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1.  Treatment of Popliteal Pterygium Using an Ilizarov External Fixator 
Clinics in Orthopedic Surgery  2009;1(4):236-239.
Popliteal pterygium syndrome is a rare congenital disorder that consists of popliteal webs and craniofacial, genitourinary and extremity anomalies. Only moderate successful surgical excision of the fibrotic band within the popliteal web has been reported because the nerves and vessels in the affected site are short and displaced into the web and they are attached to adjacent tissues. We performed hamstring tenotomy on the ischial tuberosity, tenotomy of the flexor hallucis longus and Z-lengthening of the Achilles tendon on the ankle in our patient, and this was followed by gradual correction using an Ilizarov external fixator. Full extension of the knee joint was achieved at the ninth postoperative week. However, some recurrence of flexion contracture was noted at two years follow-up. Gradual soft tissue lengthening with an Ilizarov external fixator can be one of the optimal procedures when excision of a fibrous band and Z-plasty are not possible due to severe adhesion of the nerves and vessels into a fibrotic band. However, a cautious approach is recommended when considering the high risk of recurrence.
doi:10.4055/cios.2009.1.4.236
PMCID: PMC2784965  PMID: 19956482
Popliteal pterygium syndrome; External fixator; Ilizarov
2.  Popliteal pterygium syndrome: a clinical study of three families and report of linkage to the Van der Woude syndrome locus on 1q32 
Journal of Medical Genetics  1999;36(12):888-892.
Popliteal pterygium syndrome (PPS) is a rare autosomal dominant disorder, thought to occur with an incidence of approximately 1 in 300 000 live births. The main clinical manifestations are popliteal webbing, cleft lip, cleft palate, lower lip pits, syndactyly, and genital and nail anomalies. This report describes the clinical features in two families with PPS and one isolated case, showing the range of anomalies found both within and between the families. PPS has some features in common with Van der Woude syndrome (VWS), also inherited as an autosomal dominant condition, with cleft lip/palate and, more distinctively, lower lip pits. Although the gene for VWS has not yet been identified, it has been localised to within 1.6 cM in the region 1q32-41. To determine whether PPS and VWS represent allelic forms of the same gene, three families were genotyped for markers flanking and within the critical region. A multipoint lod score of 2.7 was obtained, with no evidence of recombination, supporting the hypothesis that these two disorders are allelic.


Keywords: pterygium; van der Woude syndrome; cleft lip; cleft palate
PMCID: PMC1734268  PMID: 10593995
3.  Mutations in IRF6 cause Van der Woude and popliteal pterygium syndromes 
Nature genetics  2002;32(2):285-289.
Interferon regulatory factor 6 (IRF6) belongs to a family of nine transcription factors that share a highly conserved helix–turn–helix DNA-binding domain and a less conserved protein-binding domain. Most IRFs regulate the expression of interferon-α and -β after viral infection1, but the function of IRF6 is unknown. The gene encoding IRF6 is located in the critical region for the Van der Woude syndrome (VWS; OMIM 119300) locus at chromosome 1q32–q41 (refs 2,3). The disorder is an autosomal dominant form of cleft lip and palate with lip pits4, and is the most common syndromic form of cleft lip or palate. Popliteal pterygium syndrome (PPS; OMIM 119500) is a disorder with a similar orofacial phenotype that also includes skin and genital anomalies5. Phenotypic overlap6 and linkage data7 suggest that these two disorders are allelic. We found a nonsense mutation in IRF6 in the affected twin of a pair of monozygotic twins who were discordant for VWS. Subsequently, we identified mutations in IRF6 in 45 additional unrelated families affected with VWS and distinct mutations in 13 families affected with PPS. Expression analyses showed high levels of Irf6 mRNA along the medial edge of the fusing palate, tooth buds, hair follicles, genitalia and skin. Our observations demonstrate that haploinsufficiency of IRF6 disrupts orofacial development and are consistent with dominant-negative mutations disturbing development of the skin and genitalia.
doi:10.1038/ng985
PMCID: PMC3169431  PMID: 12219090
4.  Escobar Syndrome (Multiple Pterygium Syndrome) Associated with Thoracic Kyphoscoliosis, Lordoscoliosis, and Severe Restrictive Lung Disease: A Case Report 
HSS Journal  2005;1(1):35-39.
