Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 10  |  Issue : 5  |  Page : 465-467  

Acromegaly presenting as obstructive sleep apnea syndrome


Department of Pulmonary Medicine, TNMC and BYL Nair Hospital, Mumbai, Maharashtra, India

Date of Submission15-Oct-2016
Date of Acceptance10-Jan-2017
Date of Web Publication14-Nov-2017

Correspondence Address:
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
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/MJDRDYPU.MJDRDYPU_254_16

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  Abstract 


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

How to cite this URL:
Laldayal D, Desai U, Joshi JM. Acromegaly presenting as obstructive sleep apnea syndrome. Med J DY Patil Univ [serial online] 2017 [cited 2019 May 24];10:465-7. Available from: http://www.mjdrdypu.org/text.asp?2017/10/5/465/218181




  Introduction Top


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.[1] Acromegaly, though a rare endocrine disorder resulting from excessive secretion of growth hormone (GH) in adults shows a high prevalence of OSAS.


  Case Report Top


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 2: Patient showing widened nasal bridge and thick lips

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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 Top


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.[2] In children, it manifests as gigantism. Estimated prevalence of acromegaly is 40–70 cases/million.[3] 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.[2] In most cases, source of excess GH is pituitary, though ectopic GH and GH releasing hormone secretion from hypothalamus and nonendocrine tissues are seen.[4] 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.[5] 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.[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.[6] 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.[7] 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.[6] Medical treatments in acromegaly include somatostatin analogs, dopamine agonists, and GH receptor antagonists.[8] Surgical resection is the mainstay of treatment in both pituitary micro- and macro-adenomas.[8] 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.[7] 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.[9] 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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Grunstein RR, Ho KY, Sullivan CE. Sleep apnea in acromegaly. Ann Intern Med 1991;115:527-32.  Back to cited text no. 1
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2.
Giustina A, Casanueva FF, Cavagnini F, Chanson P, Clemmons D, Frohman LA, et al. Diagnosis and treatment of acromegaly complications. J Endocrinol Invest 2003;26:1242-7.  Back to cited text no. 2
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3.
Ribeiro-Oliveira A Jr., Barkan A. The changing face of acromegaly – Advances in diagnosis and treatment. Nat Rev Endocrinol 2012;8:605-11.  Back to cited text no. 3
    
4.
Melmed S, Casanueva FF, Klibanski A, Bronstein MD, Chanson P, Lamberts SW, et al. Aconsensus on the diagnosis and treatment of acromegaly complications. Pituitary 2013;16:294-302.  Back to cited text no. 4
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5.
Fatti LM, Scacchi M, Pincelli AI, Lavezzi E, Cavagnini F. Prevalence and pathogenesis of sleep apnea and lung disease in acromegaly. Pituitary 2001;4:259-62.  Back to cited text no. 5
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6.
Davi' MV, Dalle Carbonare L, Giustina A, Ferrari M, Frigo A, Lo Cascio V, et al. Sleep apnoea syndrome is highly prevalent in acromegaly and only partially reversible after biochemical control of the disease. Eur J Endocrinol 2008;159:533-40.  Back to cited text no. 6
    
7.
Attal P, Chanson P. Endocrine aspects of obstructive sleep apnea. J Clin Endocrinol Metab 2010;95:483-95.  Back to cited text no. 7
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8.
Katznelson L, Laws ER Jr., Melmed S, Molitch ME, Murad MH, Utz A, et al. Acromegaly: An endocrine society clinical practice guideline. J Clin Endocrinol Metab 2014;99:3933-51.  Back to cited text no. 8
    
9.
Coughlin SR, Mawdsley L, Mugarza JA, Calverley PM, Wilding JP. Obstructive sleep apnoea is independently associated with an increased prevalence of metabolic syndrome. Eur Heart J 2004;25:735-41.  Back to cited text no. 9
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    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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