Paediatric Interventional Neuroradiology at Great Ormond Street Hospital

Through his work at Great Ormond Street Hospital (GOSH) Dr Adam Rennie is one of the few doctors in the UK performing interventional neuroradiology procedures in children.

The Paediatric interventional neuroradiology practice at Great Ormond Street Hospital is the largest in the world, and he works with world class Neurologists, Neurosurgeons, Anaesthetists and Intensivists to perform complex procedures in a safe and high quality environment.

Dr Adam Rennie provides the Nationally Commissioned service of Vein of Galen Malformation treatment. He is also involved in delivering stereotactic radiosurgery to paediatric AVMs, another NHS England commissioned service.

Vein of Galen Malformation

This is a very rare vascular malformation that usually presents as a newborn baby or in infancy. In this malformation there are abnormal connections between arteries and veins deep inside the child’s brain. As a newborn baby these connections have enormous amount of blood flow through them, which causes the baby’s heart to not work well and also causes a reduction in blood supply to the rest of the baby’s body.

In infancy the presentation is different. These children suffer less from heart failure and instead have problems with the venous drainage of their brain.  They also suffer from problems associated with the enlargement of the water-spaces within the brain (called hydrocephalus). Together this presentation is usually called ‘hydrovenous’.

Treatment of Vein of Galen Malformation is complex, but requires the injection of glue or coils to block up the abnormal communications between the arteries and veins.


"Mend My Brain"

Dr Adam Rennie featured in the BAFTA nominated 2015 BBC Great Ormond Street Hospital Series in the episode entitled “Mend My Brain”. In this episode he treats two patients with Vein of Galen Malformation.

See a preview via the BBC iPlayer below, or watch the full story in the media.

A Mother's Story

I'd never heard of vein of Galen malformations, and I couldn't really take it all in. Looking back, I think both Steve and I were numb with shock. Watching the documentary, I was completely thrown when I heard Dr Rennie talk about the risks involved in surgery - I now realise that at the time I wasn't really absorbing that information at all. Steve and I didn't do any research into the condition. Instead, we placed our faith in Dr Rennie and his team and I am so glad we did. Our family owe him everything.


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Paediatric Pial Arteriovenous Fistulas

Pial Arteriovenous Fistulas are rare high-flow direct communications between brain supplying arteries and veins.  They can present similarly to Vein of Galen Malformations, with heart failure and problems with venous drainage of the brain.

The treatment of these malformations also involves the injection of glue or coils to block up the abnormal connections, keeping all the normal brain supplying arteries intact.

Paediatric Arteriovenous Malformations

Arteriovenous Malformations (AVMs) are abnormal clusters of vessels (the nidus) located within the brain (or occasionally spine) through which blood passes rapidly without supplying the brain tissue.

The propensity for these AVMs to develop is probably present at birth but grow over time.

Paediatric Arteriovenous Malformation

AVMs can present in several ways in children.  Most commonly they present when they bleed, caused by rupture of the tiny vessels within the nidus. Other presentations include seizures or headaches, which in both cases would have led to an MRI scan on which the diagnosis is made.

Treatment

Once diagnosed by MRI scan, most patients will need a catheter angiogram to investigate the AVM and its nidus and other features fully.

Decision making about the treatment options of AVMs of the brain and spine is complex and depend on numerous factors such as whether it has bled, the future risk of bleeding, the size and other angiographic features of the AVM and the child’s age.

Four options exist for treatment of paediatric brain AVMs:

  • Endovascular embolisation. Insertion of small catheters into the brain arteries to inject a liquid to block up the abnormal connections.
  • Open surgery to remove the AVM. This is the most definitive method to treat AVMs, and is often combined with a pre-operative embolisation to tackle deeper and more difficult to reach sections to make the subsequent surgery easier.
  • Stereotactic radiosurgery. The use of high dose concentrated radiation treatment to gradually shrink the AVM.  This is effective at treating many AVMs but is limited by the slow effect (often over 2 or 3 years).
  • No treatment, and continue to observe the AVM often with serial MRI scans.

At Great Ormond Street Hospital we are able to provide all options in treating AVMs, including stereotactic radiosurgery. Our Neurovascular Multidisciplinary Team meeting is crucial in the decision making process.

Intra-arterial Chemotherapy for Retinoblastoma

Retinoblastoma is a rare eye cancer, representing 3% of all childhood cancers.  It is inherited in about one third of cases. In the UK there are about 40 new cases per year.

Treatment of retinoblastoma depends on the stage of the disease.  Small tumours with a limited amount of disease get local therapies (laser or cryotherapy).  More extensive tumours have IV chemotherapy as the first line treatment.

Current practice in the UK is to treat those children who relapse following IV chemotherapy with a course of chemotherapy infused directly into the main blood supply of the eye, the ophthalmic artery. This allows a high dose of chemotherapy to reach the tumour, but requires positioning a small catheter in the artery at the time of an angiogram.

The procedure is effective in saving vision in about 60% of the eyes, where the only alternative option would be enucleation.


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