Dental Patient with Acromegaly – a Case Report

clinical case

Katarzyna Potoczek-Wallner

_{, Marek Bolanowski}

_{, Beata Kawala}

Dental Patient with Acromegaly – a Case Report

Pacjent stomatologiczny z akromegalią – opis przypadku

1 _{Department of Maxillofacial, Orthopaedics and Orthodontics, Wrocław Medical University}

2 _{Department and clinic of endocrinology, Diabetology and isotope Therapy, Wrocław Medical University}

Abstract

acromegaly is an acquired disorder related to excess of production of growth hormone from pituitary gland caused by pituitary adenoma. it must be considered in patients whose growth and mineralization has been developed and closure of epiphysis is done. it has to be differentiated with gigantism (giantism) – childrens’ disease which causes long bones’ growth. at onset of acromegaly, many patients are treated symptomatically without the proper diagnosis. The incidence is rare (3–4 cases/mln/year) because of small prevalence (50–70/mln inhabitants) but also because of the tumor’s slow growth. characteristic changes in appearance emerge relatively late but small symp-toms in the masticatory system can be a signal of the disease which leads to effective treatment by earlier diagnosis (Dent. Med. Probl. 2011, 48, 4, 590–593).

Key words: acromegaly, growth hormone, macroglosy, prognatism.

Streszczenie

akromegalia to uogólniona choroba spowodowana nadmiernym wydzielaniem hormonu wzrostu z przysadki mózgowej na skutek powstania łagodnego guza tego gruczołu. Występuje u osób dorosłych, u których proces wzrastania kości został zakończony, a zatem doszło do mineralizacji i zamknięcia nasad kości długich. akromegalię należy różnicować z gigantyzmem, chorobą dotyczącą dzieci, objawiającą się m.in. wzrostem kości długich. Wielu pacjentów, zwłaszcza na początku choroby, jest leczonych objawowo bez postawienia głównego rozpo-znania. Późna rozpoznawalność akromegalii wynika z jej rzadkości (3–4 przypadki/mln/rok) oraz małej częstości występowania (50–70/mln mieszkańców), jak również powolnego wzrostu guza odpowiedzialnego za jej objawy. charakterystyczne zmiany w wyglądzie całego ciała pojawiają się stosunkowo późno, ale mniejsze zmiany obecne w narządzie żucia mogą być sygnałem choroby, prowadzić do wcześniejszej diagnostyki, jak również zwiększyć skuteczność terapii (Dent. Med. Probl. 2011, 48, 4, 590–593).

Słowa kluczowe: akromegalia, hormon wzrostu, makroglosja, prognatyzm.

Dent. Med. Probl. 2011, 48, 4, 590–593

issn 1644-387X © copyright by Wroclaw Medical University and Polish Dental society

acromegaly as a disorder was first described in 1886 by famous French neurologist Pierre Ma-rie. The name acromegaly derives from Greek

ak-ros (extremities) and megas (big) [1]. The disease

is mostly caused by benign pituitary tumor (ad-enoma) and hypersecretion of its growth hormone (GH). The clinical symptoms are caused by the in-creased iGF-1 (insuline-like growth factor-1) and its influence to the tissues and also by the mass effect of local tumour growth [1]. The incidence of acromegaly is rare (3–4 cases/mln/year) and the prevalence is small 50–70/mln inhabitants [2]. Women suffer from acromegaly twice as much as men [3]. it must be considered in patients whose

growth and mineralization has beed developed and closure of epiphysis is done. it’s important to differentiate with the gigantism (giantism) – chil-drens’ disease which causes long bones’ growth.

The main clinical symptoms are: skeletal growth, broaded extremities (hands and feet) [1, 4], but also head and face different look: the nose is widened and thickened, cheek bones becomes prominent, the lips are thickened, the forehead and overlying skin leads to bossing profile [5, 6]. a progressive deepening of voice is also observed (especially in women) – acromegalic dysphonia – rough, male voice [7, 8]. There are also unspe-cific symptoms like: malodorous sweating

espe-Dental Patient with acromegaly 591

cially during the night, fatigue, weakness, muscle reduced capacity, joint distorsion, neuropathies, cardiovascular manifestation such as: hyperten-sion, arrhythmy, cardiomiopathy [9]. There are also smaller symptoms, more like pre-symptoms, connected to the masticatory system – increased spacing between the teeth, enlarged tounge and mandible prognathism due to the increased skel-etal growth of base and ramus of the mandible which leads to face profile change [1]. Patient with acromegaly may come to the dentist concerned about enlarged tongue, spaces between the teeth and tooth loosening, but also the malocclusion with mandible prognatism or bite opening.

Case Report

a 64 year old female patient with acromegaly reported for a consultation and a dental treatment plan. she was diagnosed with acromegaly 3 years ago but she had observed full symptoms 10 years before. she had a history of enlarged extremities (hands and feets) and slow progressive changes in her face from oval to square as well as voice deep-ening. By that time she suffered for painful stubby fingers and toes with sensory disturbanceswhich is very common with patients with acromegaly [10]. Past medical history revealed that the patient suffered for hypertension, osteoporosis, hormone system disorders, cysts in right and left kidney, la-ryngeal cyst, rectal polyps and varicose veins, thy-roid disease. The patient had a transnasal, trans-phenoidal surgery of a pituitary tumor 2 years ago but before she was treated with somatostatin analouge (sandostatin laR) first 20 mg and then 30 mg intramuscularly every 28 days. after the surgery she has been with no treatment under the control of the Departament of endocrinology, Di-abetology and isotope Therapy, Wroclaw Medical University.

in the general examination there were no changes in patient’s posture but the hands and feets were stubby which is a clinical symptom of glycosaminoglycan deposition and increased col-lagen production and joints were widened and thickened (Fig. 1). Her face was also changed: square, masculine looking, frontal bossing with prominent eyebrows connected to the hypertro-phy of frontal sinuses. Big mandible was caused by secondary growth of cartilage and fibrous tis-sue (Fig. 2). The patient’s profile was transfrontal without mandibule prognatism. a lower dental arch was wide due to the longer base and ramus of mandibule. Her maxillary part of the face was lon-ger too. Her lower and upper lips were thickened with possitive Korkhause value (Fig. 3).