Background
Escobar syndrome or multiple pterygium syndrome is characterized by a web across every flexion crease in the extremities, most notably the popliteal space. In addition, this syndrome is associated with two other structural anomalies: a vertical talus and congenital lordoscoliosis. We present a case report of a patient with Escobar syndrome who was initially managed conservatively and subsequently had severe and debilitating progression and respiratory decompensation ultimately requiring surgical intervention.
Study Design
Case report.
Methods
After preoperative evaluation by a pediatrician, pulmonologist, and otolaryngologist, the patient underwent one-stage anterior and posterior spinal fusion with instrumentation as well as multiple osteotomies, rib resections, and vertebrectomies.
Results
The patient’s postoperative course was complicated by wound necrosis requiring irrigation and debridement, a urinary tract infection, and a tracheostomy for persistent atelectasis. The patient eventually recovered from all complications. There were never any focal neurologic deficits. The patient had a 3-year follow-up with radiographically confirmed maintenance of correction. Fusion was obtained in the anterior and posterior segments. Clinically, the patient is able to stand upright, can participate in functional activities, and has not required any pain medication. The patient’s functional vital capacity improved from 23% predicted preoperatively to 60% predicted postoperatively.
Conclusions
Patients with severe spinal deformity secondary to Escobar syndrome can be successfully treated surgically. We propose early surgical intervention in this group to prevent curve progression, restrictive lung disease, and the need for complex salvage procedures.
doi:10.1007/s11420-005-0103-5
PMCID: PMC2504136  PMID: 18751807
Escobar syndrome; spinal deformity; restrictive lung disease; multiple pterygium syndrome; lordoscoliosis
5.  Missense mutations that cause Van der Woude syndrome and popliteal pterygium syndrome affect the DNA-binding and transcriptional activation functions of IRF6 
Human Molecular Genetics  2008;18(3):535-545.
Cleft lip and cleft palate (CLP) are common disorders that occur either as part of a syndrome, where structures other than the lip and palate are affected, or in the absence of other anomalies. Van der Woude syndrome (VWS) and popliteal pterygium syndrome (PPS) are autosomal dominant disorders characterized by combinations of cleft lip, CLP, lip pits, skin-folds, syndactyly and oral adhesions which arise as the result of mutations in interferon regulatory factor 6 (IRF6). IRF6 belongs to a family of transcription factors that share a highly conserved N-terminal, DNA-binding domain and a less well-conserved protein-binding domain. To date, mutation analyses have suggested a broad genotype–phenotype correlation in which missense and nonsense mutations occurring throughout IRF6 may cause VWS; in contrast, PPS-causing mutations are highly associated with the DNA-binding domain, and appear to preferentially affect residues that are predicted to interact directly with the DNA. Nevertheless, this genotype–phenotype correlation is based on the analysis of structural models rather than on the investigation of the DNA-binding properties of IRF6. Moreover, the effects of mutations in the protein interaction domain have not been analysed. In the current investigation, we have determined the sequence to which IRF6 binds and used this sequence to analyse the effect of VWS- and PPS-associated mutations in the DNA-binding domain of IRF6. In addition, we have demonstrated that IRF6 functions as a co-operative transcriptional activator and that mutations in the protein interaction domain of IRF6 disrupt this activity.
doi:10.1093/hmg/ddn381
PMCID: PMC2638798  PMID: 19036739
6.  Multiple pterygium syndrome. 
Journal of Medical Genetics  1981;18(6):451-455.
The multiple pterygium syndrome is a rare autosomal recessive condition characterised by arthrogryposis multiplex congenita, pterygia of the neck, fingers, and antecubital, popliteal, and intercrural areas, growth retardation, and facial, vertebral, and genital anomalies. We present two unrelated patients of 17 and 6 years of age, respectively, affected with this condition. We describe the natural history of their disorder since birth and review the spectrum of phenotypic variation of the multiple pterygium syndrome in 25 published cases.
