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Ophthalmic findings in scleromyxedema.

PURPOSE: To catalog ophthalmic findings in a cohort of patients with scleromyxedema. METHODS: Thirty-five biopsy-proven patients with scleromyxedema evaluated at the Mayo Clinic in Rochester, Minnesota, from 1960 to 1991 were identified. Eye examinations were performed on 17 of the patients. Ophthalmic findings not attributable to other systemic or ocular disease were recorded. RESULTS: The following abnormalities were considered secondary to scleromyxedema: corneal opacities (2 patients), thickened eyebrow or eyelid skin (4 patients), lagophthalmos (1 patient), and ectropion (2 patients). One patient who had concurrent polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome also had choroidal folds and papilledema. A corneal biopsy in one patient disclosed deposits of acid mucopolysaccharide, consistent with one of two previously published cases. CONCLUSION: A series of patients with scleromyxedema was reviewed. This systemic disorder infrequently may cause visually significant ophthalmic manifestations, including eyelid changes and corneal deposits. Further study is needed to characterize better the nature of the corneal opacities.

Adult↗

Endophthalmitis after secondary intraocular lens implantation. A case-report study.

PURPOSE: To investigate whether endophthalmitis is more common after secondary intraocular lens (IOL) implantation than after other intraocular surgeries. Risk factors, causative organisms, and visual acuity outcomes of endophthalmitis after secondary IOL implantation were investigated. METHODS: The ten cases consisted of all patients treated at the Bascom Palmer Eye Institute between January 1, 1984, and December 31, 1994, for endophthalmitis after secondary IOL implantation. The 34 control patients consisted of all patients who underwent secondary IOL implantation within 3 years of the case patients (6-year span) by the same surgeons but who did not develop endophthalmitis. Demographic and clinical data were abstracted from patients' medical records. RESULTS: Fifty percent (5 of 10) of case patients had a history of diabetes mellitus, compared with 5.9% (2 of 34) of control patients. All three posterior chamber IOLs among case patients had transscleral suture fixation, whereas all four posterior chamber IOLs among control patients were in the sulcus without scleral sutures. Case patients were more likely than control patients to have IOLs with polypropylene haptics (30.0% and 5.9%, respectively) and to have had their IOLs placed through a superior rather than a temporal incision (70.0% and 17.9%, respectively). Eighty percent of case patients and 14.7% of control patients had either eyelid abnormalities (marked blepharitis, ectropion) or postoperative wound defects. Staphylococcus epidermidis was isolated in 50% of case patients. After treatment, 90.0% of case patients achieved a visual acuity of greater than or equal to 20/100. CONCLUSION: Endophthalmitis after secondary IOL implantation was associated with diabetes, transscleral suture fixation of posterior chamber IOLs, polypropylene haptics, preoperative eyelid abnormalities, re-entry of eye through a previous wound, and postoperative wound defects. Most cases were caused by Staphylococcus epidermidis, and the post-treatment visual outcomes were generally good.

Aged↗

Involutional lower lid entropion: to shorten or not to shorten?

OBJECTIVE: Involutional entropion of the lower eyelid is a common problem in the aging population, and manifest horizontal laxity is often present. The authors therefore examined the cure rate, dependent on whether the lid had been shortened horizontally. DESIGN: A retrospective case series. PARTICIPANTS: Five hundred eighty-three surgical records of entropion surgery at Moorfields Eye Hospital over a 4-year period (1993-1996, inclusive) were examined, and those patients with involutional entropion and adequate follow-up data were selected. INTERVENTION: One hundred eighty of the 313 primary procedures included horizontal shortening, as did 28 of the 47 reoperations for recurrent entropion or consecutive ectropion. MAIN OUTCOME MEASURES: Surgical success was analyzed after primary correction or after reoperation, and the groups were compared with respect to age, gender, and length of follow-up. RESULTS: A cure after primary surgery was achieved in 178 (99%) of 180 patients in whom the lower eyelid was shortened compared with 104 (78%) of 133 patients in whom the eyelid was not shortened (P < 0.001). Reoperation for recurrent eyelid malposition cured 28 (100%) of 28 patients if the eyelid was shortened and 12 (63%) of 19 patients if the eyelid was not shortened (P < 0.001). CONCLUSIONS: Recurrent malposition of the eyelid was significantly more likely when horizontal eyelid shortening was not included at either primary repair or at reoperation for recurrence or overcorrection. As horizontal laxity is probably the main pathogenic factor for age-related entropion, it is doubtful whether surgical correction without horizontal shortening of the eyelid has any role in the treatment of this condition.

