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Hypothalamic hamartomas (HHs) are rare congenital lesions formed by heterotopic neuronal and glial cells attached to the mammillary bodies, tuber cinereum, and hypothalamus.They often present with an intractable epilepsy typically characterized by gelastic seizures but commonly associated with other types of refractory seizures. The clinical course is progressive in most of the cases, starting with gelastic seizures in infancy and deteriorating into complex seizure disorders that result in catastrophic epilepsy associated with cognitive decline and behavioral disturbances.Hamartomas are known to be intrinsically epileptogenic and the site of origin for the gelastic seizures. As antiepileptic drugs are typically ineffective in controlling HH-related epilepsy, different surgical options have been proposed as a treatment to achieve seizure control. Resection or complete disconnection of the hamartoma from the mammillothalamic tract has proved to achieve a long-lasting control of the epileptic syndrome.Usually, symptoms and their severity are typically related to the size, localization, and type of attachment. Precocious puberty appears mostly in the pedunculated type, while epileptic syndrome and behavioral decline are frequently related to the sessile type. For this reason, different classifications of HHs have been developed based on their size, extension, and type of attachment to the hypothalamus.The bigger and more complex hypothalamic hamartomas typically present with severe refractory epilepsy, behavioral disturbances, and progressive cognitive decline posing a formidable challenge for the control of these symptoms.We present here our experience with the multimodal treatment for complex hypothalamic hamartomas. After an in-depth review of the literature, we systematize our approach for the different types of hypothalamic hamartomas., (© 2024. The Author(s), under exclusive license to Springer Nature Switzerland AG.)

Objective: Hypothalamic hamartomas are congenital lesions that typically present with gelastic seizures, refractory epilepsy, neurodevelopmental delay, and severe cognitive impairment. Surgical procedures have been reported to be effective in removing the hamartomas, however, they are associated with significant morbidity. Therefore, it is not considered a safe therapeutic modality. Image-guided robotic radiosurgery (CyberKnife® Radiosurgery System) has been shown to provide good outcomes without lasting complications., Methods: This series of cases describes the clinical, radiological, radiotherapeutic, and postsurgical outcomes of five patients with epileptic encephalopathies secondary to hypothalamic hamartomas who were treated with CyberKnife®., Results: All patients exhibited refractory epilepsy with gelastic seizures and were unsuitable candidates for surgical resection The prescribed dose ranged between 16 and 25 Gy, delivered in a single fraction for four patients and five fractions for one patient while adhering strictly to visual pathway constraints. After radiosurgery, four patients maintained seizure control (one with an Engel class Ia, three with an Engel class 1d), and another presented sporadic, nondisabling gelastic seizures (with an Engel class IIa). After 24-26 months of follow-up, in three patients, their intelligence quotient scores increased. No complications were reported., Significance: This report suggests that Cyberknife may be a good option for treating hypothalamic hamartoma, particularly in cases where other noninvasive alternatives are unavailable. Nevertheless, additional studies are essential in order to evaluate the effectiveness of the technique in these cases., (© 2024 International League Against Epilepsy.)

Background: To describe and report the efficacy and safety of MR-guided laser interstitial thermal therapy (MRgLITT) in the treatment of drug-resistant epilepsy. Methods: A retrospective review of all MRgLITT procedures in our hospital was performed. All procedures were performed using a surgical laser ablation system. Demographic and outcome data were compiled and analyzed. Results: A total of 19 patients underwent MRgLITT procedures from June 2021 to November 2021. The average age at surgery was 18.1 years (3–61.4 years). The average length of hospitalization post-surgery was 4.95 days (4–7 days). Surgical substrates included 8 patients with hypothalamic hamartomas, 5 with medial temporal lobe epilepsy, 3 with deep focal cortical dysplasia, 1 with tuberous sclerosis, 1 with a cavernous malformation, and 1 with Lennox–Gastaut syndrome who underwent anterior corpus callosotomy. Complications occurred in three patients. After an average follow-up of 1 year, 6 patients were seizure-free (Engel I, 31.6%), 1 had significant seizure control (Engel II, 5.3%), 7 had seizure control (Engel III, 36.8%), and 5 had no improvement in their seizures (Engel IV, 26.3%). Fisher's exact tests did not reveal statistical significance for the association between Engel class outcome and epileptic disease. Conclusion: This study confirmed that MRgLITT, as a method for treating drug-resistant epilepsy, is minimally invasive, safe, and efficient and that it can reduce the incidence of surgery-related complications. [ABSTRACT FROM AUTHOR]

