Growth, filtration and respiration characteristics of small single-osculum demosponge Halichondria panicea explants.

Filter-feeding demosponges are modular organisms that consist of modules each with one water-exit osculum. Once a mature module has been formed, the weight-specific filtration and respiration rates do not change. Sponge modules only grow to a certain size and for a sponge to increase in size, new modules must be formed. However, the growth characteristics of a small single-osculum module sponge are fundamentally different from those of multi-modular sponges, and a theoretically derived volume-specific filtration rate scales as F/V=V-1/3, indicating a decrease with increasing total module volume (V, cm3). Here, we studied filtration rate (F, l h-1), respiration rate (R, ml O2 h-1), volume-specific (F/V) and weight-specific (F/W) filtration rates, and the ratios F/R and F/W along with growth rates of small single-osculum demosponge Halichondria panicea explants of various sizes exposed to various concentrations of algal cells. The following relationships were found: F/V=7.08V-0.24, F=a1W1.05, and R=a2W0.68 where W is the dry weight (mg). The F/R and F/W ratios were constant and essentially independent of W, and other data indicate exponential growth. It is concluded that the experimental data support the theoretical F/V∝V-1/3.

TUBB3 Arg262His causes a recognizable syndrome including CFEOM3, facial palsy, joint contractures, and early-onset peripheral neuropathy.

Microtubules are formed from heterodimers of alpha- and beta-tubulin, each of which has multiple isoforms encoded by separate genes. Pathogenic missense variants in multiple different tubulin isoforms cause brain malformations. Missense mutations in TUBB3, which encodes the neuron-specific beta-tubulin isotype, can cause congenital fibrosis of the extraocular muscles type 3 (CFEOM3) and/or malformations of cortical development, with distinct genotype-phenotype correlations. Here, we report fourteen individuals from thirteen unrelated families, each of whom harbors the identical NM_006086.4 (TUBB3):c.785G>A (p.Arg262His) variant resulting in a phenotype we refer to as the TUBB3 R262H syndrome. The affected individuals present at birth with ptosis, ophthalmoplegia, exotropia, facial weakness, facial dysmorphisms, and, in most cases, distal congenital joint contractures, and subsequently develop intellectual disabilities, gait disorders with proximal joint contractures, Kallmann syndrome (hypogonadotropic hypogonadism and anosmia), and a progressive peripheral neuropathy during the first decade of life. Subsets may also have vocal cord paralysis, auditory dysfunction, cyclic vomiting, and/or tachycardia at rest. All fourteen subjects share a recognizable set of brain malformations, including hypoplasia of the corpus callosum and anterior commissure, basal ganglia malformations, absent olfactory bulbs and sulci, and subtle cerebellar malformations. While similar, individuals with the TUBB3 R262H syndrome can be distinguished from individuals with the TUBB3 E410K syndrome by the presence of congenital and acquired joint contractures, an earlier onset peripheral neuropathy, impaired gait, and basal ganglia malformations.

Is Tadpole Pupil in an Adolescent Girl Caused by Denervation Hypersensitivity?

Tadpole pupil is a rarely encountered phenomenon caused by episodic, segmental iris dilator muscle spasm of short duration (2-15 minutes), occurring in clusters without a known precipitating factor. It has most commonly been described in women aged 28 to 48 years. A few hypotheses on pathogenesis have been discussed but none has been proved. Here, we present an adolescent girl with bilateral tadpole pupil that appeared during physical exercise. This is the first pediatric case of tadpole pupil, not caused by preceding surgery, to be published. Based on (1) this case in which tadpole pupil developed when the norepinephrine level rose during exercise, (2) the high ratio of patients with tadpole pupil who concurrently have or later develop Horner syndrome, in which denervation hypersensitivity is well described, (3) a previous report of a patient with both tadpole pupil and Horner syndrome who had denervation hypersensitivity on pharmacological testing, (4) a 29-year-old man with unilateral tadpole pupil induced by exercise, and (5) a 19-year-old man with bilateral tadpole pupil and possible autonomic neuropathy, we suggest denervation hypersensitivity as a probable pathogenic mechanism causing tadpole pupil. In addition, a suggestion for investigations to be performed in future pediatric cases is provided.