Aged↗

Ophthalmic abnormalities in patients with cutaneous T-cell lymphoma.

OBJECTIVE: To determine the frequency of ophthalmic abnormalities in patients with cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome) and T-cell lymphoma involving the skin and to describe the clinical course of the disease with selected examples. DESIGN: Retrospective, clinic-based, cross-sectional study. PARTICIPANTS: A computerized diagnostic retrieval system was used to identify all patients with T-cell lymphoma involving the skin who were examined at the Mayo Clinic (Rochester, Minnesota) between January 1, 1976 and December 31, 1990. The medical records of affected patients were reviewed. MAIN OUTCOME MEASURES: Identification of ophthalmic abnormalities. RESULTS: During the 15-year interval from 1976 through 1990, cutaneous T-cell lymphoma was diagnosed in 2155 patients. Of these, 42 (1.95%; 26 male and 16 female) had at least one ophthalmic abnormality attributable to the disease. The diagnoses in these 42 patients were mycosis fungoides in 19, clinical variants of T-cell lymphoma of the skin (most commonly peripheral T-cell lymphoma) in 11, and Sézary syndrome in 12. Cicatricial eyelid ectropion was the most common finding, affecting 17 (40.4%) of the 42 patients. Thirty-seven patients had findings that, although probably not a direct consequence of cutaneous T-cell lymphoma, have been cataloged in previous studies. CONCLUSION: Although ophthalmic abnormalities in patients with cutaneous T-cell lymphoma are relatively uncommon, the manifestations of the disease are diverse and frequently difficult to treat.

Adult↗

[Lower lid blepharoplasty].

Baggy lower eyelids result from herniated orbital fat, excess skin, hypertrophic or lax orbicularis sometimes associated with lower lid laxity resulting from degeneration of the lateral canthal tendon with age. Two main approaches are used: skin-muscle flap or the transconjunctival approach in younger patients who have herniated orbital fat with minimal or not excessive lower eyelid skin. Preoperative evaluation of the patient is extremely important in lower lid blepharoplasty before any surgical procedure: excessive skin, herniated orbital fat, retraction and laxity must be evaluated. In our practice, a lateral canthoplasty is often performed in case of horizontal lower laxity in order to avoid round eye, scleral show or lower lid ectropion. In some patients, subperiosteal elevation of malar soft tissue is associated with lower blepharoplasty via subciliary incision.

Blepharoplasty↗

Surgical management of lagophthalmos in patients with facial palsy.

PURPOSE: A prospective before-and-after trial was designed to evaluate the role of upper-lid gold weight implantation and lower lid lateral canthoplasty in the management of patients with paralytic lagophthalmos. PATIENTS AND METHODS: The study included 40 patients (age range 19 to 72, mean age 46.8), and gold weights varying from 0.6 to 1.6 g were implanted in all 40 patients. Lateral canthoplasty was performed in 14 of the patients who suffered from variable degrees of lower lid laxity. Mean follow-up period was 15.7 months (range 9 to 38). RESULTS: Complete correction of lagophthalmos and/or ectropion with resolution of preoperative symptoms was achieved in 37 of 40 patients (92.5%), and spontaneous extrusion of the gold weight occurred in only one patient (2.5%). CONCLUSION: Excellent results were achieved in the management of paralytic lagophthalmos with upper-lid gold weight insertion, and simultaneous lateral canthoplasty proved to be very helpful in patients with significant hypotonia of lower lid.

Adult↗

Facial reconstruction with latissimus dorsi myocutaneous island flap following total maxillectomy.