The article discusses the use of Magnetic Resonance-guided Laser Interstitial Thermal Therapy (MRg-LiTT) in the minimally invasive surgical treatment of epilepsy and brain neoplasms. MRg-LiTT has been a significant breakthrough in neurosurgery, providing a therapeutic alternative with demonstrated safety and efficacy. It offers advantages such as reduced surgical complications, shorter hospital stays, and faster recovery. The article includes six papers that explore various topics related to MRg-LiTT, including treating deep brain lesions and testing physics and new therapeutic hypotheses. Overall, MRg-LiTT is a viable option for treating epileptogenic conditions and complements established surgical techniques. [Extracted from the article]

Magnetic resonance‐guided laser interstitial thermal therapy (MRgLITT) has emerged as a popular minimally invasive alternative to open resective surgery for drug‐resistant epilepsy (DRE). We sought to perform a systematic review and individual participant data meta‐analysis to identify independent predictors of seizure outcome and complications following MRgLITT for DRE. Eleven databases were searched from January 1, 2010 to February 6, 2021 using the terms "MR‐guided ablation therapy" and "epilepsy". Multivariable mixed‐effects Cox and logistic regression identified predictors of time to seizure recurrence, seizure freedom, operative complications, and postoperative neurological deficits. From 8705 citations, 46 studies reporting on 450 MRgLITT DRE patients (mean age = 29.5 ± 18.1 years, 49.6% female) were included. Median postoperative seizure freedom and follow‐up duration were 15.5 and 19.0 months, respectively. Overall, 240 (57.8%) of 415 patients (excluding palliative corpus callosotomy) were seizure‐free at last follow‐up. Generalized seizure semiology (hazard ratio [HR] = 1.78, p =.020) and nonlesional magnetic resonance imaging (MRI) findings (HR = 1.50, p =.032) independently predicted shorter time to seizure recurrence. Cerebral cavernous malformation (CCM; odds ratio [OR] = 7.97, p <.001) and mesial temporal sclerosis/atrophy (MTS/A; OR = 2.21, p =.011) were independently associated with greater odds of seizure freedom at last follow‐up. Operative complications occurred in 28 (8.5%) of 330 patients and were independently associated with extratemporal ablations (OR = 5.40, p =.012) and nonlesional MRI studies (OR = 3.25, p =.017). Postoperative neurological deficits were observed in 53 (15.1%) of 352 patients and were independently predicted by hypothalamic hamartoma etiology (OR = 5.93, p =.006) and invasive electroencephalographic monitoring (OR = 4.83, p =.003). Overall, MRgLITT is particularly effective in treating patients with well‐circumscribed lesional DRE, such as CCM and MTS/A, but less effective in nonlesional cases or lesional cases with a more diffuse epileptogenic network associated with generalized seizures. This study identifies independent predictors of seizure freedom and complications following MRgLITT that may help further guide patient selection. [ABSTRACT FROM AUTHOR]

Laser ablation for treatment of hypothalamic hamartoma (HH) is a minimally invasive and effective technique used to destroy hamartomatous tissue and disconnect it from the functioning brain. Currently, the gold standard to evaluate the amount of tissue being "burned" is the use of heat maps during the ablation procedure. However, these maps have low spatial resolution and can be misleading in terms of extension of the tissue damage. The aim of this study is to use different MRI sequences immediately after each laser ablation and correlate the extension of signal changes with the volume of malacic changes in a long-term follow-up scan. During the laser ablation procedure, we imaged the hypothalamic region with high-resolution axial diffusion-weighted images (DWI) and T2-weighted images (T2WI) after each ablation. At the end of the procedure, we also added a post-contrast T1-weighted image (T1WI) of the same region. We then correlated the product of the maximum diameters on axial showing signal changes (acute oedema on T2WI, DWI restriction rim, DWI hypointense core and post-contrast T1WI rim) with the product of the maximum diameters on axial T2WI of the malacic changes in the follow-up scan, both as a fraction of the total area of the hamartoma. The area of the hypointense core on DWI acquired immediately after the laser ablation statistically correlated better with the final area of encephalomalacia, while the T2WI, hyperintense oedema, DWI rim and T1WI rim of enhancement tended to overestimate the encephalomalacic damage. In conclusion, the use of intraoperative sequences (in particular DWI) during laser ablation can give surgeons valuable information in real time about the effective heating damage on the hamartomatous tissue, with better spatial resolution in comparison to the thermal maps. [ABSTRACT FROM AUTHOR]

