Coexistence of acromegaly and raised intracranial pressure: insights from a rare case report and literature review

Abstract

Background

Idiopathic intracranial hypertension (IIH) is a rare condition characterized by increased cerebrospinal fluid pressure without an intracranial mass, mainly affecting overweight women of reproductive age. It commonly presents with headache, visual disturbance, and papilledema. Acromegaly is an uncommon endocrine disorder caused by a growth hormone–secreting pituitary adenoma.

Case presentation

A 24-year-old female presented to an ophthalmology clinic complaining of blurred vision and generalized headache. Clinical examination revealed coarse facial features and a deep voice, raising suspicion of an underlying endocrine disorder. Fundoscopic examination showed bilateral papilledema (grade II in the right eye, grade I in the left). Laboratory investigations showed elevated IGF-1 (508 ng/mL) and unsuppressed GH levels during an oral glucose tolerance test. Brain magnetic resonance imaging (MRI) demonstrated a 1.5 × 1.3 cm pituitary adenoma extending into the left cavernous sinus. These results led to the diagnosis of acromegaly with a pattern of raised intracranial pressure resembling IIH. Notably, headache and visual problems continued after surgery, requiring acetazolamide medication (250 mg twice daily) to control intracranial pressure.

Conclusion

Even in the absence of optic chiasm compression, papilledema and symptoms of raised intracranial pressure can occur in patients with acromegaly. Management of IIH generally aims to reduce intracranial pressure through weight optimization, medical therapy, or surgical intervention when indicated.

Introduction

Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri [1], is a syndrome caused by increased cerebrospinal fluid (CSF) pressure in the absence of an intracranial mass on neuroimaging [2]. This differs from secondary intracranial hypertension caused by intracranial lesions such as tumors, hemorrhage, infarction, and venous sinus thrombosis [3]. It is an uncommon condition with an annual incidence of 0.5-2 per 100,000 persons, affecting primarily women aged 15 to 45 and those who are overweight or obese [4]. Numerous systemic conditions, including Addison’s disease, hypoparathyroidism, and anaemia, as well as drugs like corticosteroids, vitamin A, and tetracyclines, are linked to IIH [5]. Headaches, diplopia, pulsating tinnitus, visual loss, and cognitive impairment are some of the usual symptoms of IIH [6]. Acromegaly is also a rare condition that results from a pituitary adenoma and causes a continuous overproduction of both growth hormone (GH) and insulin-like growth factor-1 (IGF-1) [7]. The diagnosis is supported by an oral glucose tolerance test (OGTT) with an unsuppressed GH result ≥ 1 ng/mL [8].

This report presents an uncommon case of a 24-year-old woman who presented with blurred vision, papilledema, and elevated intracranial pressure coexisting with acromegaly.

This case is unusual because, beyond the typical features of acromegaly, it also involves significant neurological manifestations related to elevated intracranial pressure, which is extremely rare. The presence of both conditions in one patient has been documented only a handful of times worldwide, making the diagnosis and treatment more difficult for clinicians. It pushes clinicians to look past the usual picture of acromegaly and consider how the neurological symptoms may occur concurrently with the underlying endocrine disorder.