Following total maxillectomy for maxillary cancer, facial reconstruction was performed using a latissimus dorsi myocutaneous island flap. Postreconstructive deformity was studied in 10 patients. In 5 patients, after simple total maxillectomy the inner raw surface of the facial skin and orbito was covered by the flap, and the other extended total maxillectomy patients where the orbital contents and facial skin were involved, reconstruction was by means of the folded flap. In the patients with simple total maxillectomy, cicatricial contracture of the facial skin and cicatricial ectropion of the lower eyelid were quite small, and in the patients with extended total maxillectomy, reconstructed facial skin did not give rise to cicatricial contracture.

Cicatrix↗

Clinical and histopathologic findings of iris nevus (Cogan-Reese) syndrome.

PURPOSE: To report a case of Cogan-Reese syndrome. METHOD: Case report. A 37-year-old man presented with Cogan-Reese syndrome. RESULTS: Visual acuity was 0.5 in the right eye and 1.0 in the left eye. There were corneal edema and pigmented nodules on the anterior surface of the iris, iris atrophy and ectropion uvea in the right eye. The intraocular pressure was 42 mmHg in the right eye and there was glaucomatous optic atrophy of the optic disk. Trabeculectomy with mitomycin C has been performed as the intraocular pressure did not decrease with the maximum medical treatment. Electron microscopic examination of the trabeculum and the iris tissue revealed a lot of melanocytic cells in the stroma. CONCLUSION: Trabeculectomy with mitomycin C might be effective in Cogan-Reese cases with glaucoma resistant to medical treatment.

Adult↗

The management of congenital malpositions of eyelids, eyes and orbits.

Congenital malformations of the eye and its adnexa which are multiple and varied can affect the whole eyeball or any part of it, as well as the orbit, eyelids, lacrimal ducts, extra-ocular muscles and conjunctiva. A classification of these malformations is presented together with the general principles of treatment, age of operating and surgical tactics. The authors give some examples of the anatomo-clinical forms, eyelid malformations such as entropion, ectropion, ptosis, levator eyelid retraction, medial canthus malposition, congenital eyelid colobomas, and congenital orbital abnormalities (Craniofacial stenosis, orbital plagiocephalies, hypertelorism, anophthalmos, microphthalmos and cryptophthalmos).

Eye Abnormalities↗

Management of peri-ocular skin tumours by laissez-faire technique: analysis of functional and cosmetic results.

AIM: The role of wound healing by secondary intention in the treatment of peri-ocular skin tumours is not well established. The object of this retrospective analysis was to evaluate the functional and cosmetic outcome of patients treated by the Laissez-faire technique in situations where primary closure would not have been possible. METHODS: Skin defects following excision of lid and peri-ocular tumours in 24 Caucasian patients were allowed to heal by granulation. The locations included lower eyelid (n = 10), upper lid (n = 6), medial canthus (n = 5), nasojugal fold (n = 2), lateral canthus (n = 1) and brow (n = 1). Four patients had lid margin involvement. The size of the initial defect, time taken to heal, discomfort during healing, the functional and cosmetic results-both from the surgeon and patient perspective, complications, secondary intervention if any and patient satisfaction were studied. RESULTS: A good functional and cosmetic result was obtained in 23 of the 25 lesions (92%). Of these 23 patients, two patients had slightly hypertrophied scars, which responded well to massage and two patients had some degree of ectropion. Of the two patients who did not have a good cosmetic result, only one needed secondary intervention. One had an exuberant granulation tissue, which responded to topical steroids and massage, but left behind a distorted lateral canthus. CONCLUSION: Healing by secondary intention of large defects following excision of peri-ocular tumours is an effective alternative to primary or staged reconstruction in selected cases.

Adult↗

Early division of a modified Cutler-Beard flap with a free tarsal graft.

