Cutis Laxa and the Value of Rhytidectomy: 4 Patients and Years of Follow-Up.

The modalities of the esthetic treatment of cutis laxa (CL) patients have rarely been discussed. Here, the authors present 4 CL rhytidectomy cases. The surgery method was designed based on the traditional full-incision face-lift technique combined with lamellar superficial musculoaponeurotic system flap elevation and plication. Temporal, buccal, mandibular, and cervical regions were targeted. The shortest follow-up was 30 months, and the longest was 72. The results were assessed by subjective satisfaction and third-party photo evaluation. The subjective satisfaction was graded into worse than preoperation, no improvement, slight improvement, moderate improvement, and obvious improvement. The satisfaction rate was the sum of moderate and obvious improvement. Eighteen uncorrelated professionals participated in the third-party photo evaluation, in which the preoperative and follow-up photos were presented, and frontal, temporal, mid-face (including jowl), and cervical (including mandibular line) regions were evaluated as improved or nonimproved. The outcome of the subjective satisfaction was graded as no improvement (2/12, 16.7%), slight improvement (3/12, 25%), moderate improvement (4/12, 33.3%), and obvious improvement (3/12, 25%). The satisfaction rate was 58.3%, most investigators denied the frontal and temporal region improvement, 60% observed mid-face improvement, and 80% noticed cervical improvement. Most patients and their parents had positive attitudes towards the treatment results; however, the full expectation was not achieved. The cervical region maintained the best improvement after our treatment and the mid-face ranked second. This surgery method conduces to the esthetic requirement of CL patients; however, skin laxity remains a challenge.

A proposal of rehabilitative approach in the rare disease "De Barsy Syndrome": case report.

De Barsy syndrome is an autosomal recessive condition characterized by an progeroid appearance with distinctive facial features and cutis laxa. Ophthalmological, orthopedic, and neurological anomalies are generally also present. This syndrome is rare and the complex therapeutic management, from a surgical but also rehabilitative point of view, has not been recognized. The aim of this paper is to describe a possible rehabilitative protocol, after an orthopedic surgical treatment, in a child with De Barsy Syndrome. A 6-year-old boy was born with a congenital bilateral hip dysplasia associated with bilateral congenital foot deformity (vertical talus). Moreover, he showed stereotypic dyskinetic movements and psychomotor delay with cognitive impairment and absent language; the sitting position was maintained with orthoses to support the trunk control and the standing position was not acquired. He was treated with pinstripe knee-highs for the foot and double nappy for the hips. At 19 months old, he underwent a two stage surgical approach for a bilateral pronated valgus foot with severe talonavicular subluxation. Satisfactory hip range of motion was achieved by conservative treatment alone. Afterwards, for the foot laxity and the flat-pronated foot corrective shoes were prescribed. The main rehabilitative goals were: attention improvement, visual exploration for foot-eye and hand-eye coordination, encourage the essential prerequisites of language, controlling the upright position using support, improving hip-knee-foot relationship, improving load transfer between the right and left sides of the body, and bimanual coordination. The rehabilitation process lasted six months, three times a week, for a time from 30 minutes to 60 minutes per session. The results were encouraging and the patient acquired the possibility of sitting with the indicated postural system, the possibility of assuming an upright position and taking a few steps with the aid of rollator with a postural stabilization system for the pelvis.

The Histopathological Findings of Patients Who Underwent Blepharoplasty Due to Dermatochalasis.

PURPOSE: To assess changes in lymphatic vessels, collagen, and elastic fiber structure in excised tissues with dermatochalasis (DC). METHODS: In this prospective histopathological study, 70 upper eyelid skins of 35 patients operated on for dermatochalasis were compared with 10 eyelid skins of 10 patients operated on for other diagnoses. In histopathological examination, the two groups of patients were compared with respect to number of lymphatic vessels, largest lymphatic vessel diameter, number of elastic fibers, number of macrophages, edema between collagen fibers, and depth of stromal collagen bed. RESULTS: As compared to the control group, the study group had a significantly greater number of dilated lymphatic vessels (p = 0.0001), largest lymphatic vessel diameter (p = 0.02), depth of stromal collagen bed (p = 0.0001), edema space between collagen fibers (p = 0.0001), elastic fiber density (p = 0.0001), and number of macrophages (p = 0.001). CONCLUSION: According to the results of the present study, in addition to an increase in the diameter and number of lymphatic vessels, a reduction in elastic fibers that are essential for the structure and function of lymphatic system, disarrangement in collagen fibers, stromal edema, and increased number of macrophages play a role in the development of dermatochalasis.

