Acquired Chiari I Malformation and Syringomyelia after Valveless Lumboperitoneal Shunt in Infancy

Riffaud, Laurent; Moughty, Carolina; Henaux, Pierre-Louis; Haegelen, Claire; Morandi, Xavier

doi:10.1159/000121381

Cited by 26 publications

(17 citation statements)

References 39 publications

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“…6,13 Acquired CM-I has also been described in infants, particularly after lumbar puncture or placement of a lumboperitoneal shunt. 10 It should be noted that the first twin in the present study did undergo a lumbar puncture although only a small volume of cerebrospinal fluid was obtained for diagnostic purposes (< 1.5 ml); this . The initial MR imaging shows the cerebellar tonsils to be elongated and peg-shaped.…”

Section: Discussionmentioning

confidence: 77%

Spontaneous resolution of Chiari malformation Type I in monozygotic twins

Miller

Limbrick²,

M³

et al. 2008

PED

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The spontaneous resolution of isolated tonsillar ectopia in Chiari malformation Type I (CM-I) is a known and reported entity in 2 previous single study case reports. However, it has not been previously described in monozygotic twins. Two children, ~ 1 year of age with CM-I and presumed episodes of pallid syncope or breath-holding spells presented for neurosurgical evaluation. Although Chiari decompression was considered, the authors decided to proceed with conservative management with close follow-up due to the uncertain nature of these episodes. Approximately 4 years later, both children's symptoms had resolved. Repeated MR imaging examinations also showed spontaneous resolution of the malformation in both girls. These cases emphasize that when patients with CM-I present with atypical symptoms, spontaneous resolution or improvement is possible, which may influence the decision to pursue a trial of nonsurgical management. The possible pathophysiological mechanisms and genetic influences of CM-I are also briefly discussed.

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Section: Discussionmentioning

confidence: 77%

Spontaneous resolution of Chiari malformation Type I in monozygotic twins

Miller

Limbrick²,

M³

et al. 2008

PED

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“…A previous study reported a slight female predominance (female/male ratio 1.3:1.0) (20), although this is inconsistent with the findings in the current study (female/male ratio 1.00:1.52). CM-I is generally considered a congenital neurological condition, although in recent years its acquired form has been identified as a complication of CSF diversion procedures or chronic CSF leakage (21,22). CM-I is thought to be a multifactorial condition, although the pathogenesis is still undetermined.…”

Section: Discussionmentioning

confidence: 99%

Pediatric Chiari malformation type I: long‑term outcomes following small‑bone‑window posterior fossa decompression with autologous‑fascia duraplasty

Liu

Yang

et al. 2017

Exp Ther Med

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Abstract. Chiari malformation type I (CM-I) is a common hindbrain disorder that is associated with deformity and elongation of the cerebellar tonsils. Although CM-I occurs in both pediatric and adult patients, its prevalence, clinical features and management in the pediatric population are not well defined. The current study evaluated a consecutive case series of 92 children (38 females and 54 males) who were diagnosed with congenital CM-I. All patients underwent small-bone-window posterior fossa decompression with autologous-fascia duraplasty. Clinical and radiological features were analyzed and long-term follow-up data were recorded. Risk factors associated with clinical outcomes were investigated using comprehensive statistical methods. Out of the 92 children, 11 (12.0%) were asymptomatic. Associated ventricular dilation was observed in 24 children (26.1%) and concomitant syringomyelia was observed in 72 children (78.3%). A total of 44 children (47.8%) showed scoliosis on plain films. Follow-up data (mean duration, 88.6 months) were available for all patients. Syringomyelia was absent or markedly reduced in 56 patients (77.8%). Symptoms were alleviated in 66 patients, remained unchanged in 12 patients and progressed in 3 patients. Statistical analysis indicated that the cerebellar tonsillar descent (CTD) grade, basilar invagination and platybasia influenced the clinical outcome (P<0.05).In conclusion, early recognition and surgical treatment of CM-I in pediatric patients can lead to good outcomes. The current results suggested that small-bone-window posterior fossa decompression with autologous-fascia duraplasty was an effective safe treatment option with a low complication rate. High CTD grade, basilar invagination and platybasia were indicated to be predictors of poor clinical prognosis.