AIMS: We describe a variation of the Cutler-Beard flap in the reconstruction of upper eyelid defects. METHODS: The technique of upper eyelid reconstruction with a free tarsal graft and a cutaneous lower eyelid advancement flap divided at 2 weeks is described. Four cases where this technique was used for reconstruction of eyelid defects due to periocular malignancy are also reported. RESULTS: There were three male and one female patients ranging in age from 61 to 78 years. The underlying diagnoses were squamous cell carcinoma, trichilemmal carcinoma, and two basal cell carcinomas. Follow-up of 6-28 months revealed a good outcome in all four cases with one patient developing a mild cicatricial lower eyelid ectropion that was managed conservatively. CONCLUSIONS: A modified Cutler-Beard flap with free tarsal graft and early division may provide an effective alternative for upper eyelid reconstruction in cases with sufficient lower eyelid skin laxity.

Aged↗

Generalized eruptive keratoacanthoma (Grzybowski variant).

We describe a patient with generalized eruptive keratoacanthoma (KA) of Grzybowski showing the characteristic features of this extremely rare condition. Since the first description by Grzybowski in 1950, only 28 additional cases (including the present one) have been reported. This variety of KA most commonly affects patients during the fifth to seventh decade of life and appears as a generalized eruption of hundreds to thousands of follicular papules. The small pruriginous papules often have a keratotic centre and show microscopic features of KA. Marked facial involvement is characteristic and can lead to masked facies with ectropion, as in our patient. The course of the disease is chronic and the response to therapy is poor.

Acitretin↗

Ultrastructural features resembling those of harlequin ichthyosis in patients with severe congenital ichthyosiform erythroderma.

Congenital ichthyoses are a group of heterogeneous disorders of cornification. Autosomal recessive congenital ichthyosis (ARCI) can be clinically subdivided into congenital ichthyosiform erythroderma and lamellar ichthyosis. Ultrastructurally, ARCI is classified into four groups: ichthyosis congenita (IC) types I-IV. The genetic background of the ARCI disorders is heterogeneous, but only one disease gene, transglutaminase 1, has been detected so far. We describe six patients with severe congenital ichthyosis from six different Scandinavian families. They could not be classified ultrastructurally into the four IC groups because of atypical findings of electron microscopy. These included abnormal lamellar bodies, alterations in keratohyalin, remnant organelles and lipid inclusions in the upper epidermal cells, which resembled the ultrastructural findings of harlequin ichthyosis (HI), although the HI phenotype was not present at birth. Some clinical features, such as thick scales, erythroderma, alopecia and ectropion were common to all patients. Ichthyosis was usually accentuated in the scalp and four patients had clumped fingers and toes. None of the patients carried the transglutaminase 1 mutation. We conclude that ultrastructural findings resembling those detected in previous HI cases (type 1 and 2) can also be found in patients who do not have classic clinical features of that rare ichthyosis. This may be due to lack of specificity of ultrastructural markers for HI or to its clinical heterogeneity.

Adolescent↗

Nine-year follow-up of a case of Grzybowski type multiple keratoacanthomas and failure to demonstrate human papillomavirus.

We describe a patient with a 9-year history of generalized eruptive keratoacanthoma (KA) of the Grzybowski type whose multiple skin lesions showed steady progression, resulting in a sclerotic, mask-like facial expression and ectropion. Eleven tumour biopsies representing lesions of different stages and localizations (erupting and regressing KAs, biopsies from non-involved light-protected and light-exposed skin, dermatosclerosis and squamous cell carcinomas) were analysed for human papillomavirus (HPV) sequences using a polymerase chain reaction approach capable of detecting the majority of all presently known HPV genotypes. None of the biopsy specimens proved to be HPV-positive, although HPV was detected in weakly and heavily affected control specimens by the method applied. These findings suggest an HPV-independent aetiology of this rare type of multiple KA.

Aged↗

Lamellar ichthyosis associated with pseudoainhum of the toes and eye changes.