Correlation between increase in margin-crease distance and patient satisfaction after upper blepharoplasty / Correlação entre o aumento da distância margem-sulco e satisfação do paciente após blefaroplastia superior

Abstract Objective: To quantitatively and qualitatively evaluate postoperative outcomes and patient satisfaction after upper blepharoplasty and to correlate the findings with changes between preoperative and postoperative eyelid measurements using a digital imaging system. Methods: A total of 60 eyelids in 30 patients with dermatochalasis who were treated in the ambulatory center of the Department of Oculoplastic Surgery at the Anápolis Ophthalmology Hospital were evaluated. Patients ranged from 40 to 80 years of age. Photographs were taken before the upper blepharoplasty procedure and 90 days after as well. The images were transferred to the ImageJ 1.34n program. The parameters analyzed were palpebral fissure height in primary position and margin-crease distance. The correlations between these measurements and patient satisfaction 90 days after surgery were evaluated. Results: This study revealed an increase in the margin-crease distance after upper blepharoplasty and a high positive correlation (0.64) between the increase in this height and the level of satisfaction that the patients attributed to the surgery. There was no statistically significant difference between preoperative and postoperative palpebral fissure heights. Conclusion: The margin-crease distance may serve as a quantitative measurement of a good cosmetic and functional outcome, since it has been found to be strong correlated with patient satisfaction.

Resumo Objetivo: Avaliar de maneira quantitativa e qualitativa o resultado pós-operatório e a satisfação de pacientes submetidos à blefaroplastia superior e correlacionar com as medidas palpebrais antes e após a cirurgia utilizando o sistema de imagem digital. Métodos: Foram avaliadas 60 pálpebras de 30 pacientes com dermatocálase atendidos no ambulatório de Plástica Ocular do Hospital Oftalmológico de Anápolis, com idade entre 40 e 80 anos. Foram realizadas fotografias antes e 90 dias após blefaroplastia superior. Essas imagens foram transferidas para o programa Image J 1.34n e analisados os parâmetros de altura da fenda palpebral em posição primária do olhar e distância margem-sulco palpebral. Foram avaliadas as correlações dessas medidas com a satisfação do paciente após 90 dias de pós-operatório. Resultados: O estudo mostrou um aumento da distância margem-sulco palpebral após blefaroplastia superior e uma correlação fortemente positiva (0,64) entre o aumento dessa medida e a nota de avaliação atribuída pelo paciente à cirurgia. Não houve diferença estatisticamente significante na altura da fenda palpebral antes e após a cirurgia. Conclusão: A utilização da medida da distância margem-sulco pode servir como parâmetro quantitativo de um bom resultado estético e funcional, apresentando uma forte correlação com a satisfação dos pacientes no pós-operatório.

Assessment of the Abbreviated National Eye Institute Visual Function Questionnaire (NEI VFQ 9) in blepharoptosis and dermatochalasis.

PURPOSE: To evaluate the Abbreviated National Eye Institute Visual Function Questionnaire (NEI VFQ 9), which is shorter than those previously published, as a tool for assessing vision-related quality of life in patients with ptosis and dermatochalasis. METHODS: This is a cohort study of 46 patients who underwent blepharoptosis and/or upper eyelid blepharoplasty surgery by a single surgeon (CN) in 2013 in a public, academic, ambulatory care referral center. Patients included 29 who underwent blepharoplasty, 11 who underwent ptosis surgery, and 6 who underwent combined surgery. The NEI-VFQ 9 was administered pre- and postoperatively, and the composite scores were compared using Student's t-test. Survey duration was timed in a subset of patients. The hypothesis was that the NEI VFQ 9 could detect a statistically significant improvement in composite score after surgical intervention. RESULTS: The mean pre- and postoperative NEI VFQ 9 composite scores were 74.9 and 86.8, respectively, in the blepharoplasty-only group (P<0.0001), 72.07 and 86.41, respectively, in the ptosis-only group (P=0.004), and 75.8 and 87.2, respectively, in the combined group (P=0.022). There was no correlation between the gain in composite score and the change in upper eyelid margin to reflex distance. Twenty-five patients were timed filling out the survey, and the mean was 2.3 min. CONCLUSIONS: The NEI VFQ 9 consistently demonstrates a significant increase in visual function for blepharoptosis and dermatochalasis patients. Thus, it may be a useful tool for assessing vision-related quality of life in patients with ptosis and dermatochalasis.

The effect of upper eyelid blepharoplasty on eyelid and brow position.

