Cushing disease is caused by excessive adrenocorticotropic hormone (ACTH) production by the pituitary adenoma. Transsphenoidal surgery is its first-line treatment. The incidence of Cushing disease in children and adolescents is so rare that long-term prognoses have yet to be made in most cases. We followed-up on a 16-year-old male Cushing disease patient who presented with rapid weight gain and growth retardation. The laboratory findings showed increased 24-hour urine free cortisol and lack of overnight cortisol suppression by low-dose dexamethasone test. The serum cortisol and 24-hour urine free cortisol, by high-dose dexamethasone test, also showed a lack of suppression, and a bilateral inferior petrosal sinus sampling suggested lateralization of ACTH secretion from the right-side pituitary gland. However, after a right hemihypophysectomy by the transsphenoidal approach, the 24-hour urine free cortisol levels were persistently high. Thus the patient underwent a total hypophysectomy, since which time he has been treated with hydrocortisone, levothyroxine, recombinant human growth hormone, and testosterone enanthate. Intravenous bisphosphonate for osteoporosis had been administered for three years. At his current age of 26 years, his final height had attained the target level range; his bone mineral density was normal, and his pubic hair was Tanner stage 4. This report describes the long-term treatment course of a Cushing disease patient according to growth profile, pubertal status, and responses to hormone replacement therapy. The clinical results serve to emphasize the importance of growth optimization, puberty, and bone health in the treatment management of Cushing disease patients who have undergone transsphenoidal surgery.
Adolescent ; Adrenocorticotropic Hormone ; Bone Density ; Child ; Dexamethasone ; Follow-Up Studies* ; Hair ; Hormone Replacement Therapy ; Human Growth Hormone ; Humans ; Hydrocortisone ; Hypophysectomy ; Hypopituitarism ; Incidence ; Male ; Osteoporosis ; Petrosal Sinus Sampling ; Pituitary ACTH Hypersecretion* ; Pituitary Gland ; Pituitary Neoplasms ; Prognosis ; Puberty ; Testosterone ; Thyroxine ; Weight Gain

OBJECTIVETo study the effect of growth hormone (GH) combined with Radix Dipsaci on the body growth and the bone mineral content (BMC) of hypophysectomized rats.

METHODSThe GH deficiency rats model was established using the hypophysectomized operation through the skull and the throat. Qualified rats were divided into the sham-operation group (n = 15), the negative control group (n = 13), the GH intervention group (n = 13), and the GH combined with Radix Dipsaci group(n = 12). GH (0.25 mg/kg) was subcutaneously injected from the cervical part in the GH intervention group and the GH combined with Radix Dipsaci group at the same time, while equal volume of normal saline was injected to the rest groups. 0.7 mL/100 kg Radix Dipsaci was given by gastrogavage to the GH combined with Radix Dipsaci group at the same time, while equal volume of normal saline was given by gastrogave to the rest groups. The body weight, the tail length, and the body length were measured during the intervention period. Blood was withdrawn after 14-day intervention. The femoral bone and the tibial bone were taken out. The levels of GH, insulin-like growth factor 1 (IGF-1), alkaline phosphatase (ALP), and osteocalcin (OC) were measured. The width of the tibial epiphyseal plate was measured. The bilateral femur bone mineral density (BMD) and BMC were measured using the dual energy X-ray absorptiometry.

RESULTSThe body weight, the body length, the length of the femoral bone, the length of the tibial bone, the width of the epiphyseal plate, the levels of the GH, IGF-1, ALP, and OC increased in the GH intervention group and the GH combined with Radix Dipsaci group after 2-week intervention, showing statistical difference when compared with the model group (P < 0.01). But there was no statistical difference in the tail length though it also increased (P > 0.05). There was insignificant difference in the aforesaid indices between the two groups (P > 0.05). Compared with the model group, the BMD of the GH combined with Radix Dipsaci group increased with statistical difference (P < 0.01). Compared with the model group, the BMC of the GH intervention group and the GH combined with Radix Dipsaci group increased with statistical difference (P < 0.01). It was highest in the GH combined with Radix Dipsaci group (P < 0.01).

CONCLUSIONSGH combined with Radix Dipsaci showed unobvious effect on promoting the growth. But it could elevate BMD and BMC, and improve the bone metabolism.

