Secondary partial empty sella syndrome in an elite bodybuilder

Abstract

The pituitary gland is a hormone-responsive gland and is known to vary in size depending on the hormonal status of the patient snd the multifaceted positive and negative feedback hypothalamic-pituitary-gonadal axis. Partial empty sella syndrome with an atrophied pituitary gland is seen in primary neuroendocrinopathies such as growth hormone deficiency, primary hypothyroidism, central diabetes insipidus and hypogonadism. Partial empty sella has also been shown to occur in patients with elevations in intracranial pressure. Secondary partial empty sella syndrome with significant pituitary gland atrophy from negative feedback inhibition of long-term exogenous hormonal use has not been previously reported. We are reporting on a case of partial empty sella syndrome occurring in an elite bodybuilder with a long history of exogenous abuse of growth hormone, testosterone and thyroid hormone. The pathophysiologal mechanisms of secondary partial empty sella syndrome from exogenous hormone use and the possibility for elevations in intracranial pressure contributing to this syndrome will be discussed.     

Treatment of empty sella syndrome with ventriculoperitoneal shunt.

A symptomatic empty sella developed in a female patient undergoing bromocriptine therapy for microprolactinoma. Placement of a ventriculoperitoneal shunt dramatically improved the symptoms of headache and blurred vision. The post-operative imaging showed resolution of the empty sella. She was able to resume bromocriptine therapy without recurrence of her previous symptoms and give birth to a baby 20 months later. An MRI 44 months after surgery and on bromocriptine therapy showed no recurrence of the empty sella. We conclude that ventriculoperitoneal shunt may be a simple, and durable treatment for drug induced empty sella and allows resumption of bromocriptine therapy for preexisting microprolactinoma.     

Cerebrospinal fluid pressure and prolactin in empty sella syndrome

In 58 female patients with the primary empty sella (PES) syndrome, a study of the CSF dynamics was done by evaluating both the absorptive reserve by a lumbar infusion test at constant rate, and/or the ICP increase occurring during REM phase of nocturnal physiological sleep. In 33, prolactin (PRL) dynamics were also investigated evaluating both the response to sequential stimulating test with thyrotropin-releasing hormone (TRH) and metoclopramide (MCP) and/or the circadian variation of PRL levels. Impairment of CSF dynamics was found in the 84% who had a hormonal pattern characterized by an increase of the PRL response to TRH and MCP and a decrease of the PRL circadian variation. Twenty-one patients with impaired CSF dynamics underwent CSF shunting procedures with disappearance of the signs of intracranial hypertension. They also had restoration of normal PRL dynamics but the endocrine alterations improved only moderately. Altered CSF dynamics play a role in the pathogenesis of the PES syndrome. A correlation between elevated ICP and the hypothalamo-hypophyseal control of PRL secretion may exist.     

Intracranial pressure in patients with the empty sella syndrome without benign intracranial hypertension.

The intracranial pressure was monitored continuously for at least 48 hours in five patients with empty sella syndrome, who did not have clinical benign intracranial hypertension (BIH). It has been suggested that the empty sella syndrome is a result of chronically elevated intracranial pressure in the presence of a congenitally deficient diaphragma sellae. However, whilst the intracranial pressure in two of the five patients was abnormally high, in three patients in whom it was monitored, the CSF pressure was normal. Although these cases may represent "burnt out" forms of intracranial pressure problems, it might be that the normal pulsations of CSF are sufficient to produce the empty sella in the presence of a deficient diaphragma sella.     

Primary amenorrhea due to empty sella: an underestimated entity

We report a 16-year-old girl who had stunted growth and primary amenorrhea presenting with headache and vomiting. MRI of brain showed posterior fossa dermoid cyst with hydrocephalus and empty sella. Growth hormone (GH), Leutinizing hormone (LH) and Follicular stimulating hormone (FSH) were grossly reduced on endocrinological work-up. She underwent suboccipital midline craniectomy and complete excision of dermoid cyst .She had no additional deficit after the surgery. She had menarche six months after the surgery and developed adequate secondary sexual characters, however her height remains subnormal at follow-up after 2 years as compared to age matched controls. We briefly review the radiological features, pathogenesis and outcome of this underestimated entity.     

Spontaneous haemorrhage into an empty sella turcica mimicking pituitary apoplexy

We present a case of spontaneous haemorrhage into an empty sella turcica with the features of subclinical pituitary apoplexy. A 66-year-old woman with a previously resected pituitary adenoma presented four months later with progressive headache and visual deterioration. Cranial MRI demonstrated hyperacute blood products in a recurrent pituitary adenoma. Operative findings were of subacute blood in an empty sella turcica. There was no operative or subsequent histological evidence of tumour recurrence. The intrasellar haemorrhage was evacuated via a trans-sphenoidal approach, resulting in a rapid improvement in visual function. Endocrine deficits required thyroxine, corticosteroid and desmopressin supplementation. Haemorrhage into an empty sella turcica has not been previously described and needs to be suspected as a clinical entity in patients presenting with the features of pituitary apoplexy. Awareness of this clinical condition will prevent preoperative misdiagnosis.     