This article evaluates the effect of upper eyelid blepharoplasty on eyelid margin position and brow height. This study is a retrospective analysis of patients who underwent upper eyelid blepharoplasty without concurrent blepharoptosis repair or brow surgery. The medical records of the participants were retrospectively reviewed and an established image analysis software was used to quantify the upper margin reflex distance (MRD1) as well as brow height using high quality standardized clinical photographs. A total of 19 patients (38 eyelids and brows) met the inclusion criteria. The mean preoperative MRD1 was 2.8 mm, and the mean post-operative MRD1 was 3.5 mm, revealing an increase of MRD1 from upper blepharoplasty alone of 0.7 mm (p = 0.0001). The mean preoperative brow position was 17.5 mm above the pupil, and the mean post-operative position was 17.4 mm, for an average change of position of -0.2 mm (p = 0.39) following upper eyelid blepharoplasty. Upper eyelid blepharoplasty without ptosis surgery results in a statistically significant increase in MRD1. Brow position does not demonstrate a statistically significant change in patients who undergo upper eyelid blepharoplasty for simple dermatochalasis.

Assessment of the Abbreviated National Eye Institute Visual Function Questionnaire (NEI VFQ 9) in blepharoptosis and dermatochalasis / Avaliação do Questionário Abreviado de Função Visual do National Eye Institute (NEI VFQ 9) em blefaroptose e dermatocálase

ABSTRACT Purpose: To evaluate the Abbreviated National Eye Institute Visual Function Questionnaire (NEI VFQ 9), which is shorter than those previously published, as a tool for assessing vision-related quality of life in patients with ptosis and dermatochalasis. Methods: This is a cohort study of 46 patients who underwent blepharoptosis and/or upper eyelid blepharoplasty surgery by a single surgeon (CN) in 2013 in a public, academic, ambulatory care referral center. Patients included 29 who underwent blepharoplasty, 11 who underwent ptosis surgery, and 6 who underwent combined surgery. The NEI-VFQ 9 was administered pre- and postoperatively, and the composite scores were compared using Student's t-test. Survey duration was timed in a subset of patients. The hypothesis was that the NEI VFQ 9 could detect a statistically significant improvement in composite score after surgical intervention. Results: The mean pre- and postoperative NEI VFQ 9 composite scores were 74.9 and 86.8, respectively, in the blepharoplasty-only group (P<0.0001), 72.07 and 86.41, respectively, in the ptosis-only group (P=0.004), and 75.8 and 87.2, respectively, in the combined group (P=0.022). There was no correlation between the gain in composite score and the change in upper eyelid margin to reflex distance. Twenty-five patients were timed filling out the survey, and the mean was 2.3 min. Conclusions: The NEI VFQ 9 consistently demonstrates a significant increase in visual function for blepharoptosis and dermatochalasis patients. Thus, it may be a useful tool for assessing vision-related quality of life in patients with ptosis and dermatochalasis.

RESUMO Objetivo: Avaliar o Questionário Abreviado de Função Visual do National Eye Institute (NEI VFQ 9) como uma ferramenta para avaliar a visão relacionada com a qualidade de vida (QoL) em pacientes com blefaroptose e dermatocálase. Método: Estudo de coorte de blefaroptose e dermatocálase pacientes tratados por um único cirurgião (CN) em 2013. O acompanhamento foi de 2-3 meses. Pacientes em um centro de referência acadêmico para a atenção pública ambulatorial com ptose funcional e/ou dermatocálase de pálpebra superior foram avaliados prospectivamente utilizando o NEI-VFQ 9. Quarenta e seis pacientes completaram o estudo: 29 pacientes se submeteram somente à blefaroplastia, 11 apenas à cirurgia de ptose, e 6 pacientes à cirurgia combinada. Foram excluídos pacientes com outra cirurgia simultânea da pálpebra. O teste foi repetido em uma visita pós-operatória. A duração teste foi cronometrada em um subgrupo de pacientes. Escores compostos pré e pós-operatórios foram comparados com o teste T de Student. O desfecho principal foi a pontuação composta. Os autores propõem que a NEI VFQ 9 seria capaz de detectar uma melhora estatisticamente significativa na pontuação composta após a intervenção cirúrgica. Resultados: No grupo de blefaroplastia apenas, o NEI VFQ 9 apresentou a média composta de 74,9 e 86,8 no pré e pós-operatório, respectivamente (p<0,0001). Para os pacientes submetidos apenas à cirurgia de ptose, a pontuação média composta foi 72,07 e 86,41, no pré e pós-operatório, respectivamente (p=0,004). No grupo de cirurgias combinadas combinação, a pontuação composta média pré-operatória foi de 75,8, e a pós-operatória foi de 87,2 (p=0,022). Não houve correlação entre o ganho de pontuação composta e a mudança no MRD1. Vinte e cinco pacientes foram cronometrados ao preencher o teste, a média foi de 2,3 minutos. Conclusões: O NEI VFQ 9 demonstra consistentemente um aumento na função visual para pacientes submetidos à cirurgia de blefaroptose e dermatocálase, sendo um instrumento de pesquisa mais curto do que daqueles publicados anteriormente.