Images
PMCID: PMC1048792  PMID: 7334504
7.  Novel IRF6 mutations in families with Van Der Woude syndrome and popliteal pterygium syndrome from sub-Saharan Africa 
Orofacial clefts (OFC) are complex genetic traits that are often classified as syndromic or nonsyndromic clefts. Currently, there are over 500 types of syndromic clefts in the Online Mendelian Inheritance in Man (OMIM) database, of which Van der Woude syndrome (VWS) is one of the most common (accounting for 2% of all OFC). Popliteal pterygium syndrome (PPS) is considered to be a more severe form of VWS. Mutations in the IRF6 gene have been reported worldwide to cause VWS and PPS. Here, we report studies of families with VWS and PPS in sub-Saharan Africa. We screened the DNA of eight families with VWS and one family with PPS from Nigeria and Ethiopia by Sanger sequencing of the most commonly affected exons in IRF6 (exons 3, 4, 7, and 9). For the VWS families, we found a novel nonsense variant in exon 4 (p.Lys66X), a novel splice-site variant in exon 4 (p.Pro126Pro), a novel missense variant in exon 4 (p.Phe230Leu), a previously reported splice-site variant in exon 7 that changes the acceptor splice site, and a known missense variant in exon 7 (p.Leu251Pro). A previously known missense variant was found in exon 4 (p.Arg84His) in the PPS family. All the mutations segregate in the families. Our data confirm the presence of IRF6-related VWS and PPS in sub-Saharan Africa and highlights the importance of screening for novel mutations in known genes when studying diverse global populations. This is important for counseling and prenatal diagnosis for high-risk families.
doi:10.1002/mgg3.66
PMCID: PMC4049366  PMID: 24936515
IRF6; popliteal pterygium syndrome; sub-Saharan Africa; Van der Woude syndrome
8.  Prenatal Diagnosis of Lethal Multiple Pterygium Syndrome Using Two-and Three-Dimensional Ultrasonography 
Lethal multiple pterygium (LMP) is a series of disorders of fetal formation with a heterogeneous range of manifestations that generally include cystic hygroma, pulmonary hypoplasia, cleft palate, cryptorchidism, joint contractures, fetal akinesia, heart defects, growth restriction, and intestinal malrotation. The prenatal diagnosis of this syndrome is suspected when two-dimensional ultrasound (2DUS) scan shows several malformations.. The three-dimensional ultrasound (3DUS) in rendering mode permits the spatial visualization of these malformations, allowing better understanding of this anomaly by parents. We report a case of a fetus in the second trimester with multiple abnormalities suggestive of LMP that were identified using 2DUS, and emphasize the importance of 3DUS in counseling the parents.
doi:10.4103/2156-7514.103055
PMCID: PMC3515938  PMID: 23230547
Lethal multiple pterygium; prenatal diagnosis; three-dimensional ultrasound; two-dimensional ultrasound
9.  Comparative analysis of IRF6 variants in families with Van der Woude syndrome and popliteal pterygium syndrome using public whole exome databases 
Purpose
IRF6 is a transcription factor that, when mutated, causes allelic autosomal dominant clefting syndromes, Van der Woude syndrome (VWS) and popliteal pterygium syndrome (PPS). We compared the distribution of IRF6 coding and splice site mutations from 549 VWS or PPS families to that of variants from the 1000 Genomes and NHLBI Exome Sequencing Projects.
Methods
We compiled all published pathogenic IRF6 mutations and performed direct sequencing of IRF6 in VWS/PPS families.
Results
While mutations causing VWS/PPS were non-randomly distributed with significantly increased frequencies in the DNA-binding domain (p=0.0001), variants found in controls were rare and evenly distributed in IRF6. Out of 194 different missense or nonsense variants described as potentially pathogenic, we identified only two in over 6000 controls. 5.9% of missense mutations in patients were reported by PolyPhen and SIFT as benign; suggesting that use of current in silico prediction models to determine function can have significant false negatives.
Conclusion
Mutation of IRF6 occurs infrequently in controls, suggesting that for IRF6 there is a high probability that disruption of the coding sequence, particularly the DNA-binding domain, will result in syndromic features. Prior associations of coding sequence variants in IRF6 with clefting syndromes have had few false positives.
doi:10.1038/gim.2012.141
PMCID: PMC3723330  PMID: 23154523
Van der Woude; exome; cleft; popliteal pterygium; mutation
10.  Comparative analysis of IRF6 variants in families with Van der Woude syndrome and popliteal pterygium syndrome using public whole-exome databases 
Genetics in Medicine  2012;15(5):338-344.
Purpose:
Mutations in the transcription factor IRF6 cause allelic autosomal dominant clefting syndromes, Van der Woude syndrome, and popliteal pterygium syndrome. We compared the distribution of IRF6 coding and splice-site mutations from 549 families with Van der Woude syndrome or popliteal pterygium syndrome with that of variants from the 1000 Genomes and National Heart, Lung, and Blood Institute Exome Sequencing Projects.
