|Year : 2017 | Volume
| Issue : 5 | Page : 465-467
Acromegaly presenting as obstructive sleep apnea syndrome
Deepthi Laldayal, Unnati Desai, Jyotsna M Joshi
Department of Pulmonary Medicine, TNMC and BYL Nair Hospital, Mumbai, Maharashtra, India
|Date of Submission||15-Oct-2016|
|Date of Acceptance||10-Jan-2017|
|Date of Web Publication||14-Nov-2017|
Jyotsna M Joshi
Department of Pulmonary Medicine, TNMC and BYL Nair Hospital, 2nd Floor, OPD Building, AL Nair Road, Mumbai Central, Mumbai - 400 008, Maharashtra
Source of Support: None, Conflict of Interest: None
A 52-year-old female presented with a history of snoring and excessive daytime sleepiness for 3 years. On evaluation with polysomnography, she was confirmed as a case of severe obstructive sleep apnea syndrome (OSAS). Macroglossia and enlargement of fingers and toes aroused suspicion; extending the evaluation to be ultimately confirmed as a case of acromegaly; changing the management protocols. Acromegaly, though a rare endocrine disorder which results from excessive secretion of growth hormone in adults, has a high prevalence of OSAS. We report one such rare case of acromegaly which initially presented to us as a case of OSAS.
Keywords: Acromegaly, growth hormone, obstructive sleep apnea
|How to cite this article:|
Laldayal D, Desai U, Joshi JM. Acromegaly presenting as obstructive sleep apnea syndrome. Med J DY Patil Univ 2017;10:465-7
| Introduction|| |
Obstructive sleep apnea syndrome (OSAS) is a sleep disorder characterized by multiple episodes of upper airway collapse during sleep resulting in apneas. It causes debilitating chronic medical conditions such as hypertension, insulin resistance, coronary artery disease, and can sometimes be associated with various endocrine disorders such as hypothyroidism and acromegaly. Such patients may initially manifest with complaints of OSAS. Simultaneous management of endocrinopathies is important for treatment of OSAS and its comorbidities. Acromegaly, though a rare endocrine disorder resulting from excessive secretion of growth hormone (GH) in adults shows a high prevalence of OSAS.
| Case Report|| |
A 52-year-old homemaker was referred for polysomnography (PSG) in view of complaints of snoring, excessive daytime sleepiness, irritability and memory lapses since 3 years. Her physical examination revealed nasal congestion, body mass index (BMI) of 32 kg/m 2 and widening of fingers and toes [Figure 1]. The facial features like widening of nasal bridge and thick lips were also noticed [Figure 2]. On inquiry, the patient had noticed an increase in size of her fingers and toes in the past 2 years. In view of complaints of recurrent rhinitis, atopy and raised serum immunoglobulin E suggesting allergic rhinitis; she was initiated on therapy with nasal spray of azelastine and fluticasone combination. On follow-up, the patient reported 50% improvement in symptoms. A 70° direct laryngoscopy was done which reported macroglossia. Computed tomography (CT) of paranasal sinuses (PNS) showed right maxillary sinusitis and anterior ethmoidal osteoid osteoma. Spirometry showed mild restrictive abnormality and PSG showed an apnea hypopnoea index (AHI) of 48.2/h suggestive of severe OSAS, respiratory distress index (RDI) of 48.2/h, periodic limb movement of 0/h, oxygen desaturation index (ODI) of 76.8/h, average saturation of oxygen of 91%, and minimum saturation of oxygen during sleep of 56%. On continuous positive airway pressure (CPAP) titration, the AHI and RDI were corrected to 6.4/h and ODI to 7.4/h with mean pressure of 14 cmH2O. The average saturation and minimum saturation corrected to 96% and 74%, respectively. Widening of her extremities, macroglossia and CT PNS report led us to suspect acromegaly. Her serum insulin like growth factor-1 (IGF-1) was measured and found to be 326 mcg/L (81–220 mcg/L) confirming acromegaly. Magnetic resonance imaging (MRI) of pituitary showed macroadenoma invading bilateral cavernous sinus [Figure 3]. Two-dimensional echocardiography (2D ECHO) and lipid profile were within normal limits. Apart from CPAP therapy, the patient was started on somatostatin analog (octreotide 250 mcg thrice daily subcutaneously) and was referred to a specialty center for transsphenoidal hypophysectomy.
