INTRODUCTION
Cutis laxa is a rare multisystem disease of connective tissue, characterized by
dysfunction of elastic fibers, generating inflexible skin with redundant folds
and a
wrinkled appearance. Thus, individuals affected by this disease complain about
their
aged appearance, in addition to having aortic aneurysms and pulmonary problems
more
frequently1-3. This disorder can be acquired or hereditary -
dominant or recessive, the latter presenting symmetrical involvement and usually
having a worse prognosis.
Elastic fibers are mainly composed of elastin, a protein from the collagen family
that enables tissue flexibility and resistance to tension. In lax cutis, loss
of
elastin was detected, with the fragmentation of elastic fibers in the reticular
dermis causing laxity in the skin, vessels, lungs, and other tissues2.
Treatments are based on the use of cosmetics or surgical techniques as a form of
relief or correction, with plastic surgery being an extremely important tool for
the
therapy of these patients4.
Faced with changes in appearance, most notably in the face, neck, armpit and groin
region, there is a great desire on the part of patients for surgical corrections,
especially those related to facial changes5.
Of the possible surgical procedures, blepharoplasty surgery, both lower and upper,
aims to improve the senile appearance and provide rejuvenation in the area around
the eyes, thus making the look appear more rested and alert6.
Traditional lower blepharoplasty is a procedure that, in most cases, brings good
results, resulting in a barely noticeable subciliary scar7.
Canthopexy is a surgical procedure performed after classic upper blepharoplasty and
lower conjunctival blepharoplasty. In the anterior segment, an ellipse of tissue
is
removed, as well as a strip of preseptal orbicularis muscle. In the lower part,
a
conjunctival approach is used to excise the pockets of fat. The surgical technique
begins by making an incision in the tissue with an approximate diameter of 2 mm,
located instantly below the lateral eyelid corner6,8.
OBJECTIVE
This article aims to report the case of a young patient who underwent superoinferior
blepharoplasty with canthopexy to correct anatomical and aesthetic periorbital
changes resulting from lax cutis.
CASE REPORT
Female patient, 17 years old, referred to the Plastic Surgery Service of the Walter
Cantídio University Hospital, Fortaleza-CE, seeking medical help for treatment
due
to dissatisfaction with her appearance, especially in the orbital region, in
addition to difficulty in social integration due to stigmatization.
On physical examination, exuberant upper dermatochalasis was evident, with bulging
due to herniation of fat pads and bilateral ptosis of the lacrimal glands,
contributing to the effect of eyelid pseudoptosis associated with herniation of
lower eyelid bags and a rounded corner with a negative canthal angle. (Figure 1). Furthermore, ptosis of the malar
tissue was identified with the prominence of the nasolabial fold, increased jowl
fat, and retrognathism. Hypertrophy of the upper pole of the ears was also noticed,
with slight effacement of the scaphoid fossa associated with turbinate
hypertrophy.
Figure 1 - Patient with lax skin in the pre-operative period.
Figure 1 - Patient with lax skin in the pre-operative period.
During the pre-operative evaluation, complementary tests were carried out looking
for
cardiopulmonary, respiratory, or large vessel changes relevant to the investigation,
which did not demonstrate any changes. During the pre-operative period, the patient
signed the Free and Informed Consent Form as approved by the Ethics Committee
of the
Walter Cantídio University Hospital (CAAE: 50728421.5.0000.5045).
The procedure was carried out on December 15, 2021. Due to the patient’s main
complaint regarding her eyelids, we began her treatment with upper and lower
blepharoplasty, removing ellipses of skin and fatty pockets, repositioning of
the
lacrimal glands associated with canthopexy without canthotomy and cantholysis,
for
better positioning of the canthal angle, refixing the inferior segment of the
lateral canthal tendon more internally on the orbital rim close to its
insertion.
In the postoperative period, the patient returned to the service’s plastic surgery
outpatient clinic on the seventh day to remove stitches from the skin synthesis.
Periodic monitoring has been maintained since then. A satisfactory result was
observed for the proposed surgery, identifying adequate correction of the existing
changes, namely significant dermatochalasis associated with prolapse of the lacrimal
glands, as well as the negative canthal angle (Figure 2).
Figure 2 - Postoperative period of upper blepharoplasty with canthopexy.
Figure 2 - Postoperative period of upper blepharoplasty with canthopexy.
The patient evolved without postoperative complications, being quite satisfied with
the result obtained. She continues to be monitored by the Plastic Surgery Service
at
the Walter Cantídio University Hospital with future proposals for rhytidectomy,
chin
advancement, or mental prosthesis and otoplasty.
DISCUSSION
Cutis laxa is a genetic disease that causes sagging and redundancy of the skin,
leaving an aged appearance in these patients. The changes caused by the disease
can
lead to social isolation, difficulty interacting, and the worsening of mental
illnesses.
