New Clinical and Immunofluoresence Data of Collagen VI-Related Myopathy: A Single Center Cohort of 69 Patients.

Pathogenetic mechanism recognition and proof-of-concept clinical trials were performed in our patients affected by collagen VI-related myopathies. This study, which included 69 patients, aimed to identify innovative clinical data to better design future trials. Among the patients, 33 had Bethlem myopathy (BM), 24 had Ullrich congenital muscular dystrophy (UCMD), 7 had an intermediate phenotype (INTM), and five had myosclerosis myopathy (MM). We obtained data on muscle strength, the degree of contracture, immunofluorescence, and genetics. In our BM group, only one third had a knee extension strength greater than 50% of the predicted value, while only one in ten showed similar retention of elbow flexion. These findings should be considered when recruiting BM patients for future trials. All the MM patients had axial and limb contractures that limited both the flexion and extension ranges of motion, and a limitation in mouth opening. The immunofluorescence analysis of collagen VI in 55 biopsies from 37 patients confirmed the correlation between collagen VI defects and the severity of the clinical phenotype. However, biopsies from the same patient or from patients with the same mutation taken at different times showed a progressive increase in protein expression with age. The new finding of the time-dependent modulation of collagen VI expression should be considered in genetic correction trials.

Clinical Application of Hyperdry Amniotic Membrane in Cleft Palate Repair.

OBJECTIVE: To examine the safety and efficacy of hyperdry amniotic membrane (HDAM) for wound closure after palatoplasty in cleft palate patients. METHODS: HDAMs were prepared by washing and drying under infrared rays and microwaves at temperatures less than 60°C using a hyperdrying device. A total of 16 cleft palate patients (8 males, 8 females), aged 1 to 3 years (mean age 1 year 9 months), received one-stage pushback palatoplasty. The remaining raw wound after surgery was covered by an HDAM and a plastic cover plate. The cover plate was removed 1 week after surgery and parameters including temperature, feeding, allergic reactions, postoperative bleeding, re-epithelialization, wound dehiscence, and infection were monitored during the follow-up period of 31.2 months. RESULTS: All patients could adequately ingest at 5 days postoperation and after removal of the cover plate. None of the patients had a persistent fever or allergic reactions. Ingestion was feasible immediately in all patients, and no postoperative bleeding was observed during ingestion. No secondary hemorrhages were observed during follow-up. No postoperative wound dehiscence on the midline of the palate was observed. No infections were observed after the removal of the cover plate. No patients suffered from severe scar formation or contracture of the wound in the follow-up period. Hemorrhage, undue epithelialization, and scar contracture did not occur in any patient. The mean evaluation score was 7.75 points. CONCLUSION: HDAM can be used safely and effectively for wound closure following palatoplasty in cleft palate infants. Future studies testing the safety of patient's own amnion for palatoplasty, are required.

Molecular Characterization of Three Canine Models of Human Rare Bone Diseases: Caffey, van den Ende-Gupta, and Raine Syndromes.

One to two percent of all children are born with a developmental disorder requiring pediatric hospital admissions. For many such syndromes, the molecular pathogenesis remains poorly characterized. Parallel developmental disorders in other species could provide complementary models for human rare diseases by uncovering new candidate genes, improving the understanding of the molecular mechanisms and opening possibilities for therapeutic trials. We performed various experiments, e.g. combined genome-wide association and next generation sequencing, to investigate the clinico-pathological features and genetic causes of three developmental syndromes in dogs, including craniomandibular osteopathy (CMO), a previously undescribed skeletal syndrome, and dental hypomineralization, for which we identified pathogenic variants in the canine SLC37A2 (truncating splicing enhancer variant), SCARF2 (truncating 2-bp deletion) and FAM20C (missense variant) genes, respectively. CMO is a clinical equivalent to an infantile cortical hyperostosis (Caffey disease), for which SLC37A2 is a new candidate gene. SLC37A2 is a poorly characterized member of a glucose-phosphate transporter family without previous disease associations. It is expressed in many tissues, including cells of the macrophage lineage, e.g. osteoclasts, and suggests a disease mechanism, in which an impaired glucose homeostasis in osteoclasts compromises their function in the developing bone, leading to hyperostosis. Mutations in SCARF2 and FAM20C have been associated with the human van den Ende-Gupta and Raine syndromes that include numerous features similar to the affected dogs. Given the growing interest in the molecular characterization and treatment of human rare diseases, our study presents three novel physiologically relevant models for further research and therapy approaches, while providing the molecular identity for the canine conditions.

