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Genetic study and surgical correction of euryblepharon.

Euryblepharon is a rare congenital anomaly of the palpebral fissure which consists of an increase in its width. A case has been described in which this characteristic is very marked, and associated with ectropion, lagophthalmos, corneal ulcers; also congenital malformations as twisted feet, hypospadia and inguinal hernia. Apart from a limited increase of the palpebral fissure in the mother, the familiar anamnesis is negative, and likewise examination of the cariogram and research of eventual metabolic modifications. An estimation of the various surgical techniques proposed revealed insufficient strictness regarding the standards generally followed in palpebral plastic surgery. Consequently, a technical variation was set up that will satisfy the needs of correct anatomical-surgical practice.

Abnormalities, Multiple↗

Early gold weight eyelid implantation for facial paralysis.

Facial paralysis can result in serious keratopathy because of corneal exposure and inadequate lacrimation. Thirty-seven patients underwent thirty-eight gold weight upper lid implants to rehabilitate paralysis of the eyelid from various causes. When indicated, implantation was combined with lower lid ectropion repair, medial canthoplasty or brow lift. Because of encouraging results in patients with longstanding facial paralysis, "early" implantation (within 1 month of paralysis) was offered to patients with severe lagophthalmos in whom (1) a severe neural injury was documented at the time of transtemporal surgery or (2) delayed, incomplete return of function was expected. Gold weight implantation resulted in excellent eyelid closure, protection, and cosmesis. There were no infections or extrusions. Lagophthalmos and exposure keratitis resolved or were significantly improved in all patients, and most were able to dispense with eyedrops and salves. Visual acuity improved in 95% of patients--a benefit even those without preoperative keratitis often achieved. A mild worsening of one patient's pre-existing astigmatism developed, which resolved after reimplantation with a lighter weight. The implant is easily removed from those patients who, having undergone early implantation, eventually recover adequate function. Gold weight loading has become our procedure of choice for eyelid rehabilitation.

Adolescent↗

Blepharo-cheilo-dontic (BCD) syndrome: case report.

OBJECTIVE: To report a case of blepharo-cheilo-dontic (BCD) syndrome, to compare with the previous cases, and to discuss the possible treatment. PATIENT: The patient was a 14-day-old boy born uneventfully on August 7, 2002. His scalp hair was sparse and curly. A tumor on the top of his head, which was thought to be a dermoid cyst, and a nevus on the back of his left hand were observed. All typical symptoms of blepharo-cheilo-dontic syndrome, such as euryblepharon with ectropion of the lower eyelids, distichiasis, bilateral cleft lip and palate, severe oligodontia, and conical teeth, were observed. Because there was no fatal complication, and growth and development were about normal, it was recommended that a long and active treatment plan be considered for this patient.

Cleft Lip↗

Ophthalmic abnormalities in patients with cutaneous T-cell lymphoma.

PURPOSE: 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. METHODS: 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. RESULTS: During the 15-year interval from 1976 through 1990, cutaneous T-cell lymphoma was diagnosed in 2,155 patients. Of these 2,155 patients, 42 (1.95%; 26 male and 16 female) had at least 1 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↗

Acute and reconstructive management of the burned eyelid.

Introduced in 1875, the use of full-thickness skin graft for release of a lower eyelid ectropion secondary to burn contractures remains a mainstay of current burn treatment. The authors address issues such as acute care, the exposed cornea, scar management, and surgical management of eyelid deformities.

Burns, Chemical↗

Grzybowski's generalized eruptive keratocanthomas: a case report.

A 47-year old woman with Grzybowski's generalised eruptive keratoacanthomas is described. There was no history of skin disease in her family, except for an uncle's basal cell carcinoma. From 1995 she developed multiple lesions of various size, ranging from hundreds of small follicular lesions to large typical keratoacanthomas up to 5 cm in diameter, scleroderma-like facial skin and marked ectropion. Histological examination of small and large skin lesions was typical of keratoacanthoma, and no human papillomavirus was detected by polymerase chain reaction. Oral treatment with acitretin had no effect. Both cyclophosphamide and methotrexate therapy were refused by the patient despite the progressive course of the disease. Blepharoplastic surgery had some effect on eye symptoms. The etiology of this rare disease is unknown, but is probably related to some genetic defect.

