Kamal Gharnizadeh, Alireza Mohammadi, Zahra Malekpoor, Maryam Sohrabi

Oral and Maxillofacial Surgery Department, Dental Division of Bou-Ali Hospital, Tehran Islamic Azad University, Tehran, Iran

Introduction

Acromegaly is a clinical syndrome which results from excessive production of growth hormone (GH); the most common cause of this condition is adenoma of the pituitary gland. Acromegaly is associated with a progressive acquired body disfigurement mainly affecting the face and extremities.1
The uncontrolled secretion of growth hormone especially after puberty leads to excessive growth of mandible and facial bones which is due to the reactivation of growth centers in condyles .2 The major clinical manifestations such as broad extremities, thickening of soft tissues and skin, frontal bossing, enlargement of the nose and jaw, thick lips, marked facial lines, widening of maxilla as well as prominent cheek bones, prognathism, tooth separation, and jaw malocclusion can be seen in these patients.The malformations in acromegaly mostly progress slowly and can be seen in years. In severe conditions, skeletal disfigurement can also be seen such as dorsal kyphosis and abnormalities in the ribs and the chest cavity leading to the classical “Punchinello” aspect . Moreover, headaches, nocturnal foul odor sweating, and arthralgia may also be seen in some cases.1
Mandibular prognathism (MP) or skeletal Class III malocclusion is known as a severe maxillofacial deformity; when a bilateral symmetrical MP is detected in a case, acromegaly should be considered as a differential diagnosis 3,4 Because of the skeletal relapse of MP after the orthognathic surgery procedure, the effectiveness of this method still remains uncertain.5,6 In this study, we aim to introduce a new method which may diminish the relapse of skeletal deformities after orthognathic surgery in a patient suffering from acromegaly.

Case report

In this report we present a 33 years-old man, suffering from acromegaly, who was admitted to oral and maxillofacial surgery ward of Bou-Ali hospital for correction of prognathism. In his past medical history he mentioned that his extremities began to get abnormally enlarged 12 years prior to this admission
and trans-sphenoidal hypophysectomy was conducted because of pituitary adenoma after 3 years. In his first admission, a concave profile with prominent supraorbital ridges, prognathism, large lips and bulbous nose were detected. His GH level was at normal range at the time of admission.
Cephalometrical analysis declared Class III skeletal growth form as well as about 14 mm reverse over jet. Macroglossia was also detected in this patient (Figure 1).

Discussion

Acromegaly is a rare disease occurs in3-4 cases per million per year.4,7 Due to its slow progression, it is mostly diagnosed in late adulthood; It occurs equally in both men and women.8,9 Surgical procedures aiming to correct skeletal deformities and endocrine malfunction are performed in acromegaly.10 Among them, sphenoidal hypophysectomy is considered as the most effective and the most prevalent procedure.11 The early outcomes of this procedure were convincible in all patients; however, the long term outcomes differ from patient to patient. In a study it was declared that in some patients the acromegaly recurred even after a successful drainage of adenoma.12 On the contrary, only in one study, it was found that the surgical outcome in acromegaly has no difference from that one in normal patients; Moreover, it was declared that in such patients after endocrine balance, mandibular osteotomy must be conducted in order to achieve facial contour and perfect occlusion.13 However, in most cases of acromegaly the risk of recurrence of GH level abnormalities and facial deformities is more than other cases; thus, by sphenoidal hypophysectomy these recurrences may be mild and clinically undetectable.14 Furthermore, in another study, sphenoidal hypophysectomy was considered as a preventive procedure which is conducted in order to inhibit unpredictable mandibular growth, while it can also cause the mandibular function loss during the time.15 In the present case, hypophysectomy was performed twice and the adenoma was totally extracted. The GH level was preserved in normal ranges before the surgery and also one year after the surgery. It is assumed that keeping GH in a normal range before the surgery and also evaluation of orthognathic aspect and endocrine status of the patient in the follow-ups is effective in preservation of orthognathic surgery outcomes.

Acknowledgments

Authors wanted to appreciate the scientific writing and editing of the manuscript by scientific writing committee of Sadra-tech™ Company, Shiraz University of Medical Sciences, Shiraz, Iran.