Classic lamellar ichthyosis is a rare, autosomal recessive, genetically heterogeneous skin disease caused by mutations in the transglutaminase 1 gene. Pseudoainhum is characterized by the appearance of a constricting band around a digit which may lead to spontaneous amputation. We describe a 41-year-old man with classic lamellar ichthyosis with unusual eye changes and pseudoainhum of both the fifth and the third right toes. Eye abnormalities included bilateral ectropion of the lower eyelids, chronic blepharitis, and nuclear cataract. A radiometric assay revealed greatly reduced skin transglutaminase activity. To the best of our knowledge, this report is unique as classic lamellar ichthyosis with deficient transglutaminase activity has never been associated with pseudoainhum of the toes and the early development of nuclear cataract.

Adult↗

Mycosis fungoides causing severe lower eyelid ulceration.

Mycosis fungoides is a form of cutaneous malignant T-cell lymphoma initially confined to the skin. A range of ophthalmic manifestations has been described; these usually appear in advanced disease. Lid involvement is most common and ranges from ectropion, usually cicatricial, to diffuse thickening and placoid tumours. A case of a 72-year-old man presenting with progressive, full thickness, lower eyelid ulceration is presented. The patient had a long history of placoid skin lesions, previously diagnosed as discoid lupus. Repeat biopsies of these lesions including the eyelid eventually revealed mycosis fungoides. The ulceration progressed to destroy most of the lower lid before the correct diagnosis was made. This type of eyelid involvement has not previously been reported in mycosis fungoides. This case also highlights the diagnostic difficulties encountered in the early phases of mycosis fungoides. If suspected, multiple biopsies are essential to establish the diagnosis.

Aged↗

Suspension sutures aid facial reconstruction.

BACKGROUND: Suspension, pexing or tacking sutures, which have a long history of use in facial cosmetic surgery, have only recently begun use in reconstruction. OBJECTIVE: In this study, the suspension suture was used to fix the closure line at the junction of cosmetic units, to prevent distortion of free margins secondary to wound contraction, eg, ectropion or eclabium, to prevent tenting across concavities, and to prevent scar spreading of wounds closed under tension. The acute complications and long-term effectiveness of this suturing technique in achieving the 4 desired results were evaluated. METHODS: During a 10-year period, 136 cases of surgical defects of the face created by the removal of nonmelanoma skin cancer by Mohs micrographic surgery were repaired using absorbable suspension sutures. The wounds were evaluated initially for acute complications of hemorrhage, infection, dehiscence, and necrosis; and later over 3 years for chronic sequela of hypertrophic scar, spread scar, contraction deformities such as distortion of free margins, and pigmentary changes. RESULTS: Suspension sutures were used in 60 cases of primary layered closure and 76 flaps of the face. Suspension sutures allowed primary closure of defects that would have required flaps in 72% of the cases and prevented distortion of free margins. There were more acute and long-term complications related to excess tension with primary closure than with flaps. Suspension sutures fixed the closure line at the junction of cosmetic units, prevented tenting across concavities, and both features were maintained over the duration of the study. CONCLUSION: Suspension sutures aided in obtaining linear closures, prevented distortion of free margins, prevented tenting across concavities, and fixed the closure line at the junction of cosmetic units; however, they did not prevent spread scars.

Face↗

The use of combined nasolabial V-Y advancement and glabellar flaps for large medial canthal defects.

BACKGROUND: Reconstruction of the medial canthal area presents many challenges for surgeons. It has many functional and cosmetic peculiarities. The combined use of glabellar and nasolabial V-Y advancement flaps is an effective method for repairing large medial canthal defects. OBJECTIVE: To describe and illustrate a technique to repair medial canthal defects. METHODS: Our experience with combined usage of nasolabial V-Y flap and glabellar flaps for medial canthal closure after tumor resection in 23 patients is reported. RESULTS: All resections and reconstructive procedures were performed under local anesthesia. All patients were treated without any complications such as flap loss, wound dehiscence, lymphedema, or ectropion. Fine results were achieved from both the functional and aesthetic point of view. CONCLUSION: This combination has many advantages over skin grafts and other local flaps. It is easy to design, reliable, and offers good cosmetic results. This combination is recommended to plastic surgeons as a safe and effective procedure for reconstruction of medial the canthal area.

Basal Cell Carcinoma↗