Acitretin↗

Chemical injuries to the eye in Benin City, Nigeria.

Chemical injury to the eye is still an important cause of blindness and serious complications in Benin City, Nigeria. Twelve patients were seen with chemical injury to the eye over a five year period. They were all male patients. Ten were assault cases and two were accidental cases. The most common injurious agent was ammonia. Complications seen were corneal opacification in ten patients, symblepharon in nine patients, entropion, ectropion, cataract and staphyloma developed in some of the patients. There were four cases of bilateral blindness and six cases of unilateral blindness. Late presentation to hospital and failure to initiate adequate first aid treatment were contributing factors to the poor outcome. Health education of the public about the importance of prompt, prolonged irrigation of the chemically injured eye and early presentation to the hospital is advised.

Accidents↗

[Adjustable suture for marginal lid malpositions].

PURPOSE: Inversing and eversing sutures may be a treatment for inferior eyelid entropion and ectropion, as single treatment or combined with other surgical technique. We propose the use of adjustable sutures for these patients. MATERIAL AND METHODS: 85 cases were considered. 41 of them were treated with adjustable sutures and the other 44 underwent classical sutures as treatment. RESULTS: The 100% of the eyelids treated with adjustable sutures had a successful eyelid surgery. This was observed at the end of the procedure, the fourth day after surgery, and after the sixth month. CONCLUSIONS: These adjustable sutures provide more patient comfort and a more accurate result than the classical technique at least after six months of follow up.

Aged↗

Surgical options for eyelid problems.

BACKGROUND: Eyelid problems may be vision threatening, painful, irritating, cause watering, or just unsightly. Many are treatable surgically. OBJECTIVE: This article describes some of the more common eyelid conditions, their presentation, investigation and surgical treatment. DISCUSSION: Common eyelid conditions include ectropion, entropion, ptosis, thyroid eye disease and facial palsy. Treatments, including surgical options, are discussed. Most eyelid surgery on adults can be done as day surgery under local anaesthetic with intravenous sedation.

Adult↗

Lethal arthrogryposis with icthyosis: overlap with Neu-Laxova syndrome, restrictive dermopathy and harlequin fetus.

We describe a stillborn female with joint contractures, subcutaneous oedema, ectropion, a severely flattened nose, an 'O' shaped open mouth and extensive peeling of skin. The head circumference was normal. She was born at 33 weeks of gestation to consanguineous parents, who had one previous offspring affected with non-immune hydrops fetalis. Pathological, radiological and prenatal findings are reported. The features of the present case are compared with those of Neu-Laxova syndrome, restrictive dermopathy and harlequin fetus.

Arthrogryposis↗

[Cutis laxa syndrome. Case report].

INTRODUCTION: Cutis laxa is a heterogeneous group of connective tissue disorders,characterized by loose skin and variable systemic involvement. The characteristic symptomatological pattern is resulting from paucity of elastic fibers. CASE REPORT: A 4 year-old boy with a congenital cutis laxa was sent by his pediatrician for ophthalmic examination. His examination revealed an ectropion of the right lower lid and an entropion of the left lower lid. His general physical examination showed multiple visceral involvement, with inguino-scrotal hernia, multiple dental caries and severe pulmonary emphysema responsible for death two weeks after. DISCUSSION: Cutis laxa is an extremely rare group of disorders. Congenital and acquired varieties have been described. The association of ocular anomalies has been described in the autosomal recessive form. Through this case report we shall discuss the ophthalmological signs of this disease as well as its clinical and genetic manifestation and its physiopathology.

Child, Preschool↗

[The clinical-and-morphological specificity of using xenopericardium in plasty of the eyelids and orbit].