Methods:
We compiled all published pathogenic IRF6 mutations and performed direct sequencing of IRF6 in families with Van der Woude syndrome or popliteal pterygium syndrome.
Results:
Although mutations causing Van der Woude syndrome or popliteal pterygium syndrome were nonrandomly distributed with significantly increased frequencies in the DNA-binding domain (P = 0.0001), variants found in controls were rare and evenly distributed in IRF6. Of 194 different missense or nonsense variants described as potentially pathogenic, we identified only two in more than 6,000 controls. PolyPhen and SIFT (sorting intolerant from tolerant) reported 5.9% of missense mutations in patients as benign, suggesting that use of current in silico prediction models to determine function can have significant false negatives.
Conclusion:
Mutation of IRF6 occurs infrequently in controls, suggesting that for IRF6 there is a high probability that disruption of the coding sequence, particularly the DNA-binding domain, will result in syndromic features. Prior associations of coding sequence variants in IRF6 with clefting syndromes have had few false positives.
doi:10.1038/gim.2012.141
PMCID: PMC3723330  PMID: 23154523
cleft; exome; mutation; popliteal pterygium; Van der Woude
11.  Popliteal pterygium syndrome: a phenotypic and genetic analysis. 
Journal of Medical Genetics  1978;15(1):35-42.
Two additional families with popliteal pterygium syndrome are presented. Using previously published pedigrees, as well as the ones reported here, evidence is presented that supports an autosomal dominant mode of inheritance for this syndrome. Analysis of previous familial cases showed a large degree of between and within-family variation. The segregation analysis supports the dominant hypothesis (P=0.5).
Images
PMCID: PMC1012821  PMID: 633318
12.  Pterygium syndrome. 
Journal of Medical Genetics  1976;13(3):249-251.
The pterygium syndrome consists of the neck, the antecubital fossae and the popliteal regions together with flexion deformities of the limb joints and anomalies of the vertebrae. A family, three offspring of which appear to be affected with the same disorder, is presented. All three are female; there is also a normal female child of the same union.
Images
PMCID: PMC1013404  PMID: 933128
13.  An autosomal dominant multiple pterygium syndrome. 
Journal of Medical Genetics  1988;25(2):96-103.
Three sibs and their mother with features of a multiple pterygium syndrome are reported. Inheritance in this family is consistent with autosomal dominant inheritance with great variation in severity between affected subjects. The importance of examining other family members closely in cases of multiple pterygium is emphasised.
Images
PMCID: PMC1015450  PMID: 2831369
14.  Search for Genetic Modifiers of IRF6 and Genotype-Phenotype Correlations in Van der Woude and Popliteal Pterygium Syndromes 
Van der Woude syndrome is the most common form of syndromic orofacial clefting, accounting for 1-2% of all patients with cleft lip and/or cleft palate. Van der Woude and popliteal pterygium syndromes are caused by mutations in IRF6, but phenotypic variability within and among families with either syndrome suggests that other genetic factors contribute to the phenotypes. The aim of this study was to identify common variants acting as genetic modifiers of IRF6 as well as genotype-phenotype correlations based on mutation type and location. We identified an association between mutations in the DNA-binding domain of IRF6 and limb defects (including pterygia). Although we did not detect formally significant associations with the genes tested, borderline associations suggest several genes that could modify the VWS phenotype, including FOXE1, TGFB3, and TFAP2A. Some of these genes are hypothesized to be part of the IRF6 gene regulatory network and may suggest additional genes for future study when larger sample sizes are also available. We also show that families with the Van der Woude phenotype but in whom no mutations have been identified have a lower frequency of cleft lip, suggesting there may be locus and/or mutation class differences in Van der Woude syndrome.
doi:10.1002/ajmg.a.36133
PMCID: PMC3898350  PMID: 23949966
Modifier gene; Van der Woude; popliteal pterygium; cleft; lip pit
15.  Prevalence and non-random distribution of exonic mutations in Interferon Regulatory Factor 6 (IRF6) in 307 families with Van der Woude syndrome and 37 families with popliteal pterygium syndrome 
Purpose
Interferon Regulatory Factor 6 (IRF6) encodes a member of the IRF family of transcription factors. Mutations in IRF6 cause Van der Woude (VWS) and popliteal pterygium syndromes (PPS), two related orofacial clefting disorders. Here, we compared and contrasted the frequency and distribution of exonic mutations in IRF6 between two large geographically distinct collections of families with VWS and between one collection of families with PPS.
