Choroid plexus hyperplasia

Choroid plexus hyperplasia

Choroid plexus hyperplasia (CPH), also known as villous hypertrophy of the choroid plexus, is a rare benign condition that is characterized by bilateral enlargement of the entire choroid plexus in lateral ventricles without any discrete masses. This can result in overproduction of CSF and communicating hydrocephalus.

Despite the current knowledge about hydrocephalus, we remain without a complete understanding of the pathophysiology of this condition. glymphatic system (GS) could be more important than the conventional concept of reabsorption of CSF in the arachnoid villi, therefore GS could be a new key point, which will guide future investigations 1).

Histology shows an increased number of normal-sized cells.

This is best diagnosed by MRI which demonstrates a diffuse enlargement and homogeneous enhancement of choroid plexuses in a patient with communicating hydrocephalus 2).

It is a rare condition that may necessitate unusual treatment paradigms.

Although some authors recommend choroid plexus excision or coagulation, ventriculoatrial shunt insertion is a simple and effective treatment modality in cases of diffuse villous hyperplasia of the choroid plexus 3).

It can be seen in trisomy 9p where coexisting congenital heart disease additionally may complicate the therapeutic approach 4).

At 20 months of age, a Caucasian girl with trisomy 9 and a family history of an older brother and twin sister having the same syndrome displayed signs of congenital hydrocephalus due to increasing head circumferenceMagnetic resonance imaging revealed enlarged lateral ventricles and a prominent choroid plexus and the girl was treated with a ventriculoperitoneal shunt, which 2 days later had to be replaced with a ventriculoatrial shunt as cerebrospinal fluid production greatly exceeded the ability of the patient’s abdominal absorptive capability. At 16 years of age, the patient was diagnosed with cardiomyopathy and diminished ejection fraction. Some months later, she was admitted to the neurosurgical ward showing signs of shunt dysfunction due to a colloid cyst in the third ventricle. Cystic drainage through endoscopic puncture only helped temporarily. Revision of the shunt system showed occlusion of the ventricular drainage, and replacement was merely temporary alleviating. Intracranial pressure was significantly increased at around 30 mmHg, prompting externalization of the drain, and measurements revealed high cerebrospinal fluid production of 60-100 ml liquor per hour. Thus, endoscopic choroid plexus coagulation was performed bilaterally leading to an immediate decrease of daily cerebrospinal fluid formation to 20-30 ml liquor per hour, and these values were stabilized by pharmaceutical treatment with acetazolamide 100 mg/kg/day and furosemide 1 mg/kg/day. Subsequently, a ventriculoperitoneal shunt was placed. Follow-up after 1 and 2 months displayed no signs of hydrocephalus or ascites.

High cerebrospinal fluid volume load and coexisting heart disease in children with trisomy 9p may call for endoscopic choroid plexus coagulation and pharmacological therapy to diminish the daily cerebrospinal fluid production to volumes that allow proper ventriculoperitoneal shunting 5).


A 1-year-old patient was diagnosed with communicating hydrocephalus; ventricle peritoneal shunt (VPS) is installed and ascites developed. VPS is exposed, yielding volumes of 1000-1200ml/day CSF per day. MRI is performed showing generalized choroidal plexus hyperplasia. Bilateral endoscopic coagulation of thechoroid plexus was performed in 2 stages (CPC) however the high rate of CSF production persisted, needing a bilateral plexectomy through septostomy, which finally decreased the CSF outflow.

New knowledge about CSF physiology will help to propose better treatment depending on the cause of the hydrocephalus. The GS is becoming an additional reason to better study and develop new therapies focused on the modulation of alternative CSF reabsorption. 6).


In these patients, intractable ascites can occur after a ventriculoperitoneal (VP) shunting operation. However, shunt-related hydrocele is a rare complication of VP shunting. Previous reports have indicated catheter-tip migration to the scrotum as a cause of hydrocele. Here, we present the first documented case of choroid plexus hyperplasia that led to intractable ascites after shunting and a resulting hydrocele without catheter-tip migration into the scrotum 7).