Several instruments and outcomes measures have been reported in pediatric patients undergoing epilepsy surgery. The objective of this systematic review is to summarize, evaluate, and quantify outcome metrics for the surgical treatment of pediatric epilepsy that address seizure frequency, neuropsychological, and health‐related quality of life (HRQL). We performed a systematic review according to Preferred Reporting Items for Systematic Reviews and Meta‐Analyses guidelines to identify publications between 2010 and June 2021 from PubMed, Embase, and the Cochrane Database of Systematic Reviews that report clinical outcomes in pediatric epilepsy surgery. Eighty‐one articles were included for review. Overall, rates of postoperative seizure frequency were the most common metric reported (n = 78 studies, 96%). Among the seizure frequency metrics, the Engel Epilepsy Surgery Outcome Scale (n = 48 studies, 59%) was most commonly reported. Neuropsychological outcomes, performed in 32 studies (40%) were assessed using 36 different named metrics. HRQL outcomes were performed in 16 studies (20%) using 13 different metrics. Forty‐six studies (57%) reported postoperative changes in antiepileptic drug (AED) regimen, and time‐to‐event analysis was performed in 15 (19%) studies. Only 13 outcomes metrics (1/5 seizure frequency, 6/13 HRQL, 6/36 neuropsychological) have been validated for use in pediatric patients with epilepsy and only 13 have been assessed through reliability studies (4/5 seizure frequency, 6/13 HRQL, and 3/36 neuropsychological). Of the 81 included studies, 17 (21%) used at least one validated metric. Outcome variable metrics in pediatric epilepsy surgery are highly variable. Although nearly all studies report seizure frequency, there is considerable variation in reporting. HRQL and neuropsychological outcomes are less frequently and much more heterogeneously reported. Reliable and validated outcomes metrics should be used to increase standardization and accuracy of reporting outcomes in pediatric patients undergoing epilepsy surgery. [ABSTRACT FROM AUTHOR]

Hypothalamic hamartoma (HH) is a rare lesion consisting of normal neurons and neuroglia arranged in an abnormal pattern which usually causes gelastic seizures (GS). Magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) has been developed as a minimally invasive approach to treat HH and gradually become a first-line treatment. In total, this study enrolled 47 consecutive HH patients that underwent one round of ablation. Patients were followed for at least one year. Patients' medical records and surgical information were carefully reviewed, and univariate analyses were performed. Of the treated patients, 72.3% remained GS-free in this study, with an overall Engel class I rate of 68.1%. Long-term postoperative complications occurred in six patients. Factors associated with GS prognosis included Delalande classification (p = 0.033), HH volume (p = 0.01), and the ablation rate of the HH body (p = 0.035). The disconnection rate was 0.73 ± 0.14 in the Engel class Ia group as compared to 0.62 ± 0.13 in the Engel Ib–Engel IV group (p = 0.046). MRgLITT represents a safe and effective surgical procedure. Patients with larger or Delalande type IV HH may require multiple rounds of ablation. In addition to assessing the degree of disconnection, ablation volume should also be carefully considered for patients undergoing this procedure. [ABSTRACT FROM AUTHOR]