Methods
We performed direct sequence analysis of IRF6 exons on samples from three collections, two with VWS and one with PPS.
Results
We identified mutations in IRF6 exons in 68% of families in both VWS collections and in 97% of families with PPS. In sum, 106 novel disease-causing variants were found. The distribution of mutations in the IRF6 exons in each collection was not random; exons 3, 4, 7, and 9 accounted for 80%. In the VWS collections, the mutations were evenly divided between protein truncation and missense, whereas most mutations identified in the PPS collection were missense. Further, the missense mutations associated with PPS were localized significantly to exon 4, at residues that are predicted to bind directly to DNA.
Conclusion
The non-random distribution of mutations in the IRF6 exons suggests a two-tier approach for efficient mutation screens for IRF6. The type and distribution of mutations are consistent with the hypothesis that VWS is caused by haploinsufficiency of IRF6. On the other hand, the distribution of PPS-associated mutations suggests a different, though not mutually exclusive, effect on IRF6 function.
doi:10.1097/GIM.0b013e318197a49a
PMCID: PMC2789395  PMID: 19282774
Cleft lip and palate; mutation; haploinsufficiency; dominant negative; cryptic splice site; CpG
16.  Winglets of the eye: dominant transmission of early adult pterygium of the conjunctiva. 
Journal of Medical Genetics  1990;27(6):392-394.
A pterygium, a wing-like thickening, of the bulbar conjunctiva is of environmental interest because it can occur on prolonged exposure to wind and weather. We describe a family with pterygium in two generations without a history of unusual exposure to the elements. There were six females and five males (including a set of male twins) with seven bilateral and four unilateral pterygia. The onset was unique in being in early adulthood, from the late teens through the twenties. This new genetic form can be distinguished by the age of onset from congenital and mid-adult pterygia, which are inherited as autosomal dominant traits. Irrespective of age, the treatment of conjunctival pterygium is surgical excision.
PMCID: PMC1017140  PMID: 2359104
17.  Multiple pterygium syndrome: evolution of the phenotype. 
Journal of Medical Genetics  1987;24(12):733-749.
The clinical features of the multiple pterygium syndrome are multiple congenital joint contractures, multiple skin webs, camptodactyly, vertebral anomalies, short stature, ptosis, and antimongoloid eye slant. We present 11 new cases to show the evolution of the full phenotype from birth and to confirm autosomal recessive inheritance. We emphasise morbidity secondary to respiratory impairment and that conductive deafness may be part of the syndrome.
Images
PMCID: PMC1050402  PMID: 3430553
18.  Early surgical debridement in the management of infectious scleritis after pterygium excision 
Purpose
The purpose of this study was to report outcomes of infectious scleritis after pterygium surgery, managed with antibiotic therapies and early scleral debridement.
Methods
Retrospective chart review of 13 consecutive cases of infectious scleritis after pterygium excision between 1999 and 2009 was conducted. Collected data included prior medical and surgical history, latency period between pterygium surgery and presentation of infectious scleritis, culture and histopathologic findings, antibiotic regimen, length of hospital stay, visual acuity before and after treatment, and complications.
Results
Median follow-up was at 14 months. Twelve patients underwent prompt surgical debridement after infectious scleritis diagnosis (median, 2.5 days). Debridement was delayed in one patient. Median hospital stay was 3 days. Best-corrected visual acuity improved in ten patients, remained stable in one patient, and decreased in two patients following treatment. Complications included scleral thinning requiring scleral patch graft (1/13), glaucoma (3/13), and progression to phthisis bulbi (1/13). No patients required enucleation.
Conclusions
In contrast to the generally poor outcomes in the literature, early surgical debridement of pterygium-associated infectious scleritis appears to offer improved prognosis.
doi:10.1007/s12348-012-0062-1
PMCID: PMC3345049  PMID: 22354483
Infectious scleritis; Pterygium excision; Surgical debridement; Biofilm; Medicine & Public Health; Ophthalmology
19.  A Case of Acquired Idiopathic Pterygium Inversum Unguis 
Annals of Dermatology  2014;26(3):374-376.