Animals ; Bone Development ; drug effects ; Bone and Bones ; metabolism ; Dipsacaceae ; chemistry ; Drugs, Chinese Herbal ; pharmacology ; Growth Hormone ; pharmacology ; Hypophysectomy ; Male ; Rats ; Rats, Sprague-Dawley

BACKGROUND AND OBJECTIVES: The aim of this study was to compare endoscopic endonasal transsphenoidal hypophysectomy (EETSA) with microscopic transseptal transsphenoidal hypophysectomy (MTTSA) in terms of clinical results during an acceleration of learning curve. SUBJECTS AND METHOD: A retrospective chart review was performed of the first 14 cases of EETSA and previous 14 cases of MTTSA. Resection results, endocrinologic outcomes, complication rates, operating time, and duration of hospital stay were investigated. A non-parametric analysis was performed to determine the significance of differences between groups. RESULTS: EETSA was performed, achieving gross total removal, based on postoperative MRI, for 12 of the 14 patients (86%) and hormonal remission for 4 of the 5 patients (80%); on the other hand, MTTSA was performed, achieving gross total removal for 9 of the 14 patients (64%) and hormonal remission for 1 of the 5 patients (20%). We found better resection results in patients who underwent EETSA than in those who did MTTSA with respect to tumors extending into suprasellar area (p<0.05). Visual improvement was achieved in all the cases with visual defect (n=5) after EETSA, whereas 4 of 6 cases (66%) were improved after MTTSA. Significant differences in complication rates, operating time and hospital stay could not be determined. All cases with CSF leakage in EETSA were successfully managed by using the nasoseptal flap. CONCLUSION: EETSA provided better resection results than MTTSA did, especially in cases extending into the suprasellar area, showing good hormonal cure and visual improvement rates. Two approaches were comparable with respect to complications during the learning curve.
Acceleration ; Endoscopes ; Hand ; Humans ; Hypophysectomy ; Learning ; Learning Curve ; Length of Stay ; Pituitary Neoplasms ; Retrospective Studies

OBJECTIVETo describe the experience with surgical treatment of pituitary adenomas via a fully transnasal endoscopic approach.

METHODSClinical records of 375 cases with pituitary adenomas underwent pure endoscopic operations between December 2006 and December 2009 were carefully assembled. Among 375 pure endoscopic operations of pituitary adenomas, 201 cases were nonfunctional adenomas and 174 cases were functional adenomas. There were 27 giant pituitary adenomas (7.2%) and 41 pituitary adenoma invaded cavernous sinus (10.9%). Intraoperative 1.5 T MRI and neuro-navigation system were used during some operations. The postoperative and follow-up data of patients were analyzed.

RESULTSThere were 234 (79.3%) cases of total resection, 56 (19.0%) cases of subtotal resection, 5 (1.7%) cases of partial resection. Sixty-eight patients had vision improved in 73 patients with vision decreasing before operation. Sixty-eight (77.3%) patients got normal endocrine in 88 hyper-prolactin patient. Fifty-five (84.1%) patients got normal growth hormone in 63 patients with somatotrophinomas. Eighteen (78.2%) patients got normal in 23 patients with corticotrophinoma. These was no death case in this group. One case (0.3%) suffered post-operative coma. Transient decreasing of vision occurred in 2 cases (0.5%). Transient palsy of oculomotor nerve or abducens nerve occurred in 7 cases of operations involved cavernous sinus. Three (0.8%) patients had cerebral meningitis. Two patients (0.5%) suffered from cerebrospinal fluid leak but none underwent operation to repair. Fourteen patients (3.7%) had transient diabetes insipidus. Six patients (1.6%) had nose bleeding.

CONCLUSIONSTrans-nasal endoscopy provides a new device for operation of pituitary adenomas which is effective and safe. Comparing with microscope, endoscopic visual field is clearer, closer and wider.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Endoscopy ; Female ; Follow-Up Studies ; Humans ; Hypophysectomy ; methods ; Male ; Middle Aged ; Nose ; surgery ; Pituitary Neoplasms ; surgery ; Retrospective Studies ; Sphenoid Sinus ; surgery ; Treatment Outcome ; Young Adult

