Pineal Cyst Pathway
Introduction​
Pineal cysts are a common incidental finding on MRI scan occurring in approximately 5% of MRI brain scans. Whilst the size of the cyst may change over time in most cases no clinical symptoms occur(1,2,3,4,5,6,7,8) They occur more commonly in the third and fourth decade and less frequently under 10 years of age with a predilection for women (3:1 female to male ratio). 

Only a small number of pineal cysts have the potential to grow with cyst shrinkage more common (2,8). The configuration of the pineal cyst does not predict future growth. Pineal cysts do not require routine long term neurosurgical follow up in asymptomatic patients. (2,3,4,5,7). Intervention is rarely needed except in cases of obstructive hydrocephalus (1,4,6,7,8). 

A 10 year audit at Southampton to determine the incidence and management of pineal cysts between 2013-2023 found 139 patients with pineal cyst. One patient had clinical symptoms of raised intracranial pressure with a 16mm pineal cyst plus dilated ventricles, necessitating fenestration of the cyst and resolution of hydrocephalus. No other patient required intervention and no pineal cysts were subsequently diagnosed as a pineal tumour.

Concern regarding presence of a pineal tumour associated with the pineal cyst causes patient anxiety and clinician uncertainty leading to repeated surveillance imaging.

The guidelines aims to address the need for surveillance MRI imaging for pineal cysts and to provide a standardised referral pathway where needed. ​
Scope and Purpose
This guideline applies to all patients under the age of 16 years with a pineal cyst identified on brain imaging.
Definitions​
Pineal Cyst – a fluid filled space within the pineal gland. They can present as a single cyst, multiple cysts or a cyst with septations within it. 

ICP - intracranial pressure 

Parinaud syndrome (also known as dorsal midbrain syndrome) – vertical gaze palsy with convergence retraction nystagmus, pupillary hyporeflexia and lid retraction 

CISS sequence - (Constructive Interference in Steady State sequence) is a high resolution T2 weighted sequence that is good for showing cysts. It is a Siemens specific sequence but there will be equivalent sequences on scanners from other manufacturers. ​
Flowchart
Patient Information Leaflet
Process for Monitoring Compliance
The PIER network will review problems associated with this guideline through governance process. 
Document Review
Guideline to be reviewed after three years or sooner as a result of audit findings or as any changes to practice occurs.
References​
  1. Michael D Jenkinson, Samantha Mills, Conor L Mallucci and Thomas Santarius. Management of pineal and colloid cysts. Pract Neurol. 2021 Aug; 21(4): 292–299.  Available here. 
  2. ​Gaillard F, Botz B, Jones J, et al. Pineal cyst. Reference article. Radiopaedia.org October 2022
  3. Erkan Gokce and Murat Beyhan. Evaluation of pineal cysts with magnetic resonance imaging. World J Radiol. 2018 Jul 28; 10(7): 65–77.  Available here. 
  4. Mathew Storey, Konstantinos Lilimpakis, Nora Sangvik Grandal, Chittoor Rajaraman, Shailendra Achawal, Masood Hussain. Pineal cyst surveillance in adults - a review of 10 years' experience. Br J Neurosurg . 2020 Oct;34(5):565-568.  Available here. 
  5. Edward J Nevins, Kumar Das, Maneesh Bhojak, Rohan S Pinto, Mohammed N Hoque, Michael D Jenkinson, Emmanuel Chavredakis. Incidental Pineal Cysts: Is Surveillance Necessary? World Neurosurg. 2016 Jun:90:96-102.  Available here. 
  6. Hirotaka Hasegawa, Akitoshi Inoue, Ahmed Helal, Kosuke Kashiwabara Fredric B. Meyer. Pineal cyst: results of long-term MRI surveillance and analysis of growth and shrinkage rates JNS: May 2022 113-119 vol 138:issue 1
  7. Cormac O. Maher, MD; Joseph H. Piatt, Jr, MD; David P. Gruber, MD; Andrew H. Jea, MD; Douglas Brockmeyer, MD; Ann Ritter, MD. Incidental Findings on Brain and Spine Imaging in Children. Pediatrics (2015) 135 (4): e1084–e1096
  8. Martin Majovsky & Vladimir Benes. Natural course of pineal cysts—a radiographic study. Chinese Neurosurgical Journal volume 4, Article number: 33 (2018).  Available here. 
Document Version: 
1.0

Lead Authors: 
Dr Georgina Bird-Lieberman, Paediatric Neurologist
Ms Wahab, Paediatric Neurosurgeon
Mr Waters, Paediatric Neurosurgeon
Dr Joy, Consultant Neuroradiologist
​Shona Mackie, Paediatric Neurology Nurse Specialist
Approving Network:
Wessex Paediatric Neurosciences Clinical Network

Date of Approval: 
November 2024

Review Due:
November 2027