Abstract
Patients with acromegaly usually present with characteristic clinical features or comorbidities associated with excess insulinlike growth factor 1 (IGF-1)/growth hormone (GH) or may come to medical attention secondary to mass effects causing visual field distortions. Herein, we report a case of spontaneous cerebrospinal fluid (CSF) rhinorrhea as the presenting symptom of acromegaly. A 68-year-old man presented to an outside facility with a 2-day history of headache associated with nausea, vomiting, dizziness, and clear nasal discharge and underwent 2 attempted repairs of a sphenoid sinus CSF leak. Examination on admission to our hospital was significant for fluctuating level of consciousness. Subsequently, subtle coarse facial features were appreciated. Pituitary function testing showed thyrotropin and gonadotropin deficiencies along with an elevated age- and sex-matched IGF-1 of 285 (normal level, 59–225 ng/mL). Nadir GH during oral glucose tolerance test was 5.5 ng/mL and confirmed the diagnosis of acromegaly. Magnetic resonance imaging showed pneumocephalus, an enlarged sella with an elongated pituitary stalk, and partial erosion of the anterior wall of the sphenoid sinus. A distinct adenoma could not be identified. An endoscopic, transnasal, transsphenoidal exploration and biopsy with multilayered skull base reconstruction were performed. Histologic examination of the biopsy contents was consistent with a GH-producing adenoma. Postoperatively, the patient’s fluctuating level of consciousness improved and returned to baseline after his successful skull base repair. During the follow-up period, he had an IGF-1 of 713 ng/mL and started treatment with a somatostatin analogue. To our knowledge, this is the first reported case of a GH-producing pituitary adenoma presenting with spontaneous CSF rhinorrhea. Pituitary adenomas should be considered in the differential diagnosis of patients presenting with spontaneous CSF rhinorrhea with abnormal sellar image, and these patients should undergo a thorough hormonal evaluation.
1
Introduction
Acromegaly is a dysmorphic condition resulting from growth hormone (GH) excess. Elevated GH and insulinlike growth factor 1 (IGF-1) levels lead to disfigurement, cardiovascular diseases, and malignant transformation. Suspicion for acromegaly usually arises because of the recognition of the characteristic facial appearance, complaint of headache, carpal tunnel syndrome, amenorrhea, sleep apnea, or visual abnormalities. Biochemical testing confirms the diagnosis. In spite of a 32% increased risk of all-cause mortality in patients with acromegaly, there is a substantial delay of 8 to 10 years in diagnosis because of the insidious onset of the condition and subtle clinical features . We report the first case of spontaneous cerebrospinal fluid (CSF) rhinorrhea as the first symptom leading to the diagnosis of a GH-producing adenoma.
2
Case report
2.1
Presentation
A 68-year-old man presented to an outside hospital with complaints of spontaneous onset of rhinorrhea from the right nostril and headache for 2 days. The headache was debilitating and was associated with nausea, vomiting, and dizziness, which were worsening since onset. There was no history of sudden exertion, nose blowing, or recent physical activity before the onset of symptoms. He underwent 2 attempted yet unsuccessful transnasal repairs of the defect.
At the time of transfer to our facility, the patient had a fluctuating level of consciousness and a Glasgow Coma Scale score of 11 to 12. Physical examination was significant for subtle acromegalic features of the face, hands, and feet.
2.2
Investigations
Endocrinological investigations at the time of initial presentation are shown in Table 1 .
| Hormone | Test value | Reference range |
|---|---|---|
| Corticotropin stimulation test | ||
| Baseline | 13.6 | |
| 30 min | 21.3 | |
| 60 min | 22.7 | |
| IGF-1 | 285 | 59–225 ng/mL |
| GH | 9.8 | 0.0–3.0 ng/mL |
| Thyrotropin | 4.7 | 0.4–5.5 μ U/mL |
| Free thyroxine | 0.3 | 0.7–1.8 ng/mL |
| Testosterone | <10 | 220–1000 ng/dL |
| Lutenizing hormone | 2.2 | 1.5–9.3 mU/mL |
| Follicle-stimulating hormone | 1.6 | 1.0–10.0 mU/mL |
2.3
Imaging
The patient underwent computed tomographic (CT) imaging and magnetic resonance imaging (MRI) on presentation, which showed extensive pneumocephalus in addition to a large intrasellar arachnoidocele with a wide diaphragma sella. The pituitary stalk was visualized as elongated. The sella was significantly enlarged with signs of erosions especially anterior and inferior to the extent that the anterior wall of the sphenoid sinus was also partially eroded. The MRI also showed sagging of the brain, indicating intracranial hypotension. On the CT scan, there were postoperative changes secondary to the previously attempted closure ( Fig. 1 A and B).
2.4
Treatment
On presentation to our facility, the patient’s altered mental status was attributed to low intracranial pressure (ICP). To avoid uncontrolled CSF drainage, a lumbar subarachnoid drain was not inserted. Rather, we opted to place an intracranial catheter in the enlarged subdural/subarachnoid space with the goal of monitoring ICP and attempting drainage in a more controlled manner. The ICP values confirmed intracranial hypotension; and with the slightest attempt to drain, the patient had increased pneumocephalus, which confirmed the ongoing communication between the CSF spaces and the paranasal sinus cavities.
Because of the persistent signs and symptoms of a sphenoid sinus CSF leak, the patient underwent transnasal endoscopic exploration and reconstruction of the skull base. Intraoperatively, a large communication between the suprasellar space and the sella/sphenoid sinus was observed. Because of the low ICP, the frontal lobes and the optic apparatus were observed to be sagging through this opening. There was a large incompetent diaphragma sella, which provided the communication with the suprasellar space. A clear distinction between the pituitary gland, possible tumor, and sphenoid sinus mucosa could not be identified.
A biopsy was taken, and the defect was repaired in a multilayered fashion. First, dermal matrix (Duragen; Integra Neurosciences, Plainsboro, NJ) was placed against the frontal lobes and diaphragma. Next, acellular dermal allograft (Alloderm; LifeCell Corporation, The Woodlands, TX) was placed intracranially to cover the dermal matrix. A free mucosal graft taken from the nasal floor was placed to completely cover the entire skull base defect, and a resorbable polyethylene glycol dural sealant (Duraseal; Covidien, Boulder, CO) was sprayed over the reconstruction, which was then bolstered with abdominal fat and nonabsorbable polyvinyl alcohol sponges (Merocel; Medtronic Inc, Jacksonville, FL).
Permanent pathologic analysis of the biopsy specimen revealed monotonous proliferation of epithelioid cells with round nuclei in the lamina propria of respiratory-type mucosa. There was no atypia or mitoses. Immunohistochemical stains show that these cells are immunoreactive to synaptophysin, chromogranin, and GH ( Fig. 2 A –C). The final diagnosis was GH-producing pituitary adenoma.
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