Pterygium inversum unguis (PIU) is a rare nail abnormality in which the distal nail bed adheres to the ventral surface of the nail plate, with obliteration of the distal groove. Because of the rarity of this condition, its exact origin is unknown. This disorder can be either congenital or acquired, with or without a family history. The acquired forms may be idiopathic or secondary to systemic connective tissue diseases or other causes such as stroke, neurofibromatosis, leprosy, or the use of nail fortifiers. We present an unusual case of acquired idiopathic PIU of the 10 fingernails in a 22-year-old man.
doi:10.5021/ad.2014.26.3.374
PMCID: PMC4069649  PMID: 24966638
Malformed nails; Pterygium inversum unguis
20.  Autologous Cryoprecipitate for Attaching Conjunctival Autografts after Pterygium Excision 
Purpose:
To report the efficacy, safety, and reliability of autologous cryoprecipitate in pterygium excision surgery and to compare it with the traditional method of using absorbable sutures, in regard to surgical time and the patient comfort.
Materials and Methods:
A prospective interventional clinical study was carried out in a specialized eye clinic. A total of 54 patients (90 eyes) underwent surgical excision of the nasal pterygium (whether primary or recurrent) with conjunctival autograft obtained from the same eye. Patients were divided into two groups. Autologous cryoprecipitate was used in 47 eyes (glue group), and absorbable sutures (8/0 vicryl) were used in 43 eyes (suture group) to attach the free conjunctival graft. There were 42 primary and 48 recurrent nasal pterygia that were included in the study. The surgical time was noted, and post-operative pain was graded. Follow-up period ranged from 6 months to 18 months (mean 12 months). P < 0.05 was statistically significant.
Results:
The medians of the visual analogue scale values were significantly lower in the glue group (P < 0.05). The median surgical time was statistically significantly lower at 11 min (range 9 min to 15 min) in the glue group, compared to 21 min (range 12 min to 28 min) for the suture group (P < 0.05). No significant intraoperative or post-operative complications were noted. Recurrence rate was 12%, and all recurrence cases occurred in the sutures group.
Conclusions:
Application of autologous cryoprecipitate glue instead of sutures for attaching the free conjunctival graft in pterygium surgery resulted in less post-operative pain and shorter surgical time. Additionally, there were no cases of recurrence during the follow-up in patients who received autologous cryoprecipitate glue during pterygium surgery.
doi:10.4103/0974-9233.114801
PMCID: PMC3757635  PMID: 24014989
Autologous Cryoprecipitate; Conjunctival Autografts; Pterygium Excision
21.  Primary Pterygium in a 7-Year-Old Boy: A Report of a Rare Case and Dilemma of its Management 
Primary pterygium in children is uncommon but is associated with severe visual problems. Astigmatism is the main visual problem caused by pterygium. Significant amounts of astigmatism occur long before a pterygium encroaches the visual axis. Early surgical intervention is safe and effective. It is associated with significant visual improvement in outcome. This is a case report on seven-year-old Malay boy who presented with a growth over nasal aspect of the right eye of 1 year duration. His right eye visual acuity is affected up to 6/12. The dilemma pased to early surgical interview is the high rate of recurrancean the young age group. This problem is highlighted in this case report.
PMCID: PMC3561894  PMID: 23386804
Pterygium; astigmatism
22.  Necrotising scleritis after bare sclera excision of pterygium 
The British Journal of Ophthalmology  2000;84(9):1050-1052.
AIM—To report cases of scleral necrosis after simple pterygium excision in which adjunctive treatment was not used.
METHODS—We reviewed four patients who presented with scleral melt after pterygium excision without the use of adjunctive treatment in the form of β irradiation, mitomycin C, or thiotepa. Each patient was thoroughly investigated to exclude underlying disease.
RESULTS—Certain similarities were found between our patients with pterygium melt and cases of surgically induced necrotising scleritis including the location of melt, associated inflammation, and its response to steroid treatment in the latent period before they presented.
CONCLUSION—Bare sclera pterygium excision can cause surgically induced necrotising scleritis years after the surgery.


doi:10.1136/bjo.84.9.1050
PMCID: PMC1723636  PMID: 10966964
23.  Epidemiology of pterygium in Victoria, Australia 
AIM—To describe the prevalence of and risk factors for pterygium in a population based sample of residents of the Australian state of Victoria who were aged 40 years and older.