Gamma knife radiosurgery for GH-secreting microadenoma with empty sella.

We report a case of a growth hormone secreting microadenoma associated with empty sella treated by gamma knife radiosurgery and we evaluate the effectiveness of radiosurgery for this lesion. A 58-year-old female complained of an acromegalic appearance for 10 years. Magnetic resonance imaging revealed an enlarged sella floor with a compressed, but slightly enlarged pituitary gland. Serum GH values were 8.52 ng/ml. From these results, we diagnosed the presence of a GH-secreting pituitary microadenoma with empty sella. Stereotactic gamma knife radiosurgery was performed to treat the adenoma. The treatment dose was 30 Gy for the tumour margin (50% isodose). The patient was monitored for 5 years without any reported problem. Serum GH values were gradually decreased to 4.1 and 3.5 ng/ml at 1.6 and 3 years. Serum GH values at 5 years after radiosurgery were 1.8 ng/ml within 2 h after glucose load and serum IGF-1 was normalized. We conclude that gamma knife radiosurgery can be an useful alternative treatment modality for GH-secreting adenoma with empty sella in the selected patients when surgery is contraindicated as the primary treatment.     

侵袭性催乳素腺瘤治疗后海绵窦残留或伴空蝶鞍综合征

目的 探讨侵袭性催乳素(PRL)腺瘤经治疗后海绵窦残留伴空蝶鞍综合征的临床特征和治疗策略.方法 18例侵袭性PRL腺瘤(Knosp分级Ⅲ或Ⅳ级)经治疗后出现海绵窦肿瘤持续残留,首选药物治疗者9例,首选手术治疗结合药物和(或)放疗9例.结果 平均随访55个月,8例PRL水平正常,7例视力改善.14例因鞍内肿瘤吸收遗留空蝶鞍,7例PRL仍升高,其中5例有不同程度的视交叉下疝(P<0.05).PRL水平正常、无临床症状的7例患者采用低剂量溴隐亭治疗达到PRL水平和肿瘤体积的长期控制.结论 PRL腺瘤治疗后海绵窦肿瘤残留伴发空蝶鞍,这一现象应该引起高度重视,尤其对伴有视交叉下疝的病例,长期的密切随访是必需的.无内分泌和压迫症状的海绵窦残留病例可以采用低剂量的药物治疗达到长期的肿瘤控制和内分泌控制.     

Empty sella syndrome in childhood

The empty sella syndrome is common in middle-aged women, usually presenting with headache, and only occasionally associated with endocrine or visual abnormalities. It is rare in childhood. Childhood cases tend to present either with endocrine disturbances, visual symptoms, or with craniofacial syndromes. We present three cases of complete empty sella with childhood onset, each discovered unexpectedly during evaluation of endocrine or visual dysfunction.     

Growth hormone-secreting pituitary adenoma confined to the sphenoid sinus associated with a normal-sized empty sella.

We present a case of growth hormone (GH)-secreting ectopic pituitary adenoma confined to the sphenoid sinus associated with a normal-sized empty sella. It has been well known that acromegaly is sometimes associated with an empty sella. However, such a case usually has a macroadenoma and an empty sella that is large. The authors considered the possible mechanisms of the association between a normal-sized empty sella and an ectopic pituitary adenoma in the sphenoid sinus as the following. Primary empty sella existed originally, and the pituitary adenoma developed later. The adenoma extended into the sphenoid sinus because of the pulsatile intracranial cerebrospinal fluid pressure.     

Empty sella syndrome in nevoid basal cell carcinoma syndrome

We reported the magnetic resonance imaging of four young patients (13 to 19 years) with nevoid basal cell carcinoma syndrome (NBCCS), which showed empty sella, agenesis of the corpus callosum and empty sella, an interhemispheric lipoma with callosal dysgenesis, and an arachnoid cyst in the posterior fossa, respectively. Calcification of the diaphragma sellae, which is a protective barrier against the pulsating action of the cerebrospinal fluid, may cause the empty sella in NBCCS.     

经蝶手术治疗垂体生长激素腺瘤合并空蝶鞍综合征疗效分析

目的 探讨垂体生长激素（GH）腺瘤合并空蝶鞍综合征（ESS）的手术方法及其疗效。方法 回顾性分析2010年1月至2013年12月经蝶入路手术治疗的41例垂体GH腺瘤合并ESS患者的临床资料。所有患者术后随访1~4年,平均2.3年;空腹或随机血清GH<2.5 ng/ml,或GH葡萄糖抑制试验示GH谷值<1 ng/ml为治愈标准。结果 术后6个月治愈32例（78.0%）,未愈9例（22.0%）。随访期间32例治愈患者均未复发;9例未愈患者中,1例行放疗,其随机GH未降至正常水平、GH葡萄糖抑制试验GH谷值>1 ng/ml,其他8例患者未行特殊治疗患者中3例治愈。术后发生并发症13例（31.7%,13/41）,其中一过性电解质紊乱12例（低钾血症8例,低钠血症3例,高钠血症1例）,一过性尿崩症4例,脑膜炎1例,经积极治疗均痊愈出院。结论 经蝶手术是垂体GH腺瘤合并ESS患者的有效治疗方法。