Fig. 1. Handwrist of a patient with acromegaly Ryc. 1. Dłoń pacjentki z akromegalią

Fig. 2. Face of a patient – en-face view Ryc. 2. Twarz pacjentki enface

intra oral examination revealed toothlessness in the upper dental arch and free-end gaps on the right side of the lower arch and interdental tooth loss on the left (Fig. 4). The toothlessness in the maxilla and extensive loss of tooth in

mandibu-K. Potoczek-Wallner, M. Bolanowski, B. Kawala

592

la (34, 35, 36, 38 and 45, 46, 47, 48) were caused probably by the severe chronic periodontitis de-scribed by the patient as the tooth loosening and increased spacing. she has never been treated by any periodontologist. intra oral examination re-vealed also some dental caries in 41, 44, 32, 33, 37 where treatment was needed and also big amount of supra- and subgingival calculus and plaque but without generalized mobility of teeth. Only the second left molar (37) exhibited grade 1 mobil-ity. The tooth crowns were secondarily longer due to the periodontitis but also toothlessness in the upper arch. increased spacing was noted between the teeth as a result of bigger horizontal dimesion of a dental alveolar. also tongue appeared to have

hypertrophic features – i.e. much broaded and longer (Fig. 5).

Panoramic radiograph revealed horizontal alveolar bone loss in the mandibula with a big amount of interdental subgingival calculus. nei-ther the root structure disorders nor the hyperce-mentosis were revealed (Fig. 6).

The patient noted she had never been treated by any prosthetist and never used any denture due to the fact that the measurements of the up-per and lower jaws had been changing until the time she had an operation. The patient was in-formed about necessity of multidisciplinary den-tal treatment. First the scalling with polishing and sandblasting was planned. she also was asked to rinse her mouth with clX solution. Then the re-storative treatment of the teeth with cavities were planned. The patient also needed full periodontal and prosthetic treatment with a tempomandibular joints rehabilitation later. The sequence of occlusal splints were necessary due to the need of gradual increase in the occlusive height. The upper com-plete denture and lower removable partial denture (RPD) were made.

Fig. 3. Face of a patient – profile view Ryc. 3. Twarz pacjentki z profilu

Fig. 4. intraoral picture Ryc. 4. Zdjęcie wewnątrzustne

Fig. 5. Hypoglossy

Ryc. 5. Powiększenie języka

Fig. 6. Panoramic X-ray

Dental Patient with acromegaly 593

Discussion

acromegaly is a multisystem disorder which in 90% of patients is caused by the exposure of supraphysiological levels of the growth hormone (GH) from pituitary adenoma and insuline-like-growth factor-1 (iGF-1) [6]. The disease affects both men and women but women suffered from acromegaly twice as much as men [3]. acromegaly can cause a variety of symptoms such as: skeletal disorders such as excessive skeletal growth, wid-ened and thickwid-ened soft tissues revealing by big-ger and stubby hands and feet with widened joints but also masculine looking, square face with man-dibule prognathysm [5, 6]. characteristic changes in masticatory system such as: interdental spac-ing, hypertrophic tongue [11], mandibular prog-nathism due to the overgrowth of the ramus and base may be observed much earlier [5]. The roots of teeth, especially molars, can be enlarged due to the hypercementosis [11], but it wasn’t observed in this patient. it is very important to remember that multidisciplinary dental treatment (restorative, prosthetics, orthodontics, and ortho-surgery) should be always consulted with an endocrinolo-gist.

Rare and late incidence of the acromegaly is caused by the wide symptomatology and varied influence of GH on the different systems. This fact leads to consequences such as cardiovascular complications. additionally patients with active acromegaly suffer from bone disformation but also widening of joint spaces due to cartilage hypertro-phy, respiratory complications (sleep apnea affects 60–80% of all patients with acromegaly), neuropa-thies (symptomatic carpal tunnel syndrome is fre-quent 20–50% of all patients) [1, 9]. acromegaly is associated with 2.5 times increased mortality. if left untreated, patients with acromegaly would die about 10 years earlier than healthy subjects, that is why the early diagnosis so important [9]. Wide knowledge about acromegaly symptoms in masti-catory system, insightful dental examination and a X-ray diagnostic may lead the dentist to suspect acromegaly and refer a patient to the endocrinolo-gist specialized in diagnostic and treatment of hormone disorders. The earlier the patient is di-agnosed the bigger chances for treatment which stops the subsequent progress of the disease and limits complications due to the hormonal disor-ders. The earlier diagnosis is made the longer and better life of patients with acromegaly is.

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Address for correspondence:

Katarzyna Potoczek-Wallner Department of Maxillofacial, Orthopaedics and Orthodontics Wrocław Medical University Krakowska str 26 50-425 Wrocław Poland e-mail: kpotok@interia.pl Received: 22.08.2011 Revised: 20.10.2011 accepted: 24.10.2011

Praca wpłynęła do Redakcji: 22.08.2011 r. Po recenzji: 20.10.2011 r.