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Surgical Correction of the Acromegalic Face
By J. Timothy Katzen M.D., Joseph B. Eby M.D., Nicole E. Evans R.N., Hrayr K. Shahinian M.D.
Background
Patients with growth hormone secreting pituitary adenomas frequently develop abnormal craniofacial bone growth. Collectively, these physical features along with enlargement of the hands and feet are called acromegaly. Due to the increased amount secreted growth hormone, the skull and face grow disproportionately. The more growth hormone secreted, the more the skull and face continues to grow. These facial differences can include frontal bossing (large forehead), prominent supraorbital ridges (large eyebrow ridges), malar flatness and maxillary hypoplasia (a small upper jaw with flat cheeks), as well as mandibular prognathism (a large lower jaw) resulting in malocclusion (teeth that don't line up).[1, 4] Unfortunately, even long after the removal of a patient's pituitary tumor and when growth hormone levels return to normal, the disproportionate facial features do not return to their previous appearance.
Many patients are troubled by the way they look after their pituitary tumor is removed. Now, through surgical reconstructive techniques employed by craniofacial surgeons these abnormalities can be corrected.
Surgical Technique
Previously, surgical correction of the physical deformities of acromegaly required a large, interdisciplinary team and included multiple, long, hospital stays, weeks of the teeth being wired together, and often the placement of a tracheostomy (breathing tube in the neck). Now, correction of many of these deformities can be performed usually in a single operation. Typically, patients spend a few days the hospital, do not require wiring of their teeth, nor do they require a tracheostomy.[1, 2]
Each patient is an individual and his or her surgical requirements often vary dramatically. Consequently, each operation must be tailored to each patient's specific needs. Typically, the surgery entails three incisions. All the incisions are hidden from view. Two buccal incisions (hidden inside the mouth) are used, one is made just behind the upper lip and the other is made behind the lower lip. The operation begins with correction of the mandibular prognathism (a large lower jaw). The mandible (jaw bone) and soft tissue of the lower jaw are reduced. Then, attention is focused on the midface (cheek bones and upper jaw). Typically, the midface is augmented or increased in profile depending on the patient's appearance. Extra special care is taken to ensure that the teeth line up. In the past, this would have required that the teeth be wired shut and a tracheostomy be performed. Using a preoperatively fabricated splint, the teeth can now be aligned perfectly in the operating room, thus avoiding the need for wiring the teeth together. The coronal incision is the third incision, and is made across the top of the head, behind the hair line. This allows access to the frontal bones (forehead) and supraorbital ridges (eyebrows ridges). The forehead and eyebrow ridge are then contoured into a more normal shaped bone. Additional procedures may involve a reduction in the size of the tongue and other soft tissues.
Typically, there is a dramatic improvement in the patient's physical appearance, psyche, and speech.[3]
Conclusion
Recent advances in craniofacial reconstruction have dramatically improved cosmetic outcomes for acromegalic patients. Advances have obviated the need for a tracheostomy and have resulted in single stage operations with a short hospitalization. With the advent of new reconstructive materials and instruments, craniofacial surgeons will continue to develop improved methods of correcting the facial abnormalities that result from excess growth hormone secretion.
References:
- Chung KC, Buchman SR, Aly HM, Trotman CA. Use of modern craniofacial techniques for comprehensive reconstruction of the acromegalic face. Ann Plast Surg 1996;36:403-8; discussion 403-8.
- Jackson IT, Meland NB, Keller EE, Sather AH. Surgical correction of the acromegalic face. A one stage procedure with a team approach. J Craniomaxillofac Surg 1989;17:2-8.
- Serra Payro JM, Vinals Vinals JM, Palacin Porte JA, Estrada Cuixart J, Lerma Gonce J, Alarcon Perez J. Surgical management of the acromegalic face. J Craniofac Surg 1994;5:336-8.
- Takakura M, Kuroda T. Morphologic analysis of dentofacial structure in patients with acromegaly. Int J Adult Orthodon Orthognath Surg 1998;13:277-88.
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