Background: Approximately 1/3 of patients with epilepsy have drug-resistant epilepsy (DRE) and require surgical interventions. This meta-analysis aimed to review the effectiveness of MRI-guided laser interstitial thermal therapy (MRgLITT) in DRE.Methods: The Population, Intervention, Comparator and Outcome approach and Preferred Reporting Items for Systematic Reviews and Meta-Analyses were followed. PubMed, MEDLINE and EMBASE databases were systematically searched for English language publications from 2012 to Nov 2020. Data on the prevalence outcome using the Engel Epilepsy Surgery Outcome Scale (Class I-IV), and postoperative complications were analysed with 95% CIs.Results: Twenty-eight studies that included a total of 559 patients with DRE were identified. The overall prevalence of Engel class I outcome was 56% (95% CI 0.52% to 0.60%). Hypothalamic hamartomas (HH) patients had the highest seizure freedom rate of 67% (95% CI 0.57% to 0.76%) and outcome was overall comparable between mesial temporal lobe epilepsy (mTLE) (56%, 95% CI 0.50% to 0.61%) and extratemporal epilepsy (50% 95% CI 0.40% to 0.59%). The mTLE cases with mesial temporal sclerosis had better outcome vs non-lesional cases of mTLE. The prevalence of postoperative adverse events was 19% (95% CI 0.14% to 0.25%) and the most common adverse event was visual field deficits. The reoperation rate was 9% (95% CI 0.05% to 0.14%), which included repeat ablation and open resection.Conclusion: MRgLITT is an effective and safe intervention for DRE with different disease aetiologies. The seizure freedom outcome is overall comparable in between extratemporal and temporal lobe epilepsy; and highest with HH.Trail Registration Number: The study protocol was registered with the National Institute for Health Research (CRD42019126365), which serves as a prospective register of systematic reviews. It is an international database of prospectively registered systematic reviews with a focus on health-related outcomes. Details about the protocol can be found at https://wwwcrdyorkacuk/PROSPERO/. [ABSTRACT FROM AUTHOR]

Summary: Objective: To investigate long‐term seizure outcomes in patients with hypothalamic hamartoma (HH) following stereotactic radiofrequency thermocoagulation (SRT). Methods: A total of 131 patients with HH who underwent SRT and were followed for at least three years after the last SRT were enrolled. Seizure outcomes were evaluated for gelastic seizures (GS) and other types of seizures (nGS) separately using the International League Against Epilepsy classification. Classes 1 and 2 were considered seizure‐free. Kaplan‐Meier survival analyses were used to estimate the proportion remaining seizure‐free after the first and last SRTs. Risk factors relating to outcomes were analyzed by log‐rank tests and a multivariate Cox proportional hazards model. Results: Reoperation was performed in 34 patients (26.2%). Median total follow‐up was 61 (range, 36–202) months. Seizure freedom was obtained in 116 patients (88.6%) for GS and 85 of 108 patients (78.7%) for nGS at the last follow‐up. Mean GS‐free survival times improved from after the first (64.1 [95%CI 57.3–70.9] months) to after the last SRT (80.2 [95%CI 75.7–84.8] months). About 90% of GS recurrences after the first SRT were found within 6 months, though a few patients recurred more than 2 years after the first SRT. On the other hand, mean nGS‐free survival times after the first and last SRTs were not different between after the first SRT (84.4 [95%CI 73.0–90.7] months) and after the last SRT (83.1 [95%CI 74.1–92.0] months). There was no factor related to GS outcomes, but the significant factor for nGS‐free survival after the last SRT was multiple previous treatments (p=0.01, hazard ratio=15.65, 95%CI 1.79–137.16). Significance: The last SRT was almost equivalent to achieving complete disconnection of HHs from the hypothalamus according to our strategy. Considering the epileptogenic network, GS outcomes depend on complete disconnection, whereas nGS outcomes are not affected by surgical factors but independency of secondary epileptogenesis. [ABSTRACT FROM AUTHOR]

