scholarly journals Reproductive Axis after Discontinuation of Gonadotropin-Releasing Hormone Analog Treatment of Girls with Precocious Puberty: Long Term Follow-Up Comparing Girls with Hypothalamic Hamartoma to Those with Idiopathic Precocious Puberty

1999 ◽  
Vol 84 (1) ◽  
pp. 44-49 ◽  
Author(s):  
Penelope P. Feuillan ◽  
Janet V. Jones ◽  
Kevin Barnes ◽  
Karen Oerter-Klein ◽  
Gordon B. Cutler
2013 ◽  
Vol 11 (6) ◽  
pp. 630-634 ◽  
Author(s):  
Yoshua Esquenazi ◽  
David I. Sandberg ◽  
Harold L. Rekate

Hypothalamic hamartomas (HHs) are benign lesions that are often associated with central precocious puberty and may present with gelastic seizures. Treatment modalities for HH include medical therapy with long-term gonadotropin-releasing hormone analogs or resection. The authors report the case of a 7-year-old girl who was diagnosed with an HH due to precocious puberty and was treated medically with a gonadotropin-releasing hormone analog for 3 years. Despite normalization of her plasma levels of luteinizing hormone, follicle-stimulating hormone, and estradiol and arrest of her precocious puberty, the patient developed progressive weight gain associated with extreme hyperphagia and morbid obesity by the age of 10 years. Her compulsive eating patterns were refractory to counseling and other interventions attempted by her parents and physicians. After resection of the HH, her hyperphagia resolved and her weight stabilized. To the authors' knowledge, this is the first report describing resection of an HH for the purpose of treating hyperphagia and obesity.


2014 ◽  
Vol 14 (1) ◽  
pp. 101-107 ◽  
Author(s):  
Sunil Manjila ◽  
Timothy W. Vogel ◽  
Yunwei Chen ◽  
Mark S. Rodgers ◽  
Alan R. Cohen

Hypothalamic hamartomas (HHs) are rare developmental lesions arising from the inferior hypothalamus that may cause gelastic seizures and central precocious puberty. Cystic changes in HHs are rare, usually occurring in giant lesions. The authors describe an unusual case of cystic HH masquerading as a suprasellar arachnoid cyst in an 18-month-old girl presenting with precocious puberty. Microsurgical removal of the lesion led to complete resolution of the precocious puberty on long-term follow-up. This case is the first reported HH with pathological demonstration of corticotropin-releasing hormone immunostaining in the solid tumor and glial cells in the cyst wall of the lesion. The clinical and radiological characteristics of HHs are reviewed, along with the unique surgical strategies used to manage cystic lesions in the suprasellar region.


1996 ◽  
Vol 63 (1) ◽  
pp. 47-52 ◽  
Author(s):  
A.R. Jeyarajah ◽  
C.J. Gallagher ◽  
P.R. Blake ◽  
D.H. Oram ◽  
M. Dowsett ◽  
...  

2013 ◽  
Vol 12 (2) ◽  
pp. 151-154 ◽  
Author(s):  
Chun-de Li ◽  
Shi-qi Luo ◽  
Jian Gong ◽  
Zhen-yu Ma ◽  
Ge Jia ◽  
...  

Hypothalamic hamartoma (HH) is a rare condition that often manifests as central precocious puberty (CPP). There is a lack of information available concerning the long-term effectiveness of surgery for the treatment of CPP due to HH. Here the authors describe 3 cases of CPP due to HH, with a follow-up ranging from 9 to 11 years after surgery. Three girls experienced breast growth and menses at 5–18 months of age and 5–36 months of age, respectively. Serum concentrations of luteinizing hormone, follicle-stimulating hormone, and estradiol concentrations ranged from 2.5 to 6.5 mIU/ml, 4.8–5.9 mIU/ml, and 47.9–133.0 pg/ml, respectively. Magnetic resonance imaging confirmed that CPP was caused by HH. Lesions were resected using a right pterional approach. After surgery, endocrine hormone concentrations were normalized, breasts shrunk, and menses ceased in each patient. Moreover, all of them subsequently developed normally and experienced age-appropriate onset of puberty. Each patient's height and weight were normal at the most recent follow-up (9–11 years after surgery), and none had experienced learning difficulties. Central precocious puberty due to HH can be successfully treated with resection. In the 3 cases presented, this approach was associated with both short- and long-term efficacy.


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