The retrograde transverse cervical artery as a recipient vessel for free tissue transfer in complex head and neck reconstruction with a vessel-depleted neck.

BACKGROUND: Reconstruction in a vessel-depleted neck is challenging. The success rates can be markedly decreased because of unavailability of suitable recipient vessels. In order to obtain a reliable flow, recipient vessels away from the zone of fibrosis, radiation, or infection need to be explored. The aim of this report is to present our experience and clinical outcomes using the retrograde flow coming from the distal transverse cervical artery (TCA) as a source for arterial inflow for complex head and neck reconstruction in patients with a vessel-depleted neck. METHODS: Between July 2010 and June 2016, nine patients with a vessel-depleted neck underwent secondary head and neck reconstruction using the retrograde TCA as recipient vessel for microanastomosis. The mean age was 49.6 years (range, 36 to 68 years). All patients had previous bilateral neck dissections and all, except one, had also received radiotherapy. Indications included neck contracture release (n = 3), oral (n = 1), mandibular (n = 3) and pharyngoesophageal (n = 2) reconstruction necessitating free anterolateral thigh (n = 3) and medial sural artery (n = 1) perforator flaps, fibula (n = 3) and ileocolon (n = 2) flaps respectively. RESULTS: There was 100% flap survival rate with no re-exploration or any partial flap loss. One case of intra-operative arterial vasospasm at the anastomotic suture line was managed intra-operatively with vein graft interposition. There were no other complications or donor site morbidity during the follow-up period. CONCLUSIONS: In a vessel-depleted neck, the reverse flow of the TCA may be a reliable option for complex secondary head and neck reconstruction in selected patients.

Arthropathy, osteolysis, keloids, relapsing conjunctival pannus and gingival overgrowth: a variant of polyfibromatosis?

Polyfibromatosis is a rare fibrosing condition characterized by fibromatosis in different body areas and by keloid formation, and which can be associated with arthropathy and osteolysis. Familial occurrence has been described, but the cause remains unknown. Here, we describe a patient with characteristics of polyfibromatosis with arthropathy who had in addition severe conjunctival fibrosis, distinctive face, gingival overgrowth, and pigmented keloids. We discuss the resemblances and differences with polyfibromatosis and descriptions of other, similar patients. We conclude that at present it remains uncertain whether the patient has a variant of polyfibromatosis or a separate entity.

Identification of molecular phenotypic descriptors of breast capsular contracture formation using informatics analysis of the whole genome transcriptome.

Breast capsular contracture formation following silicone implant augmentation/reconstruction is a common complication that remains poorly understood. The aim of this study was to identify potential biomarkers implicated in breast capsular contracture formation by using, for the first time, whole genome arrays. Biopsy samples were taken from 18 patients (23 breast capsules) with Baker Grade I-II (Control) and Baker Grade III-IV (Contracted). Whole genome microarrays were performed and six significantly dysregulated genes were selected for further validation with quantitative reverse transcriptase polymerase chain reaction and immunohistochemistry. Hematoxylin and eosin was also carried out to compare the histological characteristics of control and contracted samples. Microarray results showed that aggrecan, tissue inhibitor of metalloproteinase 4 (TIMP4), and tumor necrosis factor superfamily (ligand) member 11 were significantly down-regulated in contracted capsules; while matrix metallopeptidase 12, serum amyloid A 1, and interleukin 8 (IL8) were significantly up-regulated. The dysregulation of aggrecan, tumor necrosis factor superfamily (ligand) member 11, TIMP4, and IL8 was validated by quantitative reverse transcriptase polymerase chain reaction (p < 0.05). Immunohistochemistry confirmed an increased protein expression for IL8 and matrix metallopeptidase 12 in contracted capsules (p < 0.05), and decreased protein expression of TIMP4 (p < 0.05). This study has shown, for the first time, a number of unique biomarkers of significance in capsular contracture formation. IL8 and TIMP4 may serve as potential key diagnostic, therapeutic, and prognostic biomarkers in capsular contracture formation.

Effect of Carboxymethyl Chitin on Capsule Formation around Silicone Implants: An In Vivo and In Vitro Study.

