Abstract
Acromegaly is a chronic and progressive disease related to a disorder of growth hormone production. It may present with a variety of clinical signs and a dento-facial deformity and this results in a loss of self-esteem and a reduction of Quality of Life.
Presentation of case
A 38-years–old male patient, affected by acromegaly with class III malocclusion, noticeable nose deformity and macroglossia was treated. Bi-maxillary orthognathic surgery and partial glossectomy have been performed in one-step surgey. Open rhinoplasty was done in a second step. Both dento-skeletal class III and restoration of the facial appearance have been solved. Dento-skeletal class III was completly solved together with the restoration of the facial appearance and the patient satisfaction has been achieved.
Conclusions
This case report describes the successful and stable treatment of an adult patient affected by acromegaly.
Highlights
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Acromegaly is a chronic, progressive disease related to a disorder of GH production.
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Acromegalic patients have typical face and macroglossia is frequently associated.
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Macroglossia influences orthognathic treatment because that may cause a relapse.
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The goals of treatment were correct skeletal malocclusion and improve the aesthetics.
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Performe orthognathic surgery and glossectomy together is related to prevent relapse.
1
Introduction
Pathogenesis of acromegaly is well known as is the effect that GH and IGF have on tissues and organs. It is known that the face is not spared by the effects of this chronic and progressive pathology. Furthermore, even when the cause is identified and treated the effects that these hormones have had on the skeleton, skin, cartilage and muscles of the face are irreversible [ ]. Acromegaly has a subclinical onset so often at diagnosis patients have even serious complications such as: diabetes mellitus, hypertension and respiratory/cardiac failure [ , ]. However acromegaly is frequently associated with altered facial appearance at the time of diagnosis and sometimes new diagnoses of acromegaly are detected by others specialists than endocrinologists. Acromegalic patients have typical face, nose, ears and jaw protruded, with a slightly open mouth due to labial incompetence [ , ]. Macroglossia frequently is accompanied by obstructive sleep apnea syndrome (OSAS) and also influences the orthognathic treatment, because that may cause a postoperative relapse [ , ]. These disturbances can be associated with impaired self-esteem and body image distortion. These disorders of course affect the quality of life of the patient [ , ]. A multidisciplinary approach is mandatory not only in the treatment of endocrinological disorder but especially in the management of patient’s sequele.
1.1
Presentation of Case
A 38-years–old male patient suffering from acromegaly was admitted in our Department in 2016 for evaluation and treatment. He previously had been diagnosed with GH-secreting pituitary adenoma. Tumor had been resected in 2008 and he didn’t need medical therapy. Patient presented the typical acromegalic face ( Fig. 1 ). A lateral cephalogram showed a skeletal class III relationship SNA 85.41°; SNB 96.0°; ANB −10.59° ( Fig. 2 A) He displayed an anterior open bite, involving a −3.79 mm overbite and a −14.31 mm overjet. Polysomnography (PSG) revealed a moderate OSAS: Apnea–Hypopnea Index (AHI), 28.2/h; apnea index (AI) 27.5/h, hypopnea index (HI) 0.6/h. and Oxygen Desaturation Inndex (3% ODI) 17,84/h. Cone Beam Computer Tomografy (CBCT) was acquired and 3D Virtual Surgical Plan was planned. Surface scanning of each dental model arch was achieved, thereby producing an STL file that were fused with the 3D reconstruction. 3D Virtual Surgical Plan was performed and osteotomies and movement of maxilla and mandible have been simulated by the software. Bi-maxillary orthognathic surgery was performed, including Le Fort I osteotomy advancement and bilateral sagittal splint ramus osteotomy. During the first surgical step, partial glossectomy was performed using the tongue reduction Harada-Enomoto method [ ]. The open rhinoplasty was performed in 2018 included the tip-plasty, septoplasty and nasal bone osteotomies to correct the abnormal growth and shape of the nose [ ].