Case presentation

A 24-year-old female patient visited our ophthalmology clinic with the main complaint of blurred vision and a generalized headache. She initially sought medical attention for refractive assessment and possible laser vision correction. During clinical evaluation, coarse facial features and a deep voice were noted, raising suspicion of an underlying endocrine disorder. She did not have a history of cold intolerance, digestive problems, polyuria, urine retention, orthostatic hypotension, galactorrhea, nausea or vomiting, or stomach discomfort. Her menstrual cycle was regular. In addition, no medication history was reported. The range of her vital signs was normal. Her body mass index (BMI) was 27.5 kg/m², and her weight was 75 kg. Fundoscopic examination revealed bilateral papilledema grade I in the left eye and grade II in the right eye, while automated visual field testing demonstrated an enlarged blind spot and peripheral constriction bilaterally, which is consistent with papilledema (Fig. 1). The patient was referred to the endocrinology clinic for further evaluation. Initial laboratory investigations, including complete blood count and metabolic profile, were largely within normal limits, as summarized in Table 1. Hormonal tests showed IGF-1 508 ng/mL (borderline high), TSH 0.72 µIU/mL, free T4 1.04 ng/dL, prolactin 6.8 ng/mL, morning cortisol 8.8 µg/dL (repeat measurement performed under standardized conditions yielded 18 µg/dL, ruling out adrenal insufficiency), and ACTH 38.9 pg/mL. An overnight 1-mg dexamethasone suppression test showed inadequate suppression (cortisol 2.7 µg/dL, against a cutoff of < 1.8 µg/dL). However, to account for a potential false-positive result, a subsequent 24-hour urinary free cortisol test was performed and was within normal limits, safely ruling out Cushing’s syndrome (Tables 2 and 3). GH secretion was also assessed using a standard 75-g OGTT and measured by chemiluminescent immunoassay (CLIA): Fasting GH: 10 ng/mL (normal < 1.0 ng/mL), GH after 60 min: 18 ng/mL, GH after 90 min: 15 ng/mL, GH after 120 min: 13.5 ng/mL. Failure of GH suppression below these thresholds, in conjunction with elevated age-adjusted IGF-1, is diagnostic of acromegaly. Thyroid ultrasound showed small benign-appearing nodules, with no relevance to the diagnostic work-up. Brain MRI demonstrated a pituitary adenoma measuring 1.5 × 1.3 cm located within the sella turcica, extending into the left cavernous sinus. The lesion showed marked enhancement following intravenous gadolinium administration. The optic chiasm was intact without evidence of compression, although there was mild rightward deviation of the pituitary stalk (Fig. 2). MR venography (MRV) was not performed; however, MRI showed no radiological signs suggestive of venous sinus thrombosis. A neurological consultation was obtained, which also confirmed bilateral papilledema and recommended further evaluation to rule out raised intracranial pressure. Based on the multidisciplinary decision, an endoscopic transsphenoidal resection of the tumor was performed. At 8:00 AM after surgery, cortisol levels were 13.1 µg/dL. Histopathological examination of the resected sellar mass confirmed a pituitary adenoma. Microscopy showed a uniform population of pituitary cells with stippled chromatin and inconspicuous nucleoli. The best-corrected visual acuity (BCVA) improved to 6/10 in both eyes with spectacles. Despite surgery, the patient continued to experience headache and visual disturbance. Persistent grade II papilledema in the right eye and grade I papilledema in the left eye were detected by repeated fundus examination. A lumbar puncture was performed with the patient in the lateral decubitus position and showed: Opening: 30 cm H₂O, color: Clear, red blood cells: 5/mm³, white blood cells: 3/mm³, glucose: 71 mg/dL, protein: 21 mg/dL. In light of all the preceding findings, a diagnosis of acromegaly with coexisting raised intracranial pressure (ICP) showing an IIH-like pattern was made, as the pituitary macroadenoma did not demonstrate imaging features that would account for elevated ICP (Table 4). Acetazolamide 250 mg twice daily was prescribed as therapy for intracranial pressure control. The patient was unfortunately lost to follow-up after surgery, and postoperative GH and IGF-1 levels could not be obtained (Fig. 3).

Fig. 1.

Automated computerized visual field testing demonstrating an enlarged blind spot and peripheral constriction in the left (A) and right (B) eyes before surgery

Table 1.

Blood test results

Test	Result	Normal Range
WBC	5.6 × 10³/µL	4–10 × 10³/µL
Hemoglobin	12.6 g/dL	12–15 g/dL
Platelets	176 × 10³/µL	150–400 × 10³/µL
Glucose	102 mg/dL	70–99 mg/dL
Urea	28 mg/dL	15–45 mg/dL
Triglycerides	152 mg/dL	< 150 mg/dL
Cholesterol	195 mg/dL	< 200 mg/dL
Potassium	4.2 mmol/L	3.5–5.1 mmol/L

Table 2.

Hormonal tests results

Test	Result	Normal Range
IGF-1	508 ng/mL	80–500 ng/mL
TSH	0.72 µIU/mL	0.4–4.5 µIU/mL
Free T4	1.04 ng/dL	0.8–1.8 ng/dL
Prolactin	6.8 ng/mL	5–25 ng/mL

Table 3.