Magnetic resonance-guided laser interstitial thermal therapy (MR-gLiTT) is a novel minimally invasive treatment approach for drug-resistant focal epilepsy and brain tumors. Using thermal ablation induced by a laser diode implanted intracranially in a stereotactic manner, the technique is highly effective and safe, reducing the risk associated with more traditional open surgical approaches that could lead to increased neurological morbidity. Indications for MR-gLiTT in pediatric epilepsy surgery include hypothalamic hamartoma, tuberous sclerosis complex, cavernoma-related epilepsy, SEEG-guided seizure onset zone ablation, corpus callosotomy, periventricular nodular heterotopia, mesial temporal lobe epilepsy, and insular epilepsy. We review the available literature on the topic and present our series of patients with drug-resistant epilepsy treated by MR-gLiTT. Our experience, represented by six cases of hypothalamic hamartomas, one case of tuberous sclerosis, and one case of dysembryoplastic neuroepithelial tumor, helps to confirm that MR-gLiTT is a highly safe and effective procedure for several epilepsy conditions in children. [ABSTRACT FROM AUTHOR]

Purpose: Hypothalamic hamartoma (HH), a rare congenital disorder, can cause intractable epilepsy and requires optimal surgical treatment. This study analyzed the clinical characteristics of HH and evaluated seizure outcomes and the safety of gamma-knife radiosurgery (GKS) in HH-related epilepsy to propose an optimal surgical treatment. Methods: We reviewed the medical records of 18 patients with HH treated at Samsung Medical Center (1997 to 2018), and analyzed their presenting symptoms, brain magnetic resonance imaging (MRI) findings, treatment, and response. Results: The median diagnostic age was 3.2 years. The first presenting symptom was a seizure in six (33.3%), precocious puberty in five (27.8%), both symptoms in six (33.3%), and no symptoms in one patient who was diagnosed incidentally on brain MRI. All mixed and intrahypothalamic types except one had seizures (n=12), while all five parahypothalamic types presented only precocious puberty. Eleven patients showed intractable epilepsy with medications and underwent surgical treatment (most commonly GKS). Eight patients underwent GKS, and two of them received repeated GKS for recurrent seizures. Six patients showed improved seizure control with GKS as the last treatment. Among them, two patients became seizure-free, and one patient had a decreased frequency of seizures after a single GKS. There was no adverse effect related to GKS. Conclusion: Intractable epilepsy was the most common indication for surgical treatment of pediatric HH. GKS was effective in controlling seizures in 75% of HH patients without any adverse effects. Repeated GKS could also be considered as a safe option for intractable and disabling seizures. [ABSTRACT FROM AUTHOR]

Hypothalamic hamartomas are aberrant masses, composed of abnormally distributed neurons and glia. Along endocrine and cognitive symptoms, they may cause epileptic seizures, including the specific gelastic and dacrystic seizures. Surgery is the treatment of drug-resistant hamartoma epilepsy, with associated positive results on endocrine, psychiatric, and cognitive symptoms. Recently, alternatives to open microsurgical treatment have been proposed. We review these techniques and compare their efficacy and safety. Open resection or disconnection of the hamartoma, either through pterional, transcallosal, or transventricular approach, leads to good epileptological control, but its high complication rate, up to 30%, limits its indications. The purely cisternal peduncular forms remain the only indication of open, pterional approach, while other strategies have been developed to overcome the neurological, endocrine, behavioral, or cognitive complications. Laser and radiofrequency thermocoagulation-based disconnection through robot-guided stereo-endoscopy has been proposed as an alternative to open microsurgical resection and stereotactic destruction. The goal is to allow safe and complete disconnection of a possibly complex attachment zone, through a single intraparenchymal trajectory which allows multiple laser or radiofrequency probe trajectory inside the ventricle. The efficacy was high, with 78% of favorable outcome, and the overall complication rate was 8%. It was especially effective in patients with isolated gelastic seizures and pure intraventricular hamartomas. Stereotactic radiosurgery has proved as efficacious and safer than open microsurgery, with around 60% of seizure control and a very low complication rate. Multiple stereotactic thermocoagulation showed very interesting results with 71% of seizure freedom and 2% of permanent complications. Stereotactic laser interstitial thermotherapy (LiTT) seems as effective as open microsurgery (from 76 to 81% of seizure freedom) but causes up to 20% of permanent complications. This technique has however been highly improved by targeting only the epileptogenic onset zone in the hamartoma, as shown on preoperative functional MRI, leading to an improvement of epilepsy control by 45% (92% of seizure freedom) with no postoperative morbidity. All these results suggest that the impact of the surgical procedure does not depend on purely technical matters (laser vs radiofrequency thermocoagulation or stereotactic vs robot-guided stereo-endoscopy) but relies on the understanding of the epileptic network, including inside the hamartoma, the aim being to plan an effective disconnection or lesion of the epileptogenic part while sparing the adjacent functional structures. [ABSTRACT FROM AUTHOR]

