- Choroidal artery embolization in the management of cerebrospinal fluid overproduction: case report and review of the literature. [Case Reports]
- JNJ Neurosurg Pediatr 2019 03 22; :1-12
- Diffuse villous hyperplasia of the choroid plexus (DVHCP) is a rare cause of communicating hydrocephalus. DVHCP may be diagnosed radiographically and through histological evaluation. It may be associ…
Diffuse villous hyperplasia of the choroid plexus (DVHCP) is a rare cause of communicating hydrocephalus. DVHCP may be diagnosed radiographically and through histological evaluation. It may be associated with genetic abnormalities, particularly involving chromosome 9. Due to CSF overproduction, patients with DVHCP often fail management with shunting alone and may require adjuvant interventions. The authors present the case of a child with partial trisomy 9p and delayed diagnosis of hydrocephalus with radiographic evidence of DVHCP who was successfully managed with ventriculoperitoneal shunt (VPS) placement, adjuvant bilateral endoscopic choroid plexus coagulation (CPC), and the novel application of anterior choroidal artery embolization. In addition, a systematic MEDLINE search was conducted using the keywords "diffuse villous hyperplasia," "choroid plexus hypertrophy," and "idiopathic cerebrospinal fluid overproduction." Clinicopathological characteristics and outcomes of the present case were reviewed and compared to those in the literature.A 14-month-old girl with partial trisomy 9p presented with macrocephaly and radiographic evidence of communicating hydrocephalus and DVHCP. Ventriculoperitoneal shunting resulted in distal failure due to inadequate CSF absorption, and ventriculoatrial shunt (VAS) placement was not possible due to multiple cardiac anomalies. Daily CSF production was reduced via endoscopic third ventriculostomy and bilateral CPC, followed by distal choroidal artery embolization, enabling VPS re-internalization. The embolization was complicated by radiographic evidence of an iatrogenic cerebral infarct, but this was clinically occult. Thirty-two additional cases of communicating hydrocephalus due to DVHCP are reported in the literature: 27 pediatric, 3 adult, and 2 postmortem. Genetic abnormalities were noted in 14, with 7 (50%) involving chromosome 9. Twelve patients underwent plexectomy (9 bilateral, 2 unilateral, 1 partial), and 10 underwent CPC (4 bilateral, 3 unilateral, and 3 unspecified), with or without shunting. Eight patients were successfully managed with shunting alone (6 VASs, 2 VPSs), and none underwent arterial embolization.DVHCP is a rare cause of communicating hydrocephalus that may be associated with genetic abnormalities. A thorough review of the literature highlights diagnostic criteria and interventional options involved in managing this cause of CSF overproduction. The present case demonstrates that angiographic confirmation of prominent choroidal arteries may contribute to the diagnosis DVHCP. In addition, embolization of the distal choroidal arteries may be considered as a potential adjuvant treatment in patients for whom conventional treatments have failed or are not feasible.
- [Hydrocephalus due to hyperplasia of the choroid plexuses in a patient with trisomy 9 mosaicism. A real diagnostic and therapeutic challenge]. [Case Reports]
- RNRev Neurol 2017 Aug 01; 65(3):112-116
- CONCLUSIONS: This is another example of an association between choroid plexus pathologies and chromosome 9 abnormality. Due to its scarce incidence, diagnosis of hydrocephalus secondary to plexus hyperplasia is difficult, as is selecting its most suitable treatment. In this type of hydrocephalus there is a double pathophysiological mechanism, which involves an increase in CSF production and a decrease in its reabsorption. Despite taking these considerations into account, the treatment of hydrocephalus secondary to plexus hyperplasia is a real challenge that usually leads to multiple surgical interventions ranging from plexectomy or coagulation of the choroid plexuses to the implantation of CSF drainage devices.