Adrenal axis

Test	Result	Normal Range
Cortisol 8 AM	8.8 µg/dL (repeat: 18 µg/dL)	5–25 µg/dL
ACTH	38.9 pg/mL	10–60 pg/mL
Cortisol post-Dexa	2.7 µg/dL	< 1.8 µg/dL
24-hour Urine Cortisol	Normal	10–50 µg/24 h

Fig. 2.

Pre-operative T1-weighted MRI demonstrating a 1.5 × 1.3 cm pituitary adenoma within the sella turcica, with left cavernous sinus extension and mild rightward deviation of the pituitary stalk

Table 4.

Descriptive application of Friedman criteria to illustrate the pattern of raised intracranial pressure in the context of a coexisting pituitary macroadenoma

Friedman Diagnostic Criteria for IIH [9]	Finding in This Patient
A. Papilledema	Bilateral papilledema (Grade II right, Grade I left).
B. Normal neurological examination	No neurological deficits reported.
C. Normal neuroimaging	MRI revealed a 1.5 × 1.3 cm pituitary adenoma, but with no significant mass effect, no midline shift, and no obstructive hydrocephalus that could account for the raised intracranial pressure. Venous thrombosis was excluded.
D. Normal CSF composition	Clear CSF; WBC 3/mm³; RBC 5/mm³; glucose 71 mg/dL; protein 21 mg/dL.
E. Elevated opening pressure ≥ 25 cm H₂O	Opening pressure: 30 cm H₂O.

Fig. 3.

Graphical summary of the patient’s diagnostic pathway

Discussion

Increased intracranial pressure without cerebral mass lesions or disruption of the cerebrospinal fluid outflow is known as idiopathic intracranial hypertension. A wide range of underlying conditions, such as cerebral venous thrombosis, hydrocephalus, and intracranial mass lesions, should be kept in mind for differential diagnosis [10]. IIH is also commonly linked to endocrine diseases including hypoparathyroidism and hormone changes brought on by the use of GH or steroids, as in our case, the patient had acromegaly which is known to result from high concentrations of GH [11]. For the Middle East, considering that Syria is part of it, the incidence of IIH among its population was estimated at about 2.02–2.2 per 100,000 annually, which is higher than the rates recorded in Western countries. This calls for more studies in the Middle East, especially in developing countries, to gain a better understanding of its pathological mechanisms and perhaps discover more helpful treatment methods [12]. The most common symptoms are a throbbing headache that is typically accompanied by nausea and vomiting brought on by straining, coughing, or sneezing [13]. Our patient presented with blurred vision and headache secondary to papilledema; however, she did not experience nausea or vomiting, similar to the case reported by Bagherzadeh et al. [14]. On the other hand, Rajabi et al.’s case showed the patient has nausea, headaches, vomiting, intracranial noises, diplopia due to sixth nerve paralysis, and transient visual obstructions [15]. Additionally, our patient was overweight, which is a recognized risk factor for IIH. In contrast, a unique case reported in the literature described IIH in a 32-week pregnant woman with a BMI below the normal range. In such situations, the increased intracranial pressure is attributed to pregnancy-related physiological changes rather than obesity. Late pregnancy is associated with elevated intra-abdominal pressure, which increases intrapleural pressure, reduces venous return from the brain, thus causing IIH [16].