Introduction: Hypothalamic hamartoma is rarely associated with epileptic spasms. We describe epileptic spasms in a large cohort of hypothalamic hamartoma patients. Methods: We performed a retrospective chart review between March 2011 and March 2020 to identify patients with hypothalamic hamartoma and epilepsy. Results: We identified 114 patients with hypothalamic hamartoma and epilepsy, only 3 male patients (2.6%) also had epileptic spasms. The epileptic spasms developed between 6 and 18 months of age. Epileptic spasms resolved with oral prednisolone in 1 and with vigabatrin in the second patient. The third patient continued epileptic spasms despite multiple antiepileptic drugs and partial resection of hypothalamic hamartoma. All 3 patients underwent laser-ablation of hypothalamic hamartoma at the age of 14, 29, and 63 months. The seizure burden decreased by 100%, 84%, and 93% at follow-up (3-47 months). Conclusions: Epileptic spasms are rare in hypothalamic hamartoma patients and early laser-ablation could potentially treat epileptic spasms and all other seizure types associated with hypothalamic hamartoma. [ABSTRACT FROM AUTHOR]

Objective: Magnetic resonance imaging (MRI)–guided laser interstitial thermal therapy (MRg‐LITT) is an alternative to open epilepsy surgery. We assess safety and effectiveness of MRg‐LITT for extratemporal lobe epilepsy (ETLE) in patients who are considered less favorable for open resection. Methods: We retrospectively reviewed sequential cases of patients with focal ETLE who underwent MRg‐LITT between 2012 and 2019. Epileptogenic zones were determined from standard clinical and imaging data ± stereoelectroencephalography (SEEG). Standard stereotactic techniques, MRI thermometry, and a commercial laser thermal therapy system were used for ablations. Anatomic MRI was used to calculate ablation volumes. Clinical outcomes were determined longitudinally. Results: Thirty‐five patients with mean epilepsy duration of 21.3 ± 12.2 years underwent MRg‐LITT for focal ETLE at a mean age 36.4 ± 12.7 years. A mean 2.59 ± 1.45 trajectories per patient were used to obtain ablation volumes of 8.8 ± 7.5 cm3. Mean follow‐up was 27.3 ± 19.5 months. Of 32 patients with >12 months of follow‐up, 17 (53%) achieved good outcomes (Engel class I + II) of whom 14 (44%) were Engel class I. Subgroup analysis revealed better outcomes for patients with lesional ETLE than for those who were nonlesional, multifocal, or who had failed prior interventions (P =.02). Of 13 patients showing favorable seizure‐onset patterns (localized low voltage fast activity or rhythmic spiking on SEEG) prior to ablation, 9 (69%) achieved good outcomes, whereas only 3 of 11 (27%) who show other slower onset patterns achieved good outcomes. Minor adverse events included six patients with transient sensorimotor neurologic deficits and four patients with asymptomatic hemorrhages along the fiber tract. Major adverse events included one patient with a brain abscess that required stereotactic drainage and one patient with persistent hypothalamic obesity. Three deaths—two seizure‐associated and one suicide—were unrelated to surgical procedures. Significance: MRI‐guided laser interstitial thermal therapy (or MRg‐LITT) was well‐tolerated and yielded good outcomes in a heterogeneous group of ETLE patients. Lesional epilepsy and favorable seizure‐onset patterns on SEEG predicted higher likelihoods of success. [ABSTRACT FROM AUTHOR]