1) , 6)

Paez-Nova M, Andaur K, Campos G, Garcia-Ballestas E, Moscote-Salazar LR, Koller O, Valenzuela S. Bilateral hyperplasia of choroid plexus with severe CSF production: a case report and review of the glymphatic system. Childs Nerv Syst. 2021 Nov;37(11):3521-3529. doi: 10.1007/s00381-021-05325-2. Epub 2021 Aug 19. PMID: 34410450.
3)

Iplikcioglu AC, Bek S, Gökduman CA, Bikmaz K, Cosar M. Diffuse villous hyperplasia of choroid plexus. Acta Neurochir (Wien). 2006 Jun;148(6):691-4; discussion 694. doi: 10.1007/s00701-006-0753-1. Epub 2006 Mar 8. PMID: 16523225.
4) , 5)

Henningsen MB, Gulisano HA, Bjarkam CR. Congenital hydrocephalus in a trisomy 9p gained child: a case report. J Med Case Rep. 2022 May 27;16(1):206. doi: 10.1186/s13256-022-03424-5. PMID: 35619116.
7)

Hori YS, Nagakita K, Ebisudani Y, Aoi M, Shinno Y, Fukuhara T. Choroid Plexus Hyperplasia with Intractable Ascites and a Resulting Communicating Hydrocele following Shunt Operation for Hydrocephalus. Pediatr Neurosurg. 2018;53(6):407-412. doi: 10.1159/000492333. Epub 2018 Aug 29. PMID: 30157489.

Pineal region intravascular papillary endothelial hyperplasia

Pineal region intravascular papillary endothelial hyperplasia

Intracranial intravascular papillary endothelial hyperplasia (IPEH), also referred to as Masson’s tumor, is a condition that rarely occurs in the nervous system. IPEH most frequently occurs extracranially in the skin of the face, skull, neck, and trunk and can easily be mistaken clinically, radiologically, and histologically for angiosarcoma, organizing hematoma, or other vascular malformations. IPEH accounts for roughly 2% of all vascular tumors and is extremely rare intracranially, with only 23 reported cases compared with more than 300 cases of IPEH occurring in the skin and subcutaneous tissue.

Charalambouset al. reported the case of a patient with an IPEH in the pineal region who underwent complex resection and experienced reversal of neurological symptoms 1).

There has been only one previous report of intravascular papillary endothelial hyperplasia occurrence in the pineal region. Retzlaff et al. from Milwaukee reported a second case and a review of the literature 2).

References

1)

Charalambous LT, Penumaka A, Komisarow JM, Hemmerich AC, Cummings TJ, Codd PJ, Friedman AH. Masson’s tumor of the pineal region: case report. J Neurosurg. 2018 Jun;128(6):1725-1730. doi: 10.3171/2017.2.JNS162350. Epub 2017 Aug 4. PubMed PMID: 28777021.
2)

Retzlaff AA, Arispe K, Cochran EJ, Zwagerman NT. Intravascular Papillary Endothelial Hyperplasia of the Pineal Region: A Case Report and Review of the Literature. World Neurosurg. 2019 Sep 13. pii: S1878-8750(19)32452-0. doi: 10.1016/j.wneu.2019.09.022. [Epub ahead of print] PubMed PMID: 31525486.

Spinal intravascular papillary endothelial hyperplasia

Spinal intravascular papillary endothelial hyperplasia

In the literature, only nine cases of intravascular papillary endothelial hyperplasia involving the vertebral canal with spinal cord compression has been reported 1).

This rare pathology should be in the differential diagnosiswhen spinal cord compressive myelopathy is encountered 2)

This rare benign vascular lesion may be clinically and histopathologically mistaken for an angiosarcoma. Because the intravascular papillary endothelial hyperplasia can be cured by complete surgical resection, it is important to distinguish between these two lesions to avoid inappropriate aggressive treatment. 3).

Case reports

Oktar et al from Izmir presented a 37-year-old man with thoracic location mimicking schwannoma 4).

A 32-year-old man presented with paraplegia secondary to extradural compression at the T4-5 level 5)


A 17-year-old boy was admitted with pain, numbness, paresis of the left lower extremity, and bladder dysfunction of approximately 1 month’s duration. Computed tomography and magnetic resonance imaging of the spine revealed a tumor within the spinal canal at the T12-L1 level.

The patient underwent a T12-L1 laminectomy. An epidural red nodular tumor was visualized and totally resected. The findings of the pathological examination were compatible with intravascular papillary endothelial hyperplasia. At follow-up examination 1 month after the operation, the patient had complete resolution of the pain, and the motor deficit and bladder dysfunction had improved significantly 6).

References

1) , 4)

Oktar N, M Ozer H, Demirtas E. Spinal intravascular papillary endothelial hyperplasia. Case report and review of the literature. Br J Neurosurg. 2019 Apr 9:1-3. doi: 10.1080/02688697.2019.1597832. [Epub ahead of print] PubMed PMID: 30964346.
2) , 5)

Behera BR, Panda RN, Mishra S, Dhir MK. Masson Hemangioma-An Unusual Cause of Thoracic Compressive Myelopathy. World Neurosurg. 2017 Feb;98:876.e9-876.e13. doi: 10.1016/j.wneu.201
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