Therapeutic planning to correct lesions must be individualized and carried out by
surgeons who recognize the dermatological differences of these patients. There
are
few reports in the literature on the surgical approach to the eyelids in patients
with lax skin9,10.
Ozsoy et al.11 described a case of
a 20-year-old patient diagnosed with cutis laxa who presented a satisfactory
aesthetic result with bilateral upper blepharoplasty without excision of the adipose
tissue.
Banks et al.12 reported two cases
of patients with lax cutis, one 44 years old and the other 5 years old, who
underwent rhytidoplasty with blepharoplasty to correct the aesthetic defects caused
by the disease. An acceptable result was observed in the immediate postoperative
period, with good healing, but the disease recurred months after the surgical
approach due to the chronic defect in collagen metabolism.
CONCLUSION
In view of the above, the importance of facial surgical correction in cases of lax
skin is observed, highlighting the importance of applying appropriate surgical
techniques and improving them in this patient profile.
Due to the multiplicity of changes, it is still necessary to inform patients that
the
treatment is not always completed with just one surgical procedure and that other
defects are often recommended to be addressed in other operative stages, as proposed
for the patient in this case.
REFERENCES
1. Taylor JA. Blueprints plastic surgery. Malden: Blackwell;
2005.
2. Berk DR, Bentley DD, Bayliss SJ, Lind A, Urban Z. Cutis laxa: a
review. J Am Acad Dermatol. 2012;66(5):842.e1-17. DOI:
10.1016/j.jaad.2011.01.004
3. Dantas SG, Trope BM, de Magalhães TC, Azulay DR, Quintella DC,
Ramos-E-Silva M. Blepharochalasis: A rare presentation of cutis laxa. Actas
Dermosifiliogr (Engl Ed). 2019;110(4):327-9. DOI:
10.1016/j.ad.2018.04.006
4. Beighton P, Bull JC, Edgerton MT. Plastic surgery in cutis laxa. Br
J Plast Surg. 1970;23(3):285-90. DOI:
10.1016/S0007-1226(70)80057-1
5. Jones AP, Janis JE. Essentials of Plastic Surgery. Q&A
Companion. New York: Thieme Medical Publishers; 2016.
6. Lessa S, Sebastiá R, Flores E. Uma Cantopexia Simples. Rev Bras Cir
Plást. 1999;14(1):59-70.
7. Cardim VLN, Bazzi K, Silva AS, Silva MG, Santos FM, Salomons RL, et
al. Cantopexia e reforço tarsal com retalho de periósteo. Rev Bras Cir Plást.
2013;28(1):36-40.
8. Game J, Morlet N. Lateral canthal fixation using an oblique
vertically orientated asymmetric periosteal transposition flap. Clin Exp
Ophthalmol. 2007;35(3):204-7. DOI:
10.1111/j.1442-9071.2007.01454.x
9. Misani M, Fontaine S. Cutis Laxa of the face: A case report and
review of literature. J Dermatol Res Ther. 2018;4:055. DOI:
10.23937/2469-5750/1510055
10. Xue Y, Chen H, Zeng X, Jiang Y, Sun J. Generalized acquired cutis
laxa treated with facial plastic surgery. Eur J Dermatol. 2011;21(1):141-2. DOI:
10.1684/ejd.2011.1206
11. Ozsoy Z, Gozu A, Dayicioglu D, Mete O, Buyukbabani N. Localized
cutis laxa and blepharoplasty. Dermatol Surg. 2007;33(12):1510-2; discussion
1512. DOI: 10.1111/j.1524-4725.2007.33326.x
12. Banks ND, Redett RJ, Mofid MZ, Manson PN. Cutis laxa: clinical
experience and outcomes. Plast Reconstr Surg. 2003;111(7):2434-42; discussion
2443-4. DOI: 10.1097/01.PRS.0000060800.54979.0C
1. Residente do Serviço de Cirurgia Plástica do
Hospital Universitário Dr. Walter Cantidio - HUWC
2. Universidade Federal do Ceará, Fortaleza, CE,
Brasil
3. Membro Titular SBCP - Staff Serviço de Cirurgia
Plástica do Hospital Universitário Dr. Walter Cantidio - HUWC
4. Membro Titular SBCP - Regente e Chefe do
Servico de Cirurgia Plástica e Microcirurgia Reconstrutiva do Hospital
Universitário Dr. Walter Cantidio - HUWC
Corresponding author: José Isnack Ponte de Alencar
Filho Rua Pastor Samuel Munguba, 1290, Rodolfo Teófilo, Fortaleza,
CE, Brazil, Zip Code: 60430-372, E-mail: isnack_alencar@yahoo.com
Article received: October 12, 2022.
Article accepted: October 23, 2023.
Conflicts of interest: none.