OBJECTIVE: To recognize the causes, methods of prevention and cure of sinusitis complicated by endoscopic transnasal approach in surgical treatment of pituitary adenomas. METHOD: The data of 187 patients who underwent surgical treatments of pituitary adenomas through endoscopic transnasal approach were collected, and the surgical complications were analyzed. RESULT: Endoscopic transnasal transsphenoidal approach were used successfully in 187 cases, twelve cases (6.4%) were suffered from sinusitis complication after surgery. The most frequent sinusitis complication was sphenoiditis, 9 cases (4.8%) were suffered, 2 cases (1.1%) were suffered from ethmoidal sinusitis, 1 case (0.5%) was suffered from maxillary sinusitis. Ten cases were treated with correspond medication in clinic. Two cases were ineffective with expectant treatment, and cured by second endoscopic sinusitis operation at last. CONCLUSION Endoscopic transnasal transsphenoidal approach offered a simple and rapid access to the sella, and also, it is a safe, valuable and efficient procedure for removing pituitary adenomas. It was obviously that timely accurate diagnosis for the sinusitis complication and comprehensive perioperative management may lead to less incidence of postoperative sinusitis complication in surgical treatment of pituitary adenomas through endoscopic transnasal approach, increase the achievement rate and improve survival quality obviously.
Adult ; Aged ; Endoscopy ; Female ; Humans ; Hypophysectomy ; methods ; Male ; Middle Aged ; Nose ; surgery ; Pituitary Neoplasms ; surgery ; Postoperative Complications ; prevention & control ; Sinusitis ; prevention & control ; Sphenoid Sinus ; surgery ; Treatment Outcome

This study was designed to examine the effects of recombinant human growth hormone replacement on somatic growth and cognitive function in hypophysectomized (HYPOX) female Sprague-Dawley rats. Rats (5 per group) were randomized by weight to 3 experimental groups: group 1, administered 200 microgram/kg of GH once daily for 9 days; group 2, administered 200 microgram/kg of GH twice daily; and group 3, administered saline daily. Somatic growth was evaluated by measurement of body weight daily and of the width of the proximal tibial growth plate of the HYPOX rats. Cognitive function was evaluated using the Morris water maze (MWM) test. The results indicated that GH replacement therapy in HYPOX rats promoted an increase in the body weight and the width of the tibial growth plate in a dose-dependent manner. On the third day of the MWM test, the escape latency in the GH-treated groups 1 and 2 was significantly shorter than that in the control rats (P<0.001 and P=0.032, respectively), suggesting that rhGH improved spatial memory acquisition in the MWM test. Therefore it is concluded that rhGH replacement therapy in HYPOX rats stimulates an increase in somatic growth in a dose-dependent manner and also has beneficial effects on cognitive functions.
Animals ; Body Weight ; Female ; Growth/*drug effects ; Growth Plate/drug effects/pathology ; Human Growth Hormone/administration & dosage/*pharmacology ; Humans ; *Hypophysectomy ; Rats ; Rats, Sprague-Dawley ; Spatial Behavior/*drug effects

OBJECTIVETo analyze the transsphenoidal microsurgical results of non-invasive prolactinomas, in order to provide reference for their treatment choice.

METHODSTo review the transsphenoidal microsurgical results of 234 non-invasive prolactinomas treated in our department in recent 10 years, and to analyze the prognostic factors. There were 18 males and 216 females, aged between 13 and 58 years, averaged (31.1 +/- 8.5) years. The course ranged from half a month to 20 years, averaged (47.3 +/- 44.9) months. The preoperative serum PRL level ranged between 41 and 8406 ng/ml, averaged (400.5 +/- 888.0) ng/ml, with a median of 164.1 ng/ml. The primary symptoms were amenorrhea, galactorrhea and/or infertility in 211 cases. The tumor size was small (< 1 cm) in 100, large (> or = 1 cm) in 116 and giant (> or = 3 cm) in 18 cases. All the patients received transsphenoidal microsurgery and were followed-up for 12 to 132 months, averaged (43.8 +/- 35.0) months.

RESULTSThere was no mortality. One hundred and twenty-seven (54.3%) cases had transient postoperative imbalance of water and electrolytes. One hundred and eighty-eight cases (80.3%) were cured, 12 (5.1%) experienced clinical remission, 20 (8.5%) were improved, and 14 (6.0%) were ineffective. The male patient, the giant prolactinomas and those with higher preoperative serum PRL level had a relative poor postoperative prognosis. While the other factors had no influence on prognosis, including the course, preoperative bromocriptine intake, tumor texture, tumor apoplexy and intraoperative descending extent of the diaphragm of sella. The overall operative expense for transsphenoidal microsurgery ranged from 8323.8 to 22898.5 yuan, averaged (12912.0 +/- 2361.2) yuan.

