Adib A. Abla, Scott D. Wait, Jonathan A. Forbes, Sandipan Pati, Roger E. Johnsonbaugh, John F. Kerrigan and Yu-Tze Ng
In this paper, the authors' goal was to describe the occurrence of alternating hypernatremia and hyponatremia in pediatric patients who underwent resection of hypothalamic hamartomas (HHs) for epilepsy. Hypernatremia in patients after pituitary or hypothalamic surgery can be caused by diabetes insipidus (DI), whereas hyponatremia can occur due to a syndrome of inappropriate antidiuretic hormone, cerebral salt wasting, or excessive administration of desmopressin (DDAVP). The triphasic response after surgery in the pituitary region can also explain variations in sodium parameters in such cases.
One hundred fifty-three patients with HH who underwent surgery were enrolled in a prospective study to monitor outcomes. Of these, 4 patients (2.6%) were noted to experience dramatic alterations in serum sodium values. The medical records of these patients were identified and evaluated.
Patients' ages at surgery ranged from 1.2 to 6.0 years. All patients were girls. Two patients had Delalande Type IV lesions (of 16 total Type IV lesions surgically treated) and 2 had Type III lesions (of 39 total Type III lesions). All patients had a history of gelastic seizures refractory to medication. Seizure frequency ranged from 3 to 300 per day. After surgery, all patients experienced hypernatremia and hyponatremia. The largest fluctuation in serum sodium concentration during hospitalization in a single patient was 53 mEq/L (range 123–176 mEq/L). The mean absolute difference in maximum and minimum sodium values was 38.2 mEq/L.
All patients exhibited an initial period of immediate DI (independent of treatment) after surgery followed by a period of hyponatremia (independent of treatment), with a minimum value occurring between postoperative Days 5 and 8. All patients then returned to a hypernatremic state of DI, and 3 patients still require DDAVP for DI management. A second occurrence of hyponatremia lasting several days without DDAVP administration occurred in 2 patients during their hospitalization between periods of hypernatremia. One patient stabilized in the normal range of sodium values prior to discharge from rehabilitation without the need for further intervention. At last follow-up, 3 patients are seizure-free.
Severe instability of sodium homeostasis with hypernatremia and hyponatremia is seen in up to 2.6% of children undergoing open resection of HH. This risk appears to be related to HH type, with a higher risk for Types III (2 [5.1%] of 39) and IV (2 [12.5%] of 16) lesions. Here, the authors describe alternating episodes of hypernatremia and hyponatremia in the postoperative period following HH surgery. Management of this entity requires careful serial assessment of volume status and urine concentration and will often require alternating salt replacement therapy with DDAVP administration.
Adib A. Abla, Andrew G. Shetter, Steve W. Chang, Scott D. Wait, David G. Brachman, Yu-Tze Ng, Harold L. Rekate and John F. Kerrigan
The authors present outcomes obtained in patients who underwent Gamma Knife surgery (GKS) at 1 institution as part of a multimodal treatment of refractory epilepsy caused by hypothalamic hamartomas (HHs).
Between 2003 and 2010, 19 patients with HH underwent GKS. Eight patients had follow-up for less than 1 year, and 1 patient was lost to follow-up. The 10 remaining patients (mean age 15.1 years, range 5.7–29.3 years) had a mean follow-up of 43 months (range 18–81 months) and are the focus of this report. Five patients had undergone a total of 6 prior surgeries: 1 transcallosal resection of the HH, 2 endoscopic transventricular resections of the HH, 2 temporal lobectomies, and 1 arachnoid cyst evacuation. In an institutional review board–approved study, postoperative complications and long-term outcome measures were monitored prospectively with the use of a proprietary database. Seven patients harbored Delalande Type II lesions; the remainder harbored Type III or IV lesions. Seizure frequency ranged from 1–2 monthly to as many as 100 gelastic seizures daily. The mean lesion volume was 695 mm3 (range 169–3000 mm3, median 265 mm3). The mean/median dose directed to the 50% isodose line was 18 Gy (range 16–20 Gy). The mean maximum point dose to the optic chiasm was 7.5 Gy (range 5–10 Gy). Three patients underwent additional resection 14.5, 21, and 32 months after GKS.
Of the 10 patients included in this study, 6 are seizure free (2 after they underwent additional surgery), 1 has a 50%–90% reduction in seizure frequency, 2 have a 50% reduction in seizure frequency, and 1 has observed no change in seizure frequency. Overall quality of life, based on data obtained from follow-up telephone conversations and/or surveys, improved in 9 patients and was due to improvements in seizure control (9 patients), short-term memory loss (3 patients), and behavioral symptoms (5 patients); in 1 patient, quality of life remains minimally affected. Incidences of morbidity were all temporary and included poikilothermia (1 patient), increased depression (1 patient), weight gain/increased appetite (2 patients), and anxiety (1 patient) after GKS.
Of the approximately 150 patients at Barrow Neurological Institute who have undergone treatment for HH, the authors have reserved GKS for treatment of small HHs located distal from radiosensitive structures in patients with high cognitive function and a stable clinical picture, which allows time for the effects of radiosurgery to occur without further deterioration. The lack of significant morbidity and the clinical outcomes achieved in this study demonstrated a low risk of GKS for HH with results comparable to those of previous series.