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“…Laurent Riffaud et al observed that the valveless LP shunt may expose the patient to the risk of symptomatic ACM and syringomyelia. They suggested LP shunt with an adjustable valve to prevent such complications (23). Roger Strachan et al observed that the catheter length and placement are important in reducing the risk of low pressure symptoms.…”

Section: Resultsmentioning

confidence: 99%

“…We used Chhabra system without valve and a catheter length of 7-10 cms was not associated with any permanent over drainage complication. Programmable shunt can also reduce over drainage complications (19,23).…”

Section: Resultsmentioning

confidence: 99%

Lumbar peritoneal shunt in idiopathic intra cranial hypertension

Yadav

Parihar²,

Agarwal³

et al. 2011

Turkish Neurosurgery

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AIm: Treatment options for idiopathic intracranial hypertension (IIH) are lumbar peritoneal shunt (LP), optic nerve fenestration, ventriculoperitoneal shunt and venous stenting. We report our experience of 24 cases of LP shunt. mAterIAl and methOds: All the patients had preoperative fundus examination, cerebrospinal fluid pressure estimation and examination, visual field charting, CT scan and MR venography. Postoperative fundus examination and visual field charting was done in all cases. Follow up ranged from 18 to 137 months. results: Preoperative papilledema, headache, decreased vision, optic atrophy and diplopia were seen in 24, 24, 19, 10 and 11 patients respectively. Shunt failure, CSF leak and temporary over drainage complications in the form of headache were seen in 2, 1 and 15 cases respectively. Vision improved in 10 out of 18 patients. Only one patient, out of 9 who had only perception of light and optic atrophy preoperatively, had improved vision while all patients with vision of finger counting or better without optic atrophy improved after shunt.COnClusIOn: LP shunt is safe and effective in IIH. Results in terms of improvement in vision were better in good pre operatively vision group. yÖntem ve GereÇ: Tüm hastalara ameliyat öncesinde göz dibi muayenesi, görme alanı, beyin omurilik sıvısı basıncının ölçümü ve analizi, bilgisayarlı tomografi ve manyetik rezonans görüntüleme venografisi yapılmıştır. Ameliyat sonrası tüm hastalara gözdibi muayenesi ve görme alanı değerlendirmesi yapıldı. Hastalar 18-137 ay arasında izlendi.BulGulAr: Ameliyat öncesi dönemde papilödemi, başağrısı, görmede azalma, optik atrofi ve çift görme 24,24,19,10,11 hastada sırası ile görülmüştür. Onsekiz hastadan 10'unda görme düzelmiş. Optik atrofisi olmayan 9 hastada görme daha iyi hale gelmiş, optik atrofisi olan bir hastada ise preoperatif dönemde olan ışık algılaması daha iyi hale gelmiş. sOnuÇ: İntrakraniyal basınç artışında lumboperitoneal şant güvenli ve etkili bir yöntemdir. Ameliyat öncesi dönemde görmesi iyi olan hastaların görmesindeki düzelme daha belirgin olmaktadır.

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Acquired Chiari I Malformation and Syringomyelia after Valveless Lumboperitoneal Shunt in Infancy

Cited by 26 publications

References 39 publications

Spontaneous resolution of Chiari malformation Type I in monozygotic twins

Spontaneous resolution of Chiari malformation Type I in monozygotic twins

Pediatric Chiari malformation type I: long‑term outcomes following small‑bone‑window posterior fossa decompression with autologous‑fascia duraplasty

Lumbar peritoneal shunt in idiopathic intra cranial hypertension

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