Home > Medical Specialties, Centers and Clinics > Hypothalamic Hamartoma Center > Team Approach > Neurosurgery

Role of Neurosurgery in Hypothalamic Hamartoma Treatment

Barrow Neurological Institute of St. Joseph's Hospital and Medical Center, Phoenix, Arizona

Surgical intervention is indicated only after radiographic and physical examination confirms the diagnosis of hypothalamic hamartoma (HH). The lesion can be treated surgically when patients fail to respond to medical treatment, either in terms of poor seizure control, unacceptable side effects, or both. Complete or near complete resection of hamartoma lesions can be achieved in a relatively safe manner. Surgical approaches are continuously being modified and enhanced to meet the needs of the individual patients.

Following successful removal of the tumor, the clinical manifestations of hypothalamic hamartoma, including epileptic seizures, behavioral disturbance, and precocious puberty are improved, or may return to normal. This form of intervention is being increasingly advocated, although long-term outcome remain under study.

Harold L. Rekate, M.D., Barrow Neurological Institute

The Role of the Surgeon

The role of the surgeon in a team that is managing patients with hypothalamic hamartomas (HHs) is a very challenging one. It begins with the assessment of the individual patient including his or her clinical state and the specific size and anatomy of the lesion. It is only the surgeon that can assess the specific nature of the risks of surgical intervention in an individual case.

In planning surgery there may be some potential variations in the approach and positioning that can relate to the ease of removing the hamartoma and the ability to get an adequate resection. This includes such as the selection of specific microsurgical pathways (the surgical “approach”), and how the patient positioned in surgery.

Barrow's transcallosal approach to hypothalamic hamartoma surgery

Our Approach

Two surgical procedures are now performed most commonly for hypothalamic hamartoma patients.

The first is the transcallosal interforniceal approach (“transcallosal”), in which the surgeon creates a craniotomy (a window in the skull), and navigates between the two hemispheres of the brain, then through a small portion of the corpus callosum, to reach the hypothalamic hamartoma tumor in the third ventricle.

The second procedure is the transventricular endoscopic approach (“endoscopic”) in which the surgeon uses a small burr hole in the skull to place a surgical endoscope into the ventricular system, and thus gains visualization and access to the hypothalamic hamartoma for resection.

Barrow's endoscopic approach to hypothalamic hamartoma
treatment

The take home message in all of this is that no two patients, and no two hamartomas are alike. Each patient brings with them unique challenges that inspire us to learn from them. These challenges emphasize the importance of clinical and surgical experience in managing these complex lesions. Some hypothalamic hamartoma patients require alternative surgical approaches.

For example, in patients presenting with small hamartomas that have both a seizure disorder and precocious puberty, it may not be possible to remove the mass from the standard transcallosal or endoscopic surgical approaches. In this small number of cases an alternative approach may be necessary such as the orbitozygomatic approach or a modified technique referred to as the "eyebrow approach."

One of the most important roles of the surgeon is to clearly define the risks and benefits of the surgical approach to the family and patient, specifically related to his or her own mass. Ideally, it should be the patient (or his or her surrogate) who should make the decision as to whether the benefits outweigh the risks in the treatment plan. Their questions and concerns must be addressed to ensure the family as well as the patient have a clear understanding of the recommended treatment.

Postoperative Care for Hypothalamic Hamartoma Patients

The postoperative care of our hypothalamic hamartoma patients is a shared experience by our team members who participate in the treatment plan at various phases of the recovery process. Standardized protocols and procedures have been identified to ensure successful patient outcomes. Barrow Neurological Institute is fortunate to have dedicated and highly-trained medical professionals devoted to making the experience at Barrow the best it can be.