- A balanced view of choroid plexus structure and function: Focus on adult humans. [Review]
- ENExp Neurol 2015; 267:78-86
- Recently tremendous progress has been made in studying choroid plexus (CP) physiology and pathophysiology; and correcting several misconceptions about the CP. Specifically, the details of how CP, a l…
Recently tremendous progress has been made in studying choroid plexus (CP) physiology and pathophysiology; and correcting several misconceptions about the CP. Specifically, the details of how CP, a locus of the blood-CSF barrier (BCSFB), secretes and purifies CSF, generates intracranial pressure (ICP), maintains CSF ion homeostasis, and provides micronutrients, proteins and hormones for neuronal and glial development, maintenance and function, are being understood on a molecular level. Unequivocal evidence that the CP secretory epithelium is the predominant supplier of CSF for the ventricles comes from multiple lines: uptake kinetics of tracer (22)Na and (36)Cl penetration from blood to CSF, autoradiographic mapping of rapid (22)Na and (36)Cl permeation (high permeability coefficients) into the cerebroventricles, CSF sampling from several different in vivo and in vitro CP preparations, CP hyperplasia that increases CSF formation and ICP; and in vitro analysis of CP ability to transport molecules (with expected directionality) and actively secrete fluid against an hydrostatic fluid column. Furthermore, clinical support for this CP-CSF model comes from neurosurgical procedures to remove lateral ventricle CPs in hydrocephalic children to reduce CSF formation, thereby relieving elevated ICP. In terms of micronutrient transport, ascorbic acid, folate and other essential factors are transported by specific (cloned) carriers across CP into ventricular CSF, from which they penetrate across the ependyma and pia mater deeply into the brain to support its viability and function. Without these choroidal functions, severe neurological disease and even death can occur. In terms of efflux or clearance transport, the active carriers (many of which have been cloned and expressed) in the CP basolateral and apical membranes perform regulatory removal of some metabolites (e.g. choline) and certain drugs (e.g. antibiotics like penicillin) from CSF, thus reducing agents such as penicillin to sub-therapeutic levels. Altogether, these multiple transport and secretory functions in CP support CSF homeostasis and fluid dynamics essential for brain function.
- Endoscopic coagulation of choroid plexus hyperplasia. [Case Reports]
- JNJ Neurosurg Pediatr 2012; 9(2):169-77
- Hydrocephalus is a clinical disorder resulting from an imbalance between the production of CSF and its resorption, of which the latter is mostly a disadvantage. In rare cases of choroid plexus papill…
Hydrocephalus is a clinical disorder resulting from an imbalance between the production of CSF and its resorption, of which the latter is mostly a disadvantage. In rare cases of choroid plexus papilloma or carcinoma, hydrocephalus is due to an overproduction of CSF. Choroid plexus hyperplasia (CPH) is a distinct clinicopathological entity in which the enlarged choroid plexus produces large amounts of CSF. Historically, patients with CPH were treated by shunt procedures or by microsurgical removal of the choroid plexus, which is associated with a high complication rate. In this paper the authors show that endoscopic plexus coagulation can result in restoring the equilibrium of the intracranial fluid volumes, resulting in shunt independency. In this way, both the shunt-related complications and the bleeding risks of microsurgical plexectomy are avoided. In instances of hydrocephalus, thorough efforts should be made to demonstrate the underlying pathophysiology to choose the optimal treatment, of which shunt procedures should receive the least priority.
- Multiloculated hydrocephalus. [Journal Article]
- CNChilds Nerv Syst 2011; 27(10):1609-19
- CONCLUSIONS: (1) Multiloculated hydrocephalus is a severe disease in which no single treatment has clearly been shown to be superior. (2) The goal of treatment is to restore communication between isolated intraventricular compartments in order to create the possibility of the implantation of a simple shunt with only one intraventricular catheter. More than improving the quality of life the patient, the objective is to reduce the number of surgical procedures. (3) Given the complexity of multiloculated hydrocephalus, each patient must be studied individually, and no procedure proposed by the literature should be ruled out, no matter how old fashioned may appear.