BACKGROUND: Capsular contracture is a serious complication that occurs after augmentation mammaplasty. The authors previously identified that carboxymethyl chitin had an inhibitory effect on capsule formation. This study was performed to elucidate the possible molecular mechanisms through which carboxymethyl chitin inhibits the formation of a capsule around silicone implants. METHODS: In this study, the authors cultured human dermal fibroblasts and treated them with carboxymethyl chitin in vitro. The difference in proliferation between treated and untreated cells was analyzed through the 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide assay. Protein levels of transforming growth factor beta-1 and alpha smooth muscle actin (α-SMA) were examined by Western blot analysis. Expression levels of type I and type III collagen were checked by enzyme-linked immunosorbent assay. In vivo, silicone implants were placed under the pectoralis muscle in 12 female rabbits. The thickness of the capsule was measured by histologic analysis, and the effect of carboxymethyl chitin on α-SMA, collagen type I and III expression levels was evaluated by real-time polymerase chain reaction analysis, enzyme-linked immunosorbent assay, Western blot, and immunofluorescence analysis. RESULTS: In the in vitro study, we confirmed that carboxymethyl chitin inhibited the proliferation of fibroblasts. The protein expression levels of collagen type I, transforming growth factor beta-1, and α-SMA were inhibited by carboxymethyl chitin treatment. In vivo, carboxymethyl chitin treatment reduced capsular thickness and the expression of α-SMA and collagen types I and III in capsules around silicone implants. CONCLUSION: The authors' results showed that carboxymethyl chitin could influence capsule formation around silicone implants by inhibiting the fibroblast activity, interrupting fibroblast-to-myofibroblast differentiation, and decreasing collagen synthesis. CLINICAL RELEVANCE STATEMENT: Carboxymethyl chitin influence capsule formation around silicone implants. Although more clinical studies are needed to verify the effect of carboxymethyl chitin on capsular contracture, the authors believe that it will play an effective role in the clinical application of reducing the occurrence of capsular contracture.

The effect of liposome-delivered prednisolone on collagen density, myofibroblasts, and fibrous capsule thickness around silicone breast implants in rats.

Capsular contracture is a potential adverse effect of breast implants. An inflammatory reaction is most likely the origin of fibrosis around the implant. It is possible that some substances may act to prevent this inflammatory reaction. Thus, our goal was to evaluate the effectiveness of local depot prednisolone phosphate-liposomes (PPL) on fibrous capsule formation around textured silicone breast implants. Shell prostheses (2 mL) were implanted in the right (plus PPL group) and left (plus saline solution, saline group) subcutaneous dorsum of 18 rats. In another 18 rats, the implants were positioned in the left of the back without any drug instillation (control group). In the PPL group, the capsule thickness (microm) and density (%) of collagen were significantly (p<0.0001) lower compared with the control group on days 35 and 90 postsurgery. Furthermore, in the PPL group, a significant reduction in myofibroblast count was observed on day 90 postsurgery (p<0.0001). In conclusion, a single dose of depot liposome-delivered prednisolone was effective at impairing capsule formation around the silicone implant. The results suggest a strong local and weak systemic effect of PPL on the fibrous tissue around silicone implants. To our knowledge, no study has yet assessed the effect of PPL on silicone breast implants.

Myofibroblasts and capsular tissue tension in breast capsular contracture.

BACKGROUND: This study aimed to observe the relationship between the number of myofibroblasts, the tensile strength of the breast implant capsule, and the degree of breast capsular contracture. METHODS: The study enrolled 21 women with 31 capsular contractures after aesthetic breast augmentation. The capsular tissue specimens were obtained during capsulectomy, open capsulotomy, and other revisional procedures. The tensile strength of capsular tissues (1 × 3 cm) was measured by tensiometer. The capsular tissues were immunostained by alpha smooth muscle actin, and the immunostained myofibroblasts all were counted on a 2.5-mm length of the capsule. RESULTS: Myofibroblasts were detected in 22 (71%) of 31 specimens. The myofibroblasts were on the outer layer of the capsule and made up 7.3% to 50% (average, 26.9% ± 12.7%) of the capsule thickness. The number of myofibroblasts varied according to the degree of capsular contracture, but grades 2, 3, and 4 contracture did not differ significantly (p = 0.102). The average tensile strength of the capsule was 44 ± 38 N. Tensile strength was the lowest for grade 2 (27.0 ± 22.2 N), increased for grade 3 (38.0 ± 22.6 N), and was highest for grade 4 (66.5 ± 55.4 N; p = 0.044) contracture. The tensile strength of the capsule correlated positively with the degree of capsular contracture (p = 0.029). CONCLUSION: The tensile strength of breast capsules correlated with the degree of capsular contracture. The authors think myofibroblasts appear during an active phase of wound contraction and diminish when the wound has matured.