METHODS—The strata comprised nine randomly selected clusters from the Melbourne statistical division, 14 nursing homes randomly selected from the nursing homes within a 5 kilometre radius of the nine Melbourne clusters, and four randomly selected clusters from rural Victoria. Pterygium was measured in millimetres from the tip to the middle of the base. During an interview, people were queried about previous ocular surgery, including surgical removal of pterygium, and their lifetime exposure to sunlight.
RESULTS—5147 people participated. They ranged in age from 40 to 101 years and 2850 (55.4%) were female. Only one person in the Melbourne cohort reported previous pterygium surgery, and seven rural residents reported previous surgery; this information was unavailable for the nursing home residents. Pterygium was present upon clinical examination in 39 (1.2%) of the 3229 Melbourne residents who had the clinical examination, six (1.7%) of the nursing home residents, and 96 (6.7%) of the rural residents. The overall weighted population rate in the population was 2.83% (95% CL 2.35, 3.31). The independent risk factors for pterygium were found to be age (OR=1.23, 95% CL=1.06, 1.44), male sex (OR=2.02, 95% CL=1.35, 3.03), rural residence (OR=5.28, 95% CL=3.56, 7.84), and lifetime ocular sun exposure (OR=1.63, 95% CL=1.18, 2.25). The attributable risk of sunlight and pterygium was 43.6% (95% CL=42.7, 44.6). The result was the same when ocular UV-B exposure was substituted in the model for broad band sun exposure.
CONCLUSION—Pterygium is a significant public health problem in rural areas, primarily as a result of ocular sun exposure.


doi:10.1136/bjo.84.3.289
PMCID: PMC1723391  PMID: 10684840
24.  Surgically induced necrotizing scleritis after primary pterygium surgery with conjunctival autograft 
Background
Although pterygium excision with conjunctival autograft is a widely performed surgical procedure, surgically induced necrotizing scleritis (SINS) following such surgery is extremely rare.
Methods
A 68-year-old man underwent nasal pterygium excision with conjunctival autograft uneventfully. On postoperative day 17, the conjunctival graft was avascular, with epithelial defect. Although topical steroid and antibacterial treatments were continued, the graft and sclera melted, with the ischemic sclera showing gradual thinning. The thinning area spread to the adjoining cornea, and active inflammation with epithelial defect was observed adjacent to the site of thinning.
Results
Systemic and microbiological examination was noncontributory. The patient was suspected of having SINS, and administration of oral prednisolone was started. Although the necrotic area was reduced temporarily, medication was discontinued due to nausea, and the area of thinning increased. Conjunctival flap surgery was later performed, and the graft was well accepted.
Conclusions
SINS must be considered in the differential diagnosis of patients with scleritis following pterygium surgery, especially if radiation or mitomycin C has not been used.
doi:10.2147/OPTH.S24885
PMCID: PMC3225457  PMID: 22140306
scleritis; pterygium; pterygium surgery; conjunctival autograft; SINS
25.  Long term results of intraoperative mitomycin C in the treatment of recurrent pterygium. 
AIM: The study was designed to evaluate the long term results of intraoperative mitomycin C in patients with one recurrence of pterygium. METHODS: In 45 white patients with one recurrence of pterygium the 'bare sclera technique' was performed and a sterile sponge soaked in a 0.2 mg/ml (0.02%) mitomycin C solution was placed intraoperatively on the sclera for 3 minutes. The control group underwent surgical excision only. Recurrences were analysed by the chi 2 test and the method of Kaplan-Meier (life table analysis); the difference between survival curves was tested by the log rank test. The chi 2 test with Yates's correction or Fisher's exact test were used to analyse the difference in complications and side effects between the two groups. RESULTS: After a mean postoperative follow up of 34.55 (SD 13.70) months, 6 recurrences (12.5%) were observed in the mitomycin C treated patients and 16 (35.6%) in the control patients (p = 0.027). The 24 and 48 month life table success rates were 89% and 83% in the mitomycin C treated group and 66% and 63% in the control group, respectively (p = 0.022). No severe side effects appeared during follow up. Superficial punctate keratitis appeared in the early postoperative period in only seven mitomycin C treated eyes (15.5%) (p = 0.018). CONCLUSION: This study confirms the efficacy of intraoperative mitomycin C in improving the success rate after recurrent pterygium surgical excision.
PMCID: PMC505450  PMID: 8703875

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