The evolution of treatment for hypothalamic hamartoma: a personal odyssey

Abstract

<jats:p>The prognosis for patients with hypothalamic hamartoma has improved dramatically over the last 20 years, for 3 main reasons. First, because of improved understanding of the anatomy and pathophysiology of these varied lesions. Second, due to advances in brain imaging and refinements in microsurgery, including the anterior transcallosal interforniceal approach, endoscopic, and skull-base approaches. And third, because of increasing experience with stereotactic radiosurgery, interstitial radiotherapy, and radiofrequency lesioning. Patients with hypothalamic hamartoma should be managed in comprehensive epilepsy centers where the treatments are individualized and concentrated in the hands of surgeons who can perform the full range of surgery, including approaches to the third ventricle. Total seizure-freedom rates of 52% to 66% have been achieved with surgery.</jats:p>

Hypothalamic hamartomas. Part 2. Surgical considerations and outcome

“…Broadly speaking, microsurgical approaches can be divided into those that reach the diencephalic mass from below and those that reach it from above. 65 Others advocate that disconnection from the mammillary bodies rather than complete excision of the HH is sufficient to eliminate seizures, and that this procedure carries less surgical morbidity. Still others believe that a strictly noninvasive modality such as SRS is the optimal choice for managing these difficult lesions.…”

Synchronous Occurrence of a Hemorrhagic Hypothalamic Hamartoma and a Suprasellar Teratoma

“…Although gelastic seizures are the prototypic seizures in a case of an HH, other seizure types, including generalized seizures, are known to develop secondary to the spread of seizure activity from the mammillary bodies to the thalamus and cortex via the mammillothalamic tract [27]. While confirming the intrinsic epileptogenic potential of these lesions by depth electrode placement, Shim et al [28] reported good control of all HH-associated seizures after disconnection/excision of the lesion.…”

Endoscopic disconnection of hypothalamic hamartomas: safety and feasibility of robot-assisted, thulium laser–based procedures

“…Nevertheless, resective surgery carries the risk of severe complications including memory deficits, endocrinological disturbances, electrolyte disorders, motor deficits, and oculomotor impairment. 13,[24][25][26] Furthermore, total HH removal could be attained in approximately 50% of the procedures, but postoperative seizure control largely depends on the extent of the resection. 1 Finally, the resection is often complicated by the macroscopic appearance of the hamartoma that does not easily allow the surgeon to discriminate the limits of the lesion from the hypothalamus, which is frequently distorted by the lesion.…”

“…Nonetheless, stereotactic radiosurgery may play an important role in adult patients, who are less susceptible to radio-induced complications. 25 Curry et al recently reported 2 cases of HHs treated by stereotactic laser cooled thermoablation under MRI control and thermal monitoring. Interestingly, the 980-nm laser and the integrated cooling system allowed a sharp fall off in temperature at the edge of the ablation zone, with a clear cut at the lesional zone and preservation of the surrounding important structures.…”

“…Resection of such benign lesions has been associated with a significant risk of neurological sequelae, 13,25 including memory deficits, visual defects, and damage to the capsular region, which typically results in major motor deficits. Furthermore, iatrogenic hypothalamic injuries may cause life-threatening complications, including serum electrolyte alterations, syndrome of inappropriate antidiuretic hormone release, diabetes insipidus, hyperthermia, hyperphagia, hypothyroidism, and poikilothermia.…”