|Figure 1: Widening of fingers, characteristic spade-like hands of acromegaly|
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|Figure 3: T1-weighted postcontrast image of magnetic resonance imaging pituitary showing pituitary macroadenoma indenting on sphenoid sinus|
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The patient underwent the surgery uneventfully and reported back. A repeat PSG showed AHI and RDI of 35.3/h and ODI of 33.8/h, and CPAP therapy was continued.
| Discussion|| |
The term acromegaly is derived from Greek word “Akras” meaning extremities and “Megas” meaning big. The condition results from excessive secretion of GH, usually from a pituitary tumor after epiphyseal closure in adults. In children, it manifests as gigantism. Estimated prevalence of acromegaly is 40–70 cases/million. Though rare, it is a potentially life-threatening condition due to associated cardiovascular comorbidities usually diagnosed late in its course of the disease. Patients usually have insidious onset of symptoms with coarse facial features, widening of the bridge of nose, prognathism, increase in shoe size, mild hirsutism, thick lips, jaw malocclusion, oily skin with large pores and carpel tunnel syndrome. In most cases, source of excess GH is pituitary, though ectopic GH and GH releasing hormone secretion from hypothalamus and nonendocrine tissues are seen. The GH stimulates production of IGF-1 from liver cells. GH along with IGF-1 is responsible for the biochemical and structural changes in an acromegalic patient.
OSAS is seen in up to 50% of patients with acromegaly. Craniofacial deformations, pharyngeal soft tissue hypertrophy, macroglossia, mucosal thickening of upper airways and altered neuromuscular control of pharyngeal muscles are the main reasons for the development of OSAS. Generalized soft tissue thickening is due to glycosaminoglycan deposition and increased collagen production and tissue edema. Central apneas are seen occasionally in acromegaly; thought to arise from the depression of respiratory center by high circulating levels of GH/IGF-1. Controversial areas are a correlation between GH/IGF-1 levels and OSAS, and correction of OSAS postremission of disease. Medical or surgical correction does not resolve symptoms of OSAS in 30% of patients. Acromegaly and OSAS have to be simultaneously treated to combat the cardiovascular risk associated with both diseases. A reevaluation with PSG is suggested in acromegaly patients after treatment of active disease. Untreated acromegaly patients have an increased mortality by 2–3-fold compared to general population. Our patient had new onset OSAS, spade-like hands and macroglossia as clinical features of acromegaly. Assessment for acromegaly was done by measuring IGF-1 levels. GH measurement randomly is not a standardized test for acromegaly. MRI of pituitary is the imaging of choice. Furthermore, 2D ECHO was performed to rule out left ventricle hypertrophy or dysfunction, which is seen in 50% of patients. Male gender, age, BMI, IGF-1 levels and disease duration have shown positive correlation with the development of OSAS. Medical treatments in acromegaly include somatostatin analogs, dopamine agonists, and GH receptor antagonists. Surgical resection is the mainstay of treatment in both pituitary micro- and macro-adenomas. Radiotherapy is a less preferred choice.
Endocrine and metabolic disorders associated with OSAS are obesity, diabetes mellitus, acromegaly, Cushing's syndrome, hypothyroidism, and polycystic ovarian disease. Obesity, metabolic syndrome, and diabetes mellitus are associated with obstructive sleep apnea independently. The prevalence of metabolic syndrome is 5–9-fold in patients with OSAS. Treatment of metabolic syndrome along with CPAP therapy is advised. Hypothyroidism is the next most common endocrinological derangement reported with OSAS. Only 2.9% of OSAS patients have concomitant hypothyroidism, but the reported prevalence of OSAS in newly diagnosed hypothyroidism along with myxedema is more than 90%. Deposition of mucopolysaccharides, proteins and associated obesity contribute to collapsibility of upper airways in hypothyroidism. These patients require CPAP therapy along with thyroxine supplementation in the presence of moderate to severe OSAS. Shipley et al. reported 45% prevalence of OSAS in Cushing's syndrome. However, studies on the effect of Cushing's syndrome therapy on OSAS is lacking. In general, the prevalence of endocrine disorders except metabolic syndrome is not very significant in OSAS to recommend a routine screening, but not missing on tell-tale clinical manifestations of hypothyroidism, acromegaly and Cushing's syndrome will be helpful in correcting an underlying disease and ultimately the better treatment of OSAS. Treatment of underlying acromegaly does improve OSAS, but in most cases requires simultaneous therapy with CPAP as in our case.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]