Scheimpflug imaging in dermatochalasis patients before and after upper eyelid blepharoplasty.

PURPOSE: The aim of this study was to evaluate corneal parameters obtained by Scheimpflug imaging of dermatochalasis patients both preoperatively and after blepharoplasty. MATERIALS AND METHODS: Sixty eyes of 30 patients (22 female, 8 male), which were operated upon for the upper eyelid, were included in the study. Patients were divided into two groups according to preoperative upper margin reflex distance (MRD): MRD < 2.5 mm (Group 1) and MRD ≥ 2.5 mm (Group 2). Preoperative and postoperative (third-month) central corneal thickness, anterior chamber depth, steepest keratometric reading, and astigmatic power vectors were the main outcomes. RESULTS: Postoperatively, the keratometric value of the steepest meridian increased significantly in Group 1 (p = 0.018). Preoperatively and postoperatively, J0 and J45 astigmatic power vectors were similar in each group (in Group 1, for J0 p = 0.20 and for J45 p = 0.67; in Group 2, for J0 p = 0.90 and for J45 p = 0.75). The groups were similar regarding the changes in Ksteep, in astigmatic power vector J0 naJ45 (p = 0.11, p = 0.24, p = 0.55, respectively). CONCLUSIONS: The repositioning of the upper eyelid does not lead to significant changes in visual acuity, steepest keratometric reading, anterior chamber depth, central corneal thickness, or astigmatic power vector of J0 and J45. The only parameter that was significantly different from the preoperative value was the steepest keratometry reading in Group 1 patients. According to our results, blepharoplasty seems not to cause significant changes in the main corneal indices.

Severe aortopathy due to fibulin-4 deficiency: molecular insights, surgical strategy, and a review of the literature.

UNLABELLED: Mutations in the EFEMP2 (alias FBLN4) gene, which encodes the extracellular matrix protein fibulin-4, lead to severe aortopathy with aneurysm formation and vascular tortuosity. The disease phenotype, termed autosomal recessive cutis laxa type 1B (ARCL 1B), is rare among heritable connective tissue diseases but becomes more likely when noting family consanguinity and loose, inelastic skin in the patient. Our patient presented with an intercurrent illness exacerbating upper airway obstruction due to compression from a large aortic aneurysm. Genetic testing eventually revealed the causative mutation. She was initially treated with an angiotensin II receptor blocker and beta-blocker and eventually underwent total thoracic aortic replacement via a two-stage elephant trunk-type procedure. She recovered well and is currently asymptomatic but will require lifetime follow-up due to residual vascular tortuosity and aneurysm risk. CONCLUSION: Better understanding of the importance of transforming growth factor beta signaling in the pathophysiology of aortopathies such as ARCL 1B has led to targeted medical therapies. Specific surgical techniques can lead to optimal outcomes in these patients.

Oculoplastic approach to congenital cutis laxa syndrome.

UNLABELLED: Cutis laxa is a rare congenital or acquired disorder of elastic tissue, characterized by loose skin with folds and multiple internal organ involvement, which may cause life-threatening complications. We present a patient with cutis laxa syndrome who had cross eyelids with esotropia. Bilateral lateral canthal tendon repositioning and bilateral medial rectus recession procedures were performed in a single session. The patient had acceptable eyelid and globe cosmesis after the procedure. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors http://www.springer.com/00266 .

Marked bilateral lower eyelid ectropion in cutis laxa: a paraneoplastic process in multiple myeloma.

PURPOSE: To describe a bilateral involutional lower eyelid ectropion in a patient with cutis laxa, a paraneoplastic process in multiple myeloma. DESIGN: Case report. RESULTS: A 60-year-old male presented with a marked involutional left lower eyelid ectropion. Systemic history included cutis laxa, a paraneoplastic feature of multiple myeloma. After surgical treatment, the ectropion recurred; furthermore, a right lower eyelid ectropion developed. In addition, a distinct dermatochalasis of both upper eyelids was present. CONCLUSIONS: This case is the first description of a marked bilateral involutional ectropion in cutis laxa acquisita.

Extended infrabrow excision blepharoplasty for dermatochalasis in asians.

