1.Introduction

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Acromegaly characteristically causes enlargement of the mandible, zygomatic arches and supraorbital ridges, as well as an enlarged nose and on occasion’s nasal obstruction. It is caused by growth hormone secreting pituitary adenoma and is treated surgically by hypophysectomy.

The soft tissue changes caused can be seen to regress after successful treatment of the underlying hyperpituitarism but the bony growth does not reverse. This can lead to concerning and permanent changes in appearance which can be managed surgically, the procedure required depending on the patient specific concerns and problems. [1,2]

We present a case of an acromegalic patient who had been successfully treated for the underlying pituitary adenoma and who presented to the ear, nose and throat department with ongoing concerns about her “giant nose”. Similar cases are sparsely reported in recent literature.Literature available will also be reviewed and discussed.

2. Case Report

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The patient is a 54 year old lady who presented 10 years after successful treatment for hyperpi-tuitarism caused by a pituitary adenoma. The original presenting complaint was of malocclu-sion caused by bony growth of the mandible. After treatment for the pituitary adenoma and before presenting to the ear, nose and throat de-partment, mandibular setback surgery had been carried out.

The complaint was of a very large, blocked and twisted nose. An external approach septorhinoplasty was per-formed. There was a grossly over-projected nose with a drooping columella and a large dorsal bone and cartilage hump. The anterior nasal spine was also elongated and prominent with a deviation of cartilaginous septum to the right. The nasal bones were deviated to the left. (Fig-ure1). The surgical procedure for correction of these findings was via a notched columella inci-sion. Septoplasty was performed, the hump was reduced. Lateral and medial osteotomies cor-rected the deviation of the nasal bones and the “open book deformity” which was subsequent to reduction of the dorsal hump. To shorten the columella, the anterior maxillary spine was re-duced, the caudal end of the septum and the medial crura were trimmed and overlapped.

Persistent nasal obstruction was noted in subse-quent follow up and a limited revision septop-lasty was performed 11 months after the original procedure. This was resection of septal cartilage via a Killian’s incision which resolved the ob-struction (Figure 2).

Figure 1 & 2

3. Discussion

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A search of the Medline and Embase databases with the terms terms “nasal” and “acromegaly”, “gigantism” or “hyerpit*”, revealed five cases of rhinoplasty surgery performed on acromegalic patients. These are made up of a series of three and two further individual case reports and date from between 1975 and 1994. In 1994, Dabb and Aker described a technique of performing simultaneous external rhinoplasty and using that approach for the transphenoidal hypophysecto-my. [3] In these three cases no complications were reported and good cosmetic outcomes were con-sidered but long term out comes have not been seen and this technique is not in common place use now. There are two other case reports of rhinoplasty for cosmesis in acromegalic patients that had been previously treated for hyperpitui-tarism and considered otherwise stable. As with our case, the complaint was of an enlarged nose as a consequence of tissue growth. Sugar re-ported one of these cases, a male aged 23 who had simultaneous maxillo-facial surgery to the mandible and frontal bones, the only rhinoplasty technique used was a reduction in the dorsal hump.[4] The earlier case reported in 1975 was of a 28 year old female whose first presentation of acromegaly leading to diagnosis of a pituitary adenoma was with a complaint of a large and growing nose.[5] The details of the surgery per-formed are not given except that it was per-formed under local anaesthetic after treatment of the endocrinological cause of the change in ap-pearance.

Changes in the facial appearance of patients with acromegaly are prominent and in this case were amongst the presenting complaints that led to the diagnosis of a pituitary adenoma. The bony and soft tissue growth of hyperpituitarism cause these changes and incompletely reverse following successful treatment of the underlying pituitary adenoma.

Our patient also complained of nasal obstruction found to be cause by a deviated septum. Symp-toms of nasal obstruction as well as cometic concerns are found in acromegalic patients and are a direct consequence of tissue growth caused by hyperpituitarism. This may be due to mucos-al growth within the nasal and paranasal sinuses as demonstrated by Skinner and Richards.[6] It can also be caused by growth at the bony - carti-laginous junction in the septum leading to devia-tion of the septum, as in our patient. lAs dis-cussed by these authors and exemplified in our case, these problems can be addressed surgically with success.

4. Conclusion

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The cosmetic and functional changes seen in acromegalic patients do not always regress fol-lowing successful treatment and return of nor-mal serum growth hormone levels. The patterns of tissue growth and change in features are not predictable and seem to be unrelated to length of disease or severity of growth hormone de-rangement at the time of diagnosis. A standard septorhinoplasty technique was effective in cor-recting the gross cosmetic and functional de-formity in a patient with a “Giant Nose”

Summary Bullet Points

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. Hyperpituitarism causing Acromegaly can cause permanent changes in facial features
. Nasal enlargement and obstruction are amongst these features
. Changes in nasal appearance and obstruc-tion in these patients can be improved with rhinoplasty surgery

References

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1. Vetter U, Pirsig W, Landolt A and Heinze E. Growth activities of the nasal septal cartilage in acromegaly.Rhinology 1984; 22: 125-131
2. Kunzler A and Farmand M.Changes in the vis-cerocranium in acromegaly. Journal of cranial-maxillofacial surgery 1991; 19: 332-340
3. Dabb R and Aker J. external rhinoplasty ap-proach for the treatment of acromegaly. Annals of plastic surgery 1994; 32: 630-637
4. Sugar A.Correction of residual facial deformity following treatment of acromegaly. Journal of Maxillo-facial surgery 1986; 14: 14-17
5. Hirsowitz B, Mayblum S and Kanter Y.Corrective rhinoplasty for enlargement of the nose due to acromegaly. Plastic and reconstruc-tive surgery 1975; 56(6): 665-667
6. Skinner D and RichardsS. Acromegaly - the mucosal changes within the nose and paranasal sinuses. S Journal of laryngology and otology 1988; 102: 1107-1110