Ventriculoperitoneal shunt disconnection

Ventriculoperitoneal shunt disconnection

Mechanical shunt failure from shunt disconnection or fracture is a significant cause of shunt failure 1).

Shunt catheter disconnection has been well described in the literature as a cause of shunt malfunction.

The distal component among the valve and the peritoneal catheter is the most probable site of disconnection 2).

Ventriculoperitoneal shunt disconnection risk factors.

Suspect with Undershunting.

see Shunt evaluation.

Ventriculoperitoneal shunt disconnection prevention.

Shunt catheters that migrate peritoneally bring the possibility of visceral injury, predominantly perforation of the bowel. These disconnected or fractured shunts can be revised by substituting or reconnecting the components, or by replacing the whole shunt system. In the modern era, the laparoscopic retrieval of migrated shunt catheters can be done safely, either as an emergency or an elective process 3).

A 5-year-old boy with a right-sided ventriculoperitoneal shunt presented with a 3-month history of progressively enlarging subperiosteal fluid collection in the scalp, which started in the right parietal region and had spread and extended across the midline to occupy both parietal regions. There were no changes in symptoms or signs from those observed 3 months previously. A CT scan confirmed the collection of fluid under the scalp over both parietal regions. The peritoneal catheter was found to be disconnected from the distal end of the functioning valve, which drained cerebrospinal fluid into the subperiosteal space. Distention of the parietal subperiosteal space led to stretching and tearing of the emissary veins. This resulted in the formation of a hydrohematocele. The spread of fluid to the opposite parietal region may be due to a disorganized and loose attachment of the periosteum to the widely separated sagittal suture 4).


An 8-year-old boy with a right VP shunt was referred because of progressive loss of consciousness in the morning. A CT scan of the head established moderate hydrocephalus. A shunt series presented a disconnection of the distal tube of the shunt as the distal part was free in the abdominal cavity. The patient experienced a complete shunt revision. The abdominal incision was revived and the tube removed from the abdominal cavity gently. The patient was discharged 72 h later 5).


1)

Erol FS, Ozturk S, Akgun B, Kaplan M. Ventriculoperitoneal shunt malfunction caused by fractures and disconnections over 10 years of follow-up. Childs Nerv Syst. 2017 Mar;33(3):475-481. doi: 10.1007/s00381-017-3342-0. Epub 2017 Jan 17. PMID: 28097382.
2)

Ghritlaharey RK, Budhwani KS, Shrivastava DK, Gupta G, Kushwaha AS, et al. (2007) Trans-anal protrusion of ventriculo-peritoneal shunt catheter with silent bowel perforation: report of ten cases in children. Pediatr Surg Int 23(6): 575-580.
3)

Vinchon M, Baroncini M, Laurent T, Patrick D (2006) Bowel perforation caused by peritoneal shunts catheters: diagnosis and treatment. Neurosurgery 58(1): 76-82.
4)

Choudhury AR. Cephalhydrohematocele due to catheter valve disconnection following ventriculoperitoneal shunting. Childs Nerv Syst. 1988 Dec;4(6):376-7. PubMed PMID: 3245948.
5)

Haddadi K, Qazvini HRG, Sahebi M (2017) Ventriculoperitoneal Shunt Disconnection Associated with Loss of Consciousness in a Child Patient: A Case Report and Review of Intra-Abdominal Complications of Vp Shunts. J Neurol Stroke 7(3): 00237. DOI: 10.15406/jnsk.2017.07.00237

Everolimus for subependymal giant cell astrocytoma

Everolimus for subependymal giant cell astrocytoma

As a result of a trial, the US Food and Drug Administration (FDA) approved everolimus for patients with subependymal giant cell astrocytoma (SEGA) associated with tuberous sclerosis complex who are not candidates for curative surgical resection.

Patients ≥ 3 years of age with increasing size of SEGA lesions have had a sustained reduction of SEGA volume on everolimus 1).

A retrospective study included TSC patients being treated with oral everolimus for subependymal giant cell astrocytomas (SEGAs) and angiomyolipomas (AMLs). We recorded the changes in facial angiofibroma. Changes in the Angiofibroma Grading Scale (AGS) indicators were recorded according to erythema, average lesion size, lesion density, and percent involvement on the forehead, nose, cheeks, and chin. The scores were recorded before and after the administration of oral everolimus.

Twenty-one patients being treated with oral everolimus were enrolled in this study. The mean age was 20.5 years (range 11-44 years, 4 males, and 17 females). The mean dose of oral everolimus was 3.6 mg/day. Clinically meaningful and statistically significant improvement was observed in erythema (p = 0.001), average lesion size (p < 0.001), lesion density (p < 0.001), and percent involvement (p < 0.001). Changes in the AGS findings were statistically significant on the forehead (p = 0.001), nose (p < 0.001) cheeks (p < 0.001), and chin (p = 0.004).

