was a resolution of syrinx and tonsillar herniation after GH replacement.

Resolution of Chiari I malformation following acetazolamide therapy.

A 25-year-old obese woman developed headaches and visual blurring. Examination showed papilledema and cranial MRI showed a large Chiari I malformation. Acetazolamide was initiated and a repeat cranial MRI surprisingly showed resolution of the Chiari I malformation. Chiari I malformation associated with papilledema has never been shown to resolve with acetazolamide therapy.

[Paget's disease of bone and basilar impression associated with an Arnold-Chiari type-1 malformation]. / Enfermedad ósea de Paget e impresión basilar asociadas a malformación de Arnold-Chiari tipo I.

The patient, a 78-year-old female with history of headache and progressive gait disturbance for almost one year, was admitted to our department because of dysphagia and dysphonia since three months before. Neurological examination revealed nystagmus, cerebellar ataxia, deafness, and vesical incontinence. No cerebral injuries were detected by computed tomography (CT) scan, although Paget's. Disease of Bone (PDB) was suggested, confirmed by biochemical and scintigraphic studies. The plain skull X-ray showed platybasia. As all the disarrangements were not explained by PDB complications alone, nuclear magnetic resonance imaging (MRI) was performed which demonstrated an Arnold-Chiari malformation (ACM) type I, with mild tonsillar herniation and anterior compression of the brainstem due to basilar impression, without syringomyelia. The association of PDB and ACM is a peculiarity seldom reported. The surgical approach was rejected, but the severity of symptoms and osteitis deformans biochemical activity needed a treatment; it was orientated to modify bone turnover using etidronate, a bisphosphonate, which induced clinical improvement and a decrease in serum alkaline phosphatase as well as in other bone resorption markers, without side effects. The good status and biochemical remission have been maintained a year later.