A hypothalamic hamartoma can cause many types of seizures and other symptoms, which can vary from one person to the next. Many people have some combination of the following problems:
- Gelastic or dacrystic seizures (focal seizures with laughing or crying)
- Other seizure types, such as focal seizures with impaired awareness and with motor features, and generalised tonic-clonic seizures
- Cognitive problems (such as changes in thinking, memory, attention)
- Sudden episodes of rage and aggressive behaviour
- Precocious (early) puberty
Diagnosis and management of hypothalamic hamartoma
Children and young people with hypothalamic hamartoma usually present with seizures. A small subset may not have seizures, but present with early puberty.
Seizures can start at any age, and often don’t respond well to seizure medications and are drug resistant. In addition, there can be significant impact of daily seizures, cognitive problems, and behavioural problems on the well-being of the child and their family.
It is essential that children and young people with this condition be managed at a centre of excellence for the management of hypothalamic hamartoma. This would help in accurate diagnosis, appropriate investigations, comprehensive evaluation by a multidisciplinary team and recommendations regarding different options to treat this condition.
As a part of the comprehensive evaluation, children would have a clinic appointment with the multidisciplinary team to obtain relevant information, detailed 3Tesla MRI scan of the head, video-EEG to capture the habitual seizures, neuropsychology and neuropsychiatry assessment and an endocrinology consult.
Professor Conor Mallucci, Consultant Paediatric Neurosurgeon, Alder Hey Children’s Hospital
Conor is the lead for the Northwest Paediatric Neurosciences Network and is the chairman of the British Paediatric Neurosurgery Research Network. He is committed to improving outcomes of children with brain tumours and has published extensively in this field and regarding neuroendocospy and technical advances in neurosurgery. Conor has expertise in surgical resection of midline brain tumours, and hypothalamic hamartoma.
Miss Sasha Burn, Consultant Paediatric Neurosurgeon, Alder Hey Children’s Hospital
Sasha is the lead epilepsy neurosurgeon at Alder Hey Children’s Hospital, and works within the children’s epilepsy surgery services. She has expertise in all aspects of epilepsy surgery including the surgical resection of neurodevelopmental tumours, focal dysplasias, invasive epilepsy monitoring and hypothalamic hamartoma; disconnective surgery and invasive epilepsy monitoring.
Dr Anand Iyer, Consultant Paediatric Neurologist, Alder Hey Children’s Hospital
Anand is an epileptologist and works within the children’s epilepsy surgery services. He has expertise in evaluation and management of complex epilepsies in children, and in clinical neurophysiology evaluation with video-EEG of children with different epilepsies, including hypothalamic hamartoma.
Dr Nina Swiderska, Consultant Paediatric Neurologist, Alder Hey Children’s Hospital
Nina is an epileptologist and works within the children’s epilepsy surgery services. She has expertise in evaluation and management of complex epilepsies in children, including hypothalamic hamartoma.
Dr Jeen Tan, Consultant Paediatric Neurologist, Royal Manchester Children’s Hospital
Jeen is an epileptologist and works within the children’s epilepsy surgery services. She has expertise in evaluation and management of complex epilepsies and is the clinical lead for the ketogenic diet service in children at Manchester.
Dr Mohammed Didi, Consultant Paediatric Endocrinologist, Alder Hey Children’s Hospital
Mo is a senior consultant paediatric endocrinologist and has expertise in the pre-operative evaluation of children with hypothalamic hamartoma, and management of post-operative endocrine complications in this group.
Dr Victoria Gray, Consultant Paediatric Neuropsychologist
Vicky is a consultant paediatric neuropsychologist and works within the children’s epilepsy surgery services.
Dr Lakshmi Ramasubramanian, Consultant Child Psychiatrist
Lakshmi is a consultant child and adolescent psychiatrist with expertise in the assessment and management of neuropsychiatric problems in children with learning disabilities, and works within the children’s epilepsy surgery services.
Mrs Andrea McLaren, Lead nurse epilepsy surgery network
Andrea is the lead epilepsy surgery nurse and works within the children’s epilepsy surgery services. She is instrumental in supporting the families through the process of assessment and even after surgery and is an independent nurse prescriber.
What types of treatment are available?
At Alder Hey, we offer open surgery, both the transcallosal interforniceal approach and the transcortical endoscopic approach. In all cases, we use out intraoperative MRI scanner which enables imaging of the resection during the operation enhancing both extent of the resection and safety. (Open resection of hypothalamic hamartomas for intractable epilepsy revisited, using intraoperative MRI. van Tonder L, Burn S, Iyer A, Blair J, Didi M, Carter M, Martland T, Mallucci C, Chawira A. Childs Nerv Syst. 2018 Sep; 34(9):1663-1673).
We also have a laser we can use during open surgery. We have facility to insert stereo electrodes (used during stereo-electroencephalography, sEEG) into the hamartoma, to monitor seizures and to thermocoagulate (melt) the hamartoma if deemed appropriate.
We have close links with Great Ormond Street Hospital for Children, London and other centres of excellence in the USA, where MRI guided laser thermal ablation is offered, and we can refer patients on to these units if required.
The type of surgery recommended for a person with HH is chosen based on a number of factors, such as the size and location of the hamartoma, seizure frequency, and cognitive function. A large hamartoma typically requires surgeries in different phases or a combined approach.
For any enquiries please contact Nicola Gibbons or Natasha Jacobson