Specific neuroimaging findings—MRI being the recommended method—are essential for the diagnosis of IIH. MR venography (MRV) has emerged as a crucial technique in recent years, especially for distinguishing IIH from other causes of intracranial hypertension [11]. Wall et al., in the multicenter study, showed that acetazolamide improves vision and reduces papilledema in idiopathic intracranial hypertension compared to placebo [17]. Their work established acetazolamide as the first‑line treatment, especially when vision is affected. The usual starting dose of acetazolamide is 500–1000 mg daily; however this can be adjusted based on the patient’s tolerance and clinical response [11]. In the report by Olson et al., a woman with classic acromegalic features was found to have a pituitary adenoma associated with papilledema and raised intracranial pressure without direct chiasmal compression. Surgery and acetazolamide led to full recovery and normalization of hormones [18]. In our case, the papilledema persisted in the patient even after pituitary surgery, which is consistent with a recently published retrospective study. It indicated that the resolution of papilledema in IIH cases may be delayed in some patients, as the average time for papilledema resolution was approximately 7.9 months, and 25% of patients experienced a recurrence about 4.5 months after the initial improvement [19]. This confirms that the dynamics of papilledema are complex and do not respond directly to therapeutic intervention. Therefore, through our report, we emphasize the need for long-term ophthalmologic monitoring and follow-up even after appropriate surgical management and initiation of acetazolamide therapy. In a study by King et al., the patient presented with an empty sella, papilledema, and intermittent headaches; therefore, the treatment focus was on ventriculoperitoneal shunting (VPS) prior to endoscopic transsphenoidal tumor resection [20]. Up to 75% of individuals with acromegaly might have hyperostotic skeletal abnormalities [21]. According to our case, we noted coarse facial features and a deep voice during clinical evaluation. Following a comprehensive physical examination, radiological investigation, and biochemical tests, the diagnosis of acromegaly is established. The typical diagnostic procedure involves measuring the GH response to a glucose load [22]. A published study by Koobatian et al. demonstrated that the majority of patients with acromegaly received depot somatostatin receptor ligand (SRL) injections either as monotherapy or in combination with other therapies [23]. One hypothesis addressing the potential link between IIH and acromegaly involves the direct influence of GH and IGF-1 within the central nervous system. Growth hormone is known to cross the blood–brain barrier (BBB), leading to elevated CSF levels and stimulating local IGF-1 production. This, in turn, activates IGF-1 and IGF-2 receptors in the choroid plexus, potentially increasing cerebrospinal fluid production and raising the likelihood of developing IIH [18, 24]. However, in our case, this hormonal mechanism cannot be supported, as postoperative GH and IGF-1 levels were unavailable. Therefore, the persistence of papilledema after surgery cannot be linked to hormonal status.

Conclusion

Papilledema and symptoms of raised intracranial pressure can occur in patients with acromegaly, even in the absence of optic chiasm compression. Although surgical resection addressed the pituitary adenoma, the persistence of papilledema highlights the need for long-term ophthalmologic monitoring. This case was evaluated and managed by a multidisciplinary team of neurologists, endocrinologists, and surgeons, highlighting the need for a multidisciplinary approach in similar cases to ensure patient safety. Documenting such cases contributes to the advancement of scientific knowledge, guides future practices in this area, and opens doors for international research and clinicians to further understand the pathophysiological mechanisms underlying these complications and to develop effective treatment strategies.

Limitations

MRV was not performed in our case due to limited availability at the time of evaluation; however, a detailed review of the brain MRI by the neurology team confirmed the absence of radiological signs of venous sinus thrombosis or stenosis. Endocrine follow-up was limited, as the patient did not return for scheduled postoperative hormonal evaluation; therefore, postoperative GH and IGF-1 levels were unavailable, and biochemical remission of acromegaly could not be confirmed.

Authors’ contributions

H.H. and Y.M. led the conceptualization, clinical interpretation, and overall coordination of the work. S.O., M.N.H., E.A., M.A.M., Sh.O., and M.Q. contributed to data acquisition, patient evaluation, and literature retrieval. A.R.H., R.R., L.A., N.S., and O.A. participated in data organization, figure preparation, and verification of clinical information. M.S. and A.A.A. contributed to the neurological and endocrinological interpretation of the case and critically revised the manuscript for important intellectual content. O.A. and M.S. assisted in refining the diagnostic reasoning and ensuring accuracy of the clinical narrative. All authors contributed to drafting or revising sections of the manuscript, approved the final version, and meet all four ICMJE authorship criteria, including substantial contribution to the work and accountability for its content.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Data availability

No datasets were generated or analysed during the current study.

Declarations

Ethics approval and consent to participate

Not applicable. As it’s a case report, it is exempted from ethical approval by local institution responding on the case.

Consent for publication

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Competing interests

The authors declare no competing interests.