Juvenile hyaline fibromatosis and infantile systemic hyalinosis: a unifying term and a proposed grading system.

BACKGROUND: It has been suggested that juvenile hyaline fibromatosis and infantile systemic hyalinosis represent different severities of the same disease. OBJECTIVE: We sought to redefine these disorders clearly to establish a common inclusive terminology. PATIENTS: The study included two children with early onset of similar pink papulonodular skin lesions and marked gingival hyperplasia. The first case was characterized by flexion contractures of the large joints, fractures, persistent diarrhea, recurrent chest infections, and retarded physical growth. The second patient had large swellings on the scalp and knees without systemic involvement. RESULTS: Radiologic examination revealed fractures and osteolytic bone lesions in the first case, and soft tissue masses in the second case. Laboratory tests showed anemia in both cases, and hypogammaglobulinemia, hypoalbuminemia, and electrolyte imbalance in the first case. Histopathological and ultrastructural evaluation demonstrated hyalinized fibrous tissue in the dermis in both cases. LIMITATIONS: Genetic studies were unavailable. CONCLUSION: Juvenile hyaline fibromatosis and infantile systemic hyalinosis share many common features that strongly support consideration of these conditions as different expressions of the same disorder. We propose a common term, "hyaline fibromatosis syndrome," which can be divided into mild, moderate, and severe subtypes.

Infantile systemic hyalinosis presenting as intractable infantile diarrhea.

Infantile systemic hyalinosis is an autosomal recessive disease characterized by severe progressive flexion contractures, multiple recurring subcutaneous tumours, and gingival hypertrophy. It is caused by mutations in the gene encoding capillary morphogenesis protein-2 (CMG2). Here we report a Saudi infant with infantile systemic hyalinosis who presented with intractable diarrhea, and we review the literature emphasizing recent developments in the molecular genetics of this disease.

Infantile systemic hyalinosis: Case report and review of the literature.

Infantile systemic hyalinosis (ISH) is a rare, progressive autosomal recessive disease, which is usually fatal by the age of 2 years. Clinical onset typically occurs within the first few weeks of life. The disease is characterized by joint contractures, osteopenia, failure to thrive, gingival hypertrophy, diarrhea, protein-losing enteropathy, and frequent infections. Dermatologic manifestations include thickened skin, hyperpigmentation, perianal nodules, and facial papules. Histopathology shows hyaline deposits in the dermis and visceral organs. We describe a patient with ISH confirmed by clinical and histopathologic findings, as well as DNA sequence analysis, which revealed a novel homozygous T118K mutation in the CMG2 gene.

Inhibition of Skin Wound Contraction by Nanofibrillar Cellulose Hydrogel.

BACKGROUND: Although wound contraction is an essential part of healing, excessive contraction can compromise healing through induction of scarring and fibrosis. This in turn leads to development of wound contractures that limit elasticity and function. Major research efforts have focused on development of novel therapeutic approaches to gain inhibitory control over wound contraction. Despite these efforts, the need for cost-effective, clinically feasible, and effective agents to inhibit wound contraction remains. METHODS: In this study, the authors investigated the effect of nanofibrillar cellulose hydrogel on wound contraction both in vitro and in vivo. Two different porcine full-thickness wounds (8-mm punch-biopsy wounds and 4 × 4-cm wounds covered with a 1:3-meshed split-thickness skin graft) were treated with or without nanofibrillar cellulose or carboxymethylcellulose (Purilon hydrogel), which was used as a reference treatment. Wound contraction was observed macroscopically, and histologic sections were taken at 14-day follow-up. RESULTS: Nanofibrillar cellulose hydrogel inhibited 70 percent of punch-biopsy wound contraction, whereas the carboxymethylcellulose hydrogel was ineffective. Importantly, application of nanofibrillar cellulose on split-thickness skin grafts did not inhibit epithelialization of the interstices or cell migration from the graft. CONCLUSION: The authors' results, although preliminary, indicate a potential for nanofibrillar cellulose hydrogel as a novel material for controlling excessive wound contraction.

Postburn Microstomia Prevention Using an Appliance Providing Simultaneous Horizontal and Vertical Adjustable Forces.