Surgical treatment of deep or difficult to access lesions represents a unique and significant challenge for pediatric neurosurgeons. The introduction of stereotactic magnetic resonance-guided laser interstitial thermal therapy (LITT) over the last decade has had a dramatic impact on the landscape of pediatric neurosurgery. LITT provides a safe and effective option for children with epilepsy from hypothalamic hamartoma that represents a ground-breaking new therapy for a condition which was historically very difficult to treat with previous neurosurgical techniques. LITT has also been used as an alternative surgical technique for mesial temporal sclerosis, focal cortical dysplasia, MR-negative epilepsy, cavernoma-related epilepsy, insular epilepsy, and corpus callosotomy among other epilepsy etiologies. In some cases, LITT has been associated with improved cognitive outcomes compared to standard techniques, as in mesial temporal lobe epilepsy. Initial experiences with LITT for neuro-oncologic processes are also promising. LITT is often attractive to patients and providers as a minimally invasive approach, but the differences in safety and clinical outcome between LITT and traditional approaches are still being studied. In this review, we examine the emerging indications and clinical evidence for LITT in pediatric neurosurgery. [ABSTRACT FROM AUTHOR]

Background: Our aim was to identify the preoperative factors associated with a greater risk of poor inpatient outcomes in those undergoing resection of hypothalamic hamartomas. Methods: We performed a multi-institutional retrospective cohort analysis via the Nationwide Inpatient Sample (1998 – 2007). Patients of any age who underwent resection of hypothalamic hamartomas were identified by ICD-9 coding. The primary outcomes included inpatient complications, length of stay (LOS), and total charges. Multivariate regression models were constructed to analyze the outcomes. Results: Two hundred and eighty-two patients were identified with a mean age of 27.7 years, with most being male (53.2%), Caucasian (78.9%), privately insured (69.3%), and treated electively (74.7%) at academic centers (91.7%). A majority (82.2%) had Elixhauser comorbidity scores of < 1, indicating few comorbidities. No inpatient deaths were reported. Mean LOS was 7.39 days and the mean total hospital charges were $53,935. Overall, 19.5% developed an inpatient complication, primarily stroke (16.7%). Female gender, ethnic / racial minorities, higher comorbidity scores, private insurance, and non-academic hospitals were associated with greater LOS and total charges. Private insurance (Odds Ratio, OR: 1.59, P = 0.045) and academic hospitals (OR: 1.43, P = 0.008) were associated with significantly higher odds of any complication. Minority race / ethnicity was associated with a minimal increase in the odds of postoperative stroke (OR: 1.02, P < 0.001) relative to Caucasians. Conclusions: Through an analysis of a 10-year multi-institutional database, we have described the surgical outcomes of patients undergoing resection of hypothalamic hamartomas. Results demonstrate significant inpatient morbidity, particularly postoperative stroke. Patient- and institution-level factors should be considered in determining the perioperative risk for such patients. [ABSTRACT FROM AUTHOR]

Background: The congenital simple hamartoma of the retinal pigment epithelium is a benign lesion and previous observations with noninvasive imaging have detected potential photoreceptor abnormalities and retinal function interplay. Case presentation: A 35-year-old woman was found to have an asymptomatic, solitary, circumscribed, pigmented lesion in her left eye. The patient underwent ophthalmic examination including multimodal evaluation with fluorescein angiography, near-infrared reflectance scanning laser ophthalmoscopy, blue autofluorescence, enhanced-depth imaging spectralis B-scan optical coherence tomography (EDI-SBOCT), en face OCT angiography (OCT-A) and microperimetry plus adaptive optics imaging. Ophthalmoscopic examination revealed a juxtafoveolar pigmented lesion with feeding retinal arteriole, consistent with congenital simple hamartoma of RPE. There was no macular edema, exudation, hemorrhage, traction or subretinal fluid. Multimodal imaging of the mass using fluorescein angiography revealed intra-lesion late staining, near-infrared reflectance imaging demonstrated intrinsic hyperreflectivity, short-wavelength autofluorescence and red-free filter photography revealed blocked signal, and SBOCT showed abrupt shadowing. On OCT-A, an exclusive ring-shaped vascular circuit with increased foveal avascular zone was noted. Adaptive optics revealed cell density arrangement and retinal sensitivity correlations on microperimetry. Conclusion: These findings suggest that this hamartomatous lesion might cause specific cellular changes that impact retinal sensitivity response and potentially result from vasculature malnourishment to the outer retinal layers. [ABSTRACT FROM AUTHOR]