OBJECTIVE: To describe extended infrabrow excision blepharoplasty (IBEB), whereby skin excision is extended to a substantial part of intrabrow skin and the intrabrow incision is made perpendicular to the hair shaft. METHODS: A total of 194 Asian patients with moderate to severe dermatochalasis underwent extended IBEB. The mean width of excised skin at its widest was 12.8 mm (range, 6-22 mm). RESULTS: Extended IBEB significantly reduced eyelid laxity but produced a natural-looking eyelid because it did not damage the eyelid framework. With application of eyebrow makeup by women, routine social activity was resumed soon after surgery. Infrabrow scarring became inconspicuous in patients with thick eyebrows after regrowth. CONCLUSIONS: Extended IBEB is recommended for middle-aged and older Asian women with moderate to severe dermatochalasis. With precise incision and fine suturing, regrowth alleviates eyebrow reduction and scarring among Asians who do not use makeup (male patients and young female patients).

Acquired localized cutis laxa of the face: a rare presentation.

We report a rare case of acquired localized cutis laxa in a teenage boy, without any preceding skin lesions. The area affected was the midface, extending to the chin, and involving the ears, leading to a prematurely aged appearance. Only five such cases have been previously published in the literature.

Anesthesia considerations for patients with de Barsy syndrome.

STUDY OBJECTIVE: To determine anesthetic considerations for patients with de Barsy syndrome, a rare complex whose hallmark findings include cutis laxa, progeria, and multiple orthopedic and ophthalmologic abnormalities. DESIGN: Retrospective chart review. SETTING: Medical center. MEASUREMENTS: A search of Mayo Clinic medical records from 1968 to 2007 identified two patients with de Barsy syndrome who underwent a combined total of 35 anesthetics for diagnostic and surgical procedures. Data collected included: age, gender, ASA physical status, relevant comorbidities, surgical procedures, airway management, vascular access, monitoring, anesthetic induction, maintenance, and other observations. MAIN RESULTS: A wide range of anesthetics and techniques were used. Apart from 4 episodes of intraoperative hyperthermia and postoperative tachycardia, no complications were noted. These episodes may be similar to the nonmalignant hyperthermia reported in osteogenesis imperfecta and Costello syndrome patients. CONCLUSIONS: While the safety of any anesthetic technique cannot be established or extrapolated from a small series, given the extreme rarity of the syndrome, these cases suggest the relative safety of anesthesia in de Barsy syndrome patients.

Tissue tightening technologies.

Results with current skin-tightening technologies are mild to moderate and are not intended to replace surgical procedures. Many patients will choose more subtle tightening to avoid the risks and downtime associated with surgery. Newer treatment protocols have improved treatment predictability and the extent of efficacy, and novel technologies with the potential for even greater results are currently in development. Overall patients' satisfaction can be increased by combining skin tightening with complementary, noninvasive skin treatments, such as botulinum toxin and soft-tissue fillers.

Toxic dermatolysis, tissue necrosis and impaired wound healing due to sunitinib treatment leading to forefoot amputation.

Innovative treatment strategies in urologic oncology confront the treating physician with a new spectrum of adverse events. With growing understanding of underlying pathomechanisms, we need to identify contraindications against the use of certain antiproliferative drugs. The management of toxicities involves a multidisciplinary approach and thus, the exchange of experience across medical specialties is mandatory. We report a case of fulminant toxic dermatolysis, tissue necrosis and impaired wound healing resulting in the amputation of one forefoot after 6 days of treatment with sunitinib.

Conjunctival cytology in floppy eyelid syndrome: objective assessment of the outcome of surgery.

AIMS: Upper eyelid shortening is the main surgical procedure in floppy eyelid syndrome (FES). The efficacy of surgery is assessed by improvements in the symptoms, since objective evaluation is difficult. Conjunctival impression cytology was assessed as an objective method for the evaluation of the effectiveness of surgery for FES. METHOD: The study was a prospective interventional study in 16 patients (26 eyelids) with FES, who were enrolled over a period of 8 months. Patients were examined 1-4 weeks before surgery and 16 weeks after surgery. Cases were classified into three groups according to the severity of symptoms and papillary reaction. A full-thickness pentagonal wedge resection was performed in the upper eyelid. Impression cytology was performed in all patients at 1-4 weeks before surgery and 16 weeks after surgery. RESULTS: Postoperative improvements in cellular morphology and goblet cell count were found in 20 of 22 eyelids (91%) and this led to a decrease of least one grade in Nelson's classification. In two cases (9%) the Nelson grade remained stable. Postoperative improvement on cytology was statistically significant with the Wilcoxon signed ranks test (p<0.001). CONCLUSION: Conjunctival cytology provides an objective method for the evaluation of the efficacy of surgical techniques proposed for the treatment of eyelid laxity syndromes.