Microstomia, an abnormally small oral orifice, is a complication of perioral facial burns. In this case, contraction of the circumoral tissues and hypotonia of the musculature is responsible for this microstomia, which can produce aesthetic and functional impairment with eating, swallowing, communication (speech and facial expressions), compromised dental care and maintenance due to limited oral access, social interactions, and psychological well-being. Conservative management involves providing physical resistance to scar contracture, with opposing horizontal and vertical circumoral forces by means of appliances that aim to stretch the commissures and fibrotic muscles. Numerous appliances, either intraoral or extraoral, have been described to prevent or treat microstomia by delivering a static or dynamic stretch horizontally or vertically, with most designed to stretch the mouth horizontally. Finding a comfortable effective way to stretch the mouth vertically has proved to be a challenge. This article describes the fabrication of a dynamic commissural appliance, constructed using acrylic resin and expansion screws, which provide simultaneous horizontal and vertical circumoral forces. This appliance is constructed easily and inexpensively without the need for taking impressions, can be adjusted so that it is almost painlessly inserted, and is progressively activated. It is convenient for use because the patient controls the pressure that is applied by the appliance. Its use in a case is described where the appliance has improved mouth opening and consequently functional outcomes.

[Histological and immunohistochemical investigations with capsular contracture after breast augmentation with smooth silicone gel implants]. / Histologische und immunhistochemische Untersuchungen bei Kapselkontraktur nach glatten Brustimplantaten.

INTRODUCTION: A prospective study was performed to analyse the cellular and molecular composition of fibrous capsules around silicone breast implants. The necessity of an exact histological classification for comparing objectively the different findings of capsular contracture is shown. PATIENTS AND METHODS: The prospective study (investigation time 1/2003 to 6/2005) included 24 female patients (average age: 40+/-12 years) with contracture after bilateral cosmetic breast augmentation with smooth silicone gel implants (Mentor). In each patient the baker score was determined preoperatively. Samples of capsular tissue from all patients were evaluated histologically and immunohistochemically and classified according to the histological classification introduced by Wilflingseder and co-workers. RESULTS: All capsules showed the same basic histological structure with a three-layer composition. For the correlation analysis we had to exclude one patient with repeated implant change. There was no correlation between the patient's age, time of implantation, length of implant period, and capsular contracture. Greater amounts of silicone particles were associated with increased degrees of capsular contracture (Baker: r = 0.687, n = 23, p < or = 0.001; Wilflingseder: r = 0.784, n = 23, p < or = 0.001). High silicone amounts were associated with an increased local inflammation (r = 0.489, n = 23, p , 0.05). A moderate to severe local inflammation was found in 23 patients (95.8%). In summary, there was a positive correlation (r = 0.797, n = 23; p , or = 0.001) between the clinical classification (Baker score I to IV) and the histological classification (Wilflingseder score I to IV). CONCLUSIONS: We demonstrated in our study, in spite of using implants with high gel cohesiveness (fourth generation), the presence of vacuolated macrophages with microcystic structures containing silicone and silicone particles in the capsular tissue. Greater capsular thickness was associated with an increased number of silicone particles ans silicone-loaded macrophages in the peri-implant capsule. The histological classification introduced by Wilflingseder and co-workers takes into consideration this pathogenetic mechanism of inflammatory reaction which seems to be one of the major key factors in the development of capsular contracture.

Inhibition of conjunctival scarring and contraction by a porous collagen-glycosaminoglycan implant.

PURPOSE: To study the healing processes of full-thickness wounds in the adult rabbit conjunctiva after grafting with a porous collagen-glycosaminoglycan (CG) copolymer matrix. METHODS: A 7-mm trephine was used to produce lesions of the bulbar conjunctiva down to the level of the bare sclera. Full-thickness removal of the conjunctiva and Tenon's capsule created a reproducible wound bed. Wounds either remained ungrafted (control) or were grafted with CG matrix. In previous studies, this CG matrix has induced partial regeneration of the dermis in the human, the swine, and the guinea pig. Healing of the conjunctival epithelium and underlying stroma was evaluated by histology, immunohistochemistry, and measurement of wound contraction kinetics. RESULTS: By 28 days, ungrafted wounds had closed by contraction (26.4% +/- 5.0% fornix shortening) and the formation of scarlike tissue comprising an aligned array of dense collagen populated with occasional fibroblasts. Grafting of identical defects with CG copolymer matrix resulted in inhibition of wound contraction (6.8% +/- 3.2% fornix shortening) and the formation of a tissue that resembled normal conjunctival stroma, being composed of a loose network of collagen fibers and fibroblasts. Contractile fibroblasts (myofibroblasts) were identified at the edge of both ungrafted and grafted wounds during the period of active contraction. Both ungrafted and grafted wounds were completely re-epithelialized by 28 days. CONCLUSIONS: Implantation of CG copolymer matrix drastically reduced contraction and promoted the formation of a nearly normal subconjunctival stroma.