- Single-stage bilateral choroid plexectomy for choroid plexus papilloma in a patient presenting with high cerebrospinal fluid output. [Case Reports]
- JNJ Neurosurg Pediatr 2010; 5(4):342-5
- Cerebrospinal fluid overproduction resulting in communicating hydrocephalus is observed in patients who have choroid plexus papilloma or choroid plexus carcinoma. Less often, patients with these cond…
Cerebrospinal fluid overproduction resulting in communicating hydrocephalus is observed in patients who have choroid plexus papilloma or choroid plexus carcinoma. Less often, patients with these conditions have diffuse villous hyperplasia. Prior studies report CSF production greater than 3 L per day in these patients. These patients are treated with CSF shunting or by either unilateral choroid plexectomy or staged bilateral choroid plexectomy. The authors present a patient who had a number of congenital anomalies and a karyotype that revealed balanced translocations, 5 to 7 and 9 to 11. She presented with hydrocephalus and had CSF production of 5 L per day, greater output than ever previously reported. She was treated with a single-stage bilateral choroid plexectomy. Histopathological analysis revealed a bilateral choroid plexus papilloma. Postoperatively, the patient responded well clinically and showed radiographic improvement of her hydrocephalus. Bilateral choroid plexus papilloma has been reported in the literature as a cause for neonatal and congenital hydrocephalus. It can result in high CSF output and can be successfully treated with a single-stage bilateral choroid plexectomy. Further studies are ongoing to identify genes involved in embryogenesis of the choroid plexus.
- Bilateral choroid plexus hyperplasia: a case report and management strategies. [Case Reports]
- CNChilds Nerv Syst 2009; 25(12):1617-22
- CONCLUSIONS: Of the cases reported in the literature, treatments have included CSF shunting, endoscopic coagulation, and craniotomy with plexectomy. CSF shunting was required in the majority but not all. Only those having undergone bilateral choroid plexectomy have been rendered shunt free.
- The role of endoscopic choroid plexus coagulation in the surgical management of bilateral choroid plexuses hyperplasia. [Case Reports]
- CNChilds Nerv Syst 2006; 22(6):605-8
- CONCLUSIONS: On these grounds, we suggest that endoscopic choroid plexuses coagulation, even when failing to normalize CSF production, may still be considered as a valid adjuvant procedure in the management of this condition.
- Choroid plexectomy reduces neurosurgical intervention in patients with hydranencephaly. [Journal Article]
- PNPediatr Neurosurg 2002; 36(3):148-52
- CONCLUSIONS: In our experience, choroid plexectomy in patients with hydranencephaly reduces the incidence of reoperation and readmission, the number of days of hospitalization related to the surgical procedure and the total number of complications in comparison to patients undergoing CSF diversion. Further neurosurgical intervention is minimized as is the financial burden from multiple emergency department visits and radiological procedures for shunt evaluation. Choroid plexectomy is a viable alternative to CSF diversion in patients with hydranencephaly and a rapidly enlarging head. It avoids the chronic issues and complications surrounding CSF diversion in this difficult group of patients.
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- [Hypersecretive congenital hydrocephalus due to choroid plexus villous hypertrophy associated with controlateral papilloma]. [Case Reports]
- MPMinerva Pediatr 1993; 45(9):363-7
- The authors present a case of a nine month-old baby with hypersecretive congenital hydrocephalus. The CSF overproduction was the result of a rare abnormality of the choroid plexus, a villous hypertro…
The authors present a case of a nine month-old baby with hypersecretive congenital hydrocephalus. The CSF overproduction was the result of a rare abnormality of the choroid plexus, a villous hypertrophy of the right ventricular choroid plexus and a left ventricular plexus papilloma. Surgical removal of the plexus papilloma and the plexectomy of the hypertrophic ventricular structure did not ameliorate the hydrocephalus, which has been controlled after a biventricular-peritoneal shunt. The physiopathological relationship between hydrocephalus and choroid plexus abnormalities are reported. The importance of an early diagnosis and treatment is stressed. Both hydrocephalus and choroid plexus pathology may represent a cause of psychomotor delay, for the little patient the effects of which is investigated in this paper.