acromegalic face for correction pit resource guide

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SKULL BASE INSTITUTE
Surgical Correction of the Acromegalic Face
J. Timothy Katzen M.D., Joseph B. Eby M.D., Nicole E. Evans R.N.,
Hrayr K. Shahinian M.D.
Hrayr K. Shahinian, M.D.
Skull Base Institute
8635 West Third Street, Suite 1170W
Los Angeles, CA 90048
Office: 310-691-8888
Fax: 310-691-8877
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:
1.
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.
2.
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.
3.
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.
4.
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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