Infantile systemic hyalinosis in siblings: clinical report, biochemical and ultrastructural findings, and review of the literature.

A boy presented at age 3.5 months with joint contractures, restlessness, and pain on handling. His skin was thickened and there were livid-red macular lesions over bony prominences. Infantile systemic hyalinosis (ISH) was diagnosed, a presumably autosomal recessive, progressive, and painful disorder of as yet unknown pathogenesis. Observation over three years confirmed the diagnosis as typical changes, such as nodules on both ears, pearly papules in the perinasal folds and on the neck, fleshy nodules in the perianal region, and gingival hypertrophy, developed. Skin lesions and painful joint contractures progressed in spite of intense physiotherapy, and at age 3, the child had marked motor disability. The central nervous system (CNS) appeared to be intact and the infant showed normal mental development. Radiologic findings included marked generalized osteopenia, osteolytic erosions in the metaphyses of the long bones, and cortical thinning. Electron microscopy of two skin biopsies demonstrated deposition of floccular amorphous substance that was abundant around, and appeared to originate from, small blood vessels in the dermis, partially interfering with collagen fiber formation. Lysosomal inclusions were not seen. Serum acid hyaluronidase activity was within the normal range, and the synthesis of hyaluronic acid and proteoglycans in cultured skin fibroblasts was similar to that of control cells. A younger sister presented at age two months with painful joint contractures and discrete livid-red macules over both malleoli, and showed a similar progression of the disorder over the first year of life. The diagnosis of ISH should be considered in infants and children presenting with painful joint contractures and skin lesions. The pathogenesis of this disabling and disfiguring disorder remains unclear. Our data confirm probable autosomal recessive inheritance, and do not support lysosomal storage, hyaluronidase deficiency, or a primary collagen disorder, but indicate that the amorphous material accumulating in the skin and articular soft tissues may originate from the blood circulation.

Experimental study of a newly developed bilayer artificial skin.

A bilayer artificial skin composed of an outer layer of silicone polymer and an inner sponge layer of collagen containing chondroitin 6-sulphate was developed by modifying the technique proposed by Yannas et al. The artificial skin was placed on the skin defects on the backs of rats. Histological observation indicated that fibroblasts and capillaries infiltrated into the pores and filled in lattice spaces, resulting in synthesis of the connective tissue matrix and absorption of the original network of collagen and chondroitin 6-sulphate. Epidermal cells migrated from the edge of the wound between the two layers. Post-operative contracture in the wound with the artificial skin was significantly less than in the control.

Total encapsulation and asymmetric deformation of an intraocular lens.

We report a case of marked anterior capsule contraction after uneventful cataract surgery in an 82-year-old women. The patient had implantation of a single-piece foldable acrylic intraocular lens (IOL) after phacoemulsification through a 3.0 mm superior corneal incision. Between 1 month and 5.5 months after surgery, significant anterior capsule contraction ensued with total encapsulation of the lens. The resultant fibrotic reaction, which had a localized tangential component, caused an asymmetric deformation and mild IOL displacement. The patient was treated on separate occasions with a neodymium:YAG laser anterior capsulotomy and surgical capsulotomy to release tension on the lens and clear the visual axis.

Severe capsulorhexis contracture after cataract surgery in myotonic dystrophy.

A 42-year-old woman with myotonic dystrophy developed bilateral severe capsulorhexis contracture after uneventful phacoemulsification cataract surgery with implantation of 1-piece poly(methyl methacrylate) intraocular lenses (IOLs). The anterior capsular opening in her right eye constricted to a diameter of 0.7 mm, reducing visual acuity to counting fingers. Complete closure of the capsulorhexis with IOL encapsulation developed in her left eye, reducing visual acuity to hand movements. Surgical anterior capsulectomies restored visual acuity to 6/9 in both eyes. Myotonic dystrophy may predispose to the development of severe capsulorhexis contracture after cataract surgery.