Everolimus shows evident improvement and is approved for TSC-associated SEGAs and AMLs. The current study demonstrated the efficacy of oral everolimus in reducing facial angiofibromas, showing the parallel benefits of the treatment protocol for TSC 2).

Case reports

A 21-year female patient with large bilateral angiomyolipoma (AML) in both kidneys with the longest diameter more than 12.3 cm and subependymal giant cell astrocytoma (SEGA). Treatment with everolimus (EVE) was initiated at a dose of 10.0 mg/day and continued during the following 3 years. Magnetic resonance imaging (MRI) was performed before treatment with everolimus was initiated, and consequently at 12 and 36 months for follow-up of the efficacy of the treatment. After 3 years, the total size of the largest AML decreased by ~24.0% in the longest diameter. A reduction in the total size of SEGA was also observed. The most common adverse effect of treatment was stomatitis grades 3 to 4 and one febrile episodes associated with a skin rash that required a reduced dose of EVE. In conclusion, the everolimus treatment improved even such a large renal AML and the effect persisted during the long-term administration with a small number of adverse effects. A positive effect was observed on the brain tumor as well 3).


A case of a woman with TSC and Multifocal micronodular pneumocyte hyperplasia (MMPH) who received everolimus, for the treatment of a subependymal giant cell astrocytoma (SEGA). After 3 months of therapy, a remarkable decrease in density of all pulmonary MMPH lesions was observed, without any change in size. This shows that everolimus is active on MMPH similarly to its effects on SEGA, renal angiomyolipomas, and pulmonary lymphangioleiomyomatosis in TSC, and suggests that the dysregulated activation of mTOR which characterizes TSC also plays a role in the pathogenesis of MMPH 4).


The aim of a study was to evaluate the efficacy of oral everolimus for TSC-associated angiofibromas.

This retrospective study included TSC patients being treated with oral everolimus for subependymal giant cell astrocytomas (SEGAs) and angiomyolipomas (AMLs). We recorded the changes in facial angiofibromas. Changes in the Angiofibroma Grading Scale (AGS) indicators were recorded according to erythema, average lesion size, lesion density, and percent involvement on the forehead, nose, cheeks, and chin. The scores were recorded before and after the administration of oral everolimus.

Twenty-one patients being treated with oral everolimus were enrolled in this study. The mean age was 20.5 years (range 11-44 years, 4 males, and 17 females). The mean dose of oral everolimus was 3.6 mg/day. Clinically meaningful and statistically significant improvement was observed in erythema (p = 0.001), average lesion size (p < 0.001), lesion density (p < 0.001), and percent involvement (p < 0.001). Changes in the AGS findings were statistically significant on the forehead (p = 0.001), nose (p < 0.001) cheeks (p < 0.001), and chin (p = 0.004).

Everolimus shows evident improvement and is approved for TSC-associated SEGAs and AMLs. The study demonstrated the efficacy of oral everolimus in reducing facial angiofibromas, showing the parallel benefits of the treatment protocol for TSC 5).


1)

Franz DN, Agricola K, Mays M, Tudor C, Care MM, Holland-Bouley K, Berkowitz N, Miao S, Peyrard S, Krueger DA. Everolimus for subependymal giant cell astrocytoma: 5-year final analysis. Ann Neurol. 2015 Dec;78(6):929-38. doi: 10.1002/ana.24523. Epub 2015 Nov 9. PMID: 26381530; PMCID: PMC5063160.
2)

Wei CC, Hsiao YP, Gau SY, Wu YT, Wu CT, Wu MH, Tsai JD. The Efficacy of Everolimus for Facial Angiofibromas in Tuberous Sclerosis Complex Patients Treated for Renal Angiomyolipoma/Subependymal Giant Cell Astrocytoma. Dermatology. 2020 Oct 8:1-6. doi: 10.1159/000510222. Epub ahead of print. PMID: 33032292.
3)

Rambabova Bushljetik I, Lazareska M, Barbov I, Stankov O, Filipce V, Spasovski G. Bilateral Renal Angiomyolipomas and Subependymal Giant Cell Astrocytoma Associated with Tuberous Sclerosis Complex: a Case Report and Review of The Literature. Balkan J Med Genet. 2021 Mar 23;23(2):93-98. doi: 10.2478/bjmg-2020-0017. PMID: 33816078; PMCID: PMC8009567.
4)

Daccord C, Nicolas A, Demicheli R, Chehade H, Hottinger AF, Beigelman C, Lazor R. Effect of everolimus on multifocal micronodular pneumocyte hyperplasia in tuberous sclerosis complex. Respir Med Case Rep. 2020 Nov 25;31:101310. doi: 10.1016/j.rmcr.2020.101310. PMID: 33312857; PMCID: PMC7720070.
5)

Wei CC, Hsiao YP, Gau SY, Wu YT, Wu CT, Wu MH, Tsai JD. The Efficacy of Everolimus for Facial Angiofibromas in Tuberous Sclerosis Complex Patients Treated for Renal Angiomyolipoma/Subependymal Giant Cell Astrocytoma. Dermatology. 2020 Oct 8:1-6. doi: 10.1159/000510222. Epub ahead of print. PMID: 33032292.