The authors analyzed the clinical results of transplantation of the xenopericardium in 60 patients made at the Research institute of eye diseases of the Russian academy of medical sciences from 1999 to 2003. Xenomaterial was used in reconstruction of the lower eyelids after trauma (coloboma, defects of ligaments), in ectropions of the lower eyelids, in paralytic lagophthalmos and in traumas of the orbit. The postoperative follow-up of patients ranged from 2 to 54 months (an average of 28 months). Xenopericardia removed from patients (4 cases) were histologically examined in 6 months and more after surgery. The examination was made by the method of serial sagittal paraffin sections tinted with hematoxylin or eosin or according to I. van Gieson. A moderately pronounced tissue response and good remote clinical results confirmed the possibility of using it in reconstructive surgery, in particular, in plasty of the orbit especially when made through a small incision. The peculiarities of using clinically the xenopericardium denote that it can be used in ophthalmoplasty; however, it should be fixed immediately under the skin of the eyelids very carefully in order to avoid the formation of uneven surface.

Animals↗

Microvascular free tissue transfer for treatment of osteoradionecrosis of the maxilla.

Head and neck tumors often require radiotherapy as part of the treatment protocol. Although it improves the survival rate in cancer patients, it may cause osteoradionecrosis, especially in the mandible and maxilla. Twelve patients with osteoradionecrosis of the maxilla were treated with microsurgical free tissue transplantations between April of 1996 and August of 2002. There were 10 male and two female patients, with a mean age of 60.2 years. The mean radiotherapy dose was 6674 cGy. The radiation dose could not be traced in three patients because radiotherapy was performed elsewhere. Radical sequestrectomy, soft-tissue debridement, and pathologic proof of no tumor recurrence were performed before microsurgical reconstruction. Free flaps used included the following: anterolateral thigh (n = 7), radial forearm (n = 2), rectus femoris musculocutaneous (n = 2), and supracondylar chimeric (n = 1) flaps. All flaps survived completely and reconstruction succeeded. During a mean 25-month follow-up period, ectropion, plate exposure, and mild infection were encountered in three patients and treated successfully. Radical debridement and obliteration of dead space with well-vascularized tissue are essential for successful treatment of maxillary osteoradionecrosis. The anterolateral thigh flap is most versatile for almost all types of soft-tissue defect reconstruction in the head and neck region.

Adult↗

Facial cleft syndrome--a case report.

A rare case of facio-orbital anamoly having dermolipoma with posterior extension, lid notch, ectropion, assymetric hypertelorism and lipoma of corpus callosum is presented. The classification of the facial cleft deformities and the surgical management is highlighted.

Adult↗

Ophthalmic management of seventh nerve palsy.

The management of acute and established seventh nerve palsy is discussed with reference to corneal exposure, paralytic ectropion and the cosmetic defect. Details are given of how these can all be improved with relatively simple procedures which can be performed under local anaesthesia as an outpatient.

Corneal Diseases↗

[Collodion baby. Apropos of a case with eye manifestations].

We report a case of a male collodion baby, born at term of a consanguineous couple of Afghan origin. The new-born baby displayed a pseudo-dysmorphic syndrome predominant of the face, with a marked ectropion of the upper and lower eye-lids and chemosis, without corneal involvement. The condition improved with time but complete regression was not obtained. Collodion baby syndrome is a pathologic skin condition observable during the neonatal period. Long term evolution is towards ichthyosis in 90% of cases, and resolution in 10%.

Ectropion↗

[Effectiveness of a topical medical treatment following diathermic coagulation: controlled clinical study].

A controlled clinical study has been conducted on the efficacy of a topic therapy. Kanamicine sulphate daily for 6 days and altered to polidesossiribonucleotide for the following 24 days were given after cervical cauterization for ectropion with or without AnTZ. Treated patients were 148 and untreated ones were 155. Subjective and objective parameters were evaluated. Among the former only bloody discharge (more frequent among the treated patients) and ill-smelling discharge (more frequent among the untreated patients) showed significant differences. Colposcopy instead showed better (or faster) healing of the treated group, with 83.6% vs 62% iodine dark cervices at Schiller test and 87.9% vs 59.7% SCJ at the external os or inside (P less than 0.01). Also disepitelized areas or areas with frail epithelium were less common among the treated patients.

Administration, Topical↗