CONCLUSIONSTranssphenoidal microsurgery may be chosen as the primary therapy for non-invasive prolactinomas, with the purposes of therapeutical efficacy, facilitating the patients, re-establishing the patients' self-confidence and reducing the overall expense.

Adolescent ; Adult ; Female ; Follow-Up Studies ; Humans ; Hypophysectomy ; methods ; Male ; Microsurgery ; Middle Aged ; Pituitary Neoplasms ; surgery ; Prolactinoma ; surgery ; Retrospective Studies ; Sphenoid Sinus ; surgery ; Treatment Outcome

Adolescent ; Adult ; Aged ; Cavernous Sinus ; injuries ; Female ; Humans ; Hypophysectomy ; adverse effects ; methods ; Intraoperative Complications ; Male ; Middle Aged ; Pituitary Neoplasms ; surgery ; Young Adult

OBJECTIVETo observe long-term outcomes of patients with invasive giant prolactinomas (IGPs) treated with bromocriptine followed by comprehensive treatments.

METHODSThirty-four patients met the criteria of IGPs were treated with bromocriptine initially. Among of them, 11 had radiotherapy at the same time. During the treatments, transsphenoidal surgery or/and Gamma Knife were considered to apply to the patients according to the location, shrinkage of residual tumors and resistance of bromocriptine. Small dosage of bromocriptine was kept after operation.

RESULTSThe average follow-up duration is 33.6 months. Thirty-three patients obtained significant improvement, but one failed recovery of vision due to side-injury by radiotherapy. Tumor volume on magnetic resonance imaging (MRI) was decreased on average by 91.4%, PRL by 97.1%. The number of patients with low testosterone level restored from 17 to 6 and hypoadrenalism from 10 to 6 after combined treatment with priority of medical therapy. Rhinorrhea occurred in 2 cases, 1 restored in two weeks, 1 had transsphenoidal combined with transcranial surgery to remove the tumor and repair the fistula.4 had resistance to bromocriptine to some extend.

CONCLUSIONSDopamine agonist medications are effective as a first-line therapy for IGPs. In some patients treated by bromocriptine only, the tumor may disappear on MRI. Combined with surgery and Gamma Knife, the duration of treatment could be shortened and the dosage may be minimized, but using radiotherapy should be cautions.

Adolescent ; Adult ; Aged ; Bromocriptine ; therapeutic use ; Combined Modality Therapy ; Female ; Follow-Up Studies ; Hormone Antagonists ; therapeutic use ; Humans ; Hypophysectomy ; Male ; Middle Aged ; Pituitary Neoplasms ; drug therapy ; surgery ; Prolactinoma ; drug therapy ; surgery ; Radiosurgery ; Retrospective Studies ; Time Factors ; Treatment Outcome

OBJECTIVETo assess the clinical curative effect of the endonasal transsphenoidal approach for removing pituitary adenoma (PA) under neuroendoscope-assisted.

METHODSThere were 215 patients who had undergone neuroendoscopic transsphenoidal surgery. Each patient received CT or MRI examination which showed the size and surrounding structural of tumor.

RESULTSAmong the 215 patients, 190 cases (88.4%) had total removal, 17 cases (7.9%) achieved subtotal removal and the remaining 8 cases (3.7%) with fibrous tumor was carried out partial removal. Two patients (0.9%) died after operation. Postoperative follow-up period was 1 to 10 months (the average was 3.5 months). In 182 patients, 150 cases (90.9%) got vision recovered rapidly compared with their preoperative symptoms, such as diminished acuities and visual field defects, and 15 cases (9.1%) had gotten improvements to some extend among 165 who diagnosed as pituitary macroadenoma (PMaA); There were 17 patients who diagnosed as microadenoma (PMiA) showed that the pituitary dyshormonism recovered gradually.

CONCLUSIONSThe endonasal transsphenoidal surgery under the neuroendoscope-assisted appears to be a safe, effective and micro-invasive method for PA.

Adenoma ; surgery ; Adolescent ; Adult ; Aged ; Female ; Follow-Up Studies ; Humans ; Hypophysectomy ; methods ; Male ; Middle Aged ; Nasal Cavity ; surgery ; Neuroendoscopy ; Pituitary Neoplasms ; surgery ; Retrospective Studies ; Sphenoid Sinus ; surgery ; Treatment Outcome