Chiari type 1 deformity classification

Chiari type 1 deformity classification

Nishikawa et al. classified Chiari malformation type I (CM-I) according to the mechanism of ptosis of the brain stem and cerebellum, based on a morphometric study of the posterior cranial fossa (PCF) and craniovertebral junction (CVJ). Surgery was performed to manage the mechanism of the hindbrain ptosis. They calculated the volume of the PCF (VPCF) and the area surrounding the foramen magnum (VSFM) and measured the axial length of the enchondral parts of the occipital bone (occipital bone size) and the hindbrain. According to these measures, they classified CM-I into type A (normal VPCF, normal VSFM, and normal occipital bone size), type B (normal VPCF, small VSFM, and small occipital bone size), and type C (small VPCF, small VSFM, and small occipital bone size). Foramen magnum decompression (FMD) (280 cases) was performed on CM-I types A and B. Expansive suboccipital cranioplasty (ESCP) was performed on CM-I type C. Posterior craniocervical fixation (CCF) was performed in cases with CVJ instability. Lysis of the adhesion and/or sectioning of the filum terminale was performed on cases with tethered cord syndrome. Both ESCP and FMD had a high rate of improvement of neurological symptoms (87%) and recovery rate. There was only a small number of complications. CCF had a high rate of improvement of neurological symptoms (88%) and joint stabilization. In the management of Chiari malformation, appropriate surgical methods that address ptosis of the hindbrain should be chosen. Each surgical approach resulted in a good improvement of neurological symptoms 1).


Valentini et al. suggested defining an association of Chiari type 1 deformity plus untreated sagittal synostosis, a new subtype of complex CM1. For the high percentage of complications and multiple procedures needed to solve the CM1, they advise identifying by 3D-CT scan these children before performing craniovertebral decompression (CVD). They suggest also that if left untreated, sagittal synostosis may lead to the delayed occurrence of a challenging subset of CM1 2).


Chiari malformation Type 1.5 (CM 1.5) was defined as the association of Chiari malformation Type I (CM I) and brainstem herniation.

Although CM 1.5 patients presented with brainstem herniation and more severe tonsillar herniation, other clinical and imaging features and surgical outcomes were similar to CM I patients. Liu et al. think CM 1.5 is just a subtype of CM I, rather than a unique type of Chiari malformations 3).


Taylor et al. identify two subtypes, crowded and spacious, that can be distinguished by MRI appearance without volumetric analysis. Earlier age at surgery and the presence of syringomyelia are more common in the crowded subtype. The presence of the spacious subtype suggests that crowdedness alone cannot explain the pathogenesis of Chiari I malformation in many patients, supporting the need for further investigation 4).

see Pediatric Chiari type 1 deformity.

see Chiari type 1 deformity and syringomyelia.


1)

Nishikawa M, Bolognese PA, Kula RW, Ikuno H, Takami T, Ohata K. Surgical Management of Chiari Malformations: Preliminary Results of Surgery According to the Mechanisms of Ptosis of the Brain Stem and Cerebellum. J Neurol Surg B Skull Base. 2021 Apr;82(2):264-272. doi: 10.1055/s-0039-1697977. Epub 2019 Sep 30. PMID: 33816049; PMCID: PMC8009696.
2)

Valentini LG, Saletti V, Erbetta A, Chiapparini L, Furlanetto M. Chiari 1 malformation and untreated sagittal synostosis: a new subset of complex Chiari? Childs Nerv Syst. 2019 Jul 21. doi: 10.1007/s00381-019-04283-0. [Epub ahead of print] PubMed PMID: 31327038.
3)

Liu W, Wu H, Aikebaier Y, Wulabieke M, Paerhati R, Yang X. No significant difference between Chiari malformation type 1.5 and type I. Clin Neurol Neurosurg. 2017 Mar 30;157:34-39. doi: 10.1016/j.clineuro.2017.03.024. [Epub ahead of print] PubMed PMID: 28384597.
4)

Taylor DG, Mastorakos P, Jane JA Jr, Oldfield EH. Two distinct populations of Chiari I malformation based on presence or absence of posterior fossa crowdedness on magnetic resonance imaging. J Neurosurg. 2017 Jun;126(6):1934-1940. doi: 10.3171/2016.6.JNS152998. Epub 2016 Sep 2. PubMed PMID: 27588590.
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