Long-term results using LigaSure™ 5 mm instrument for treatment of Zenker's diverticulum.

The purpose of the present study was to evaluate the long-term results and patient's satisfaction of a new approach using the LigaSure™ 5 mm instrument for treatment of Zenker's diverticulum (ZD) and to compare with other long-term results using traditional treatment modalities. Between December 2011 and August 2013, a total of 23 patients with ZD underwent endoscopic surgery using the LigaSure™ technique in our department. A retrospective evaluation of the surgery was based on medical records and additionally a long-term follow-up was performed using a standardized questionnaire that was send to all patients. The questions dealt with complaints according to a visual analog scale (VAS) and were sent a minimum of one year after the surgery (mean time 22 months, range 12-32 month). The overall response rate was 91%. The mean age of the patients was 69 years (range 37-89 years). The patients reported nine for overall satisfaction on the VAS (range 0-10: 10 being very content and 0 very uncontent, 25 and 75% quartiles: 7 and 10) regarding the final outcome of their surgery, although several of the patients had continuous symptoms within the first postoperative year. Eight patients (38%) reported no symptoms at all. Our results suggest that endoscopic management of ZD with the LigaSure™ 5 mm instrument is a minimally invasive, fast and safe method with solid long-term outcome with relief of symptoms and patient satisfaction. This new operative instrument was not found inferior to traditional endoscopic techniques and is now the standard treatment method for ZD in our departments.

Primary transoral robotic surgery with concurrent neck dissection for early stage oropharyngeal squamous cell carcinoma implemented at a Danish head and neck cancer center: a phase II trial on feasibility and tumour margin status.

There is an increasing incidence of oropharyngeal squamous cell carcinoma (OPSCC) in the western world due to human papillomavirus (HPV). According to the Danish Head and Neck Cancer Group guidelines, the current recommended treatment of patients with OPSCC in Denmark is primary radiation therapy (RT) with or without concomitant chemotherapy. This is the first study in Scandinavia from a head and neck cancer centre that aims to demonstrate the feasibility of performing primary transoral robotic surgery (TORS) and concurrent neck dissection for patients with early stage OPSCC. Between September 2014 and January 2016, 30 consecutive patients with clinical T1-T2, N0-N1 OPSCC underwent primary TORS and concurrent neck dissection. The patients were offered postoperative adjuvant therapy according to pathological risk parameters: pT >2, T-site margin <2 mm, pN >1 or extracapsular extension (ECE). Concomitant chemotherapy was offered to patients with the presence of ECE or involved margins. Twenty-nine patients had negative margins on T-site after primary resection. Only one patient had a close margin of 1 mm. Unilateral neck dissection was performed in 21 patients while nine patients underwent bilateral neck dissection. Due to an upstaging following surgery, 13 patients were referred to adjuvant therapy. Four of these patients received RT and two patients received concomitant chemo-radiation (CCR) therapy. Seven patients declined the recommended adjuvant therapy one of whom later developed an N-site recurrence and received salvage surgery with postoperative RT. In summary, 43% of the patients were referred to adjuvant therapy following primary surgery which was mainly due to N-site stage migration and ECE. Primary TORS and concurrent neck dissection is a safe and feasible procedure that may be an alternative to primary RT and CCR in a selected group of patients with early stage OPSCC.

IC3D Classification of Corneal Dystrophies-Edition 3.

PURPOSE: The International Committee for the Classification of Corneal Dystrophies (IC3D) was created in 2005 to develop a new classification system integrating current information on phenotype, histopathology, and genetic analysis. This update is the third edition of the IC3D nomenclature. METHODS: Peer-reviewed publications from 2014 to 2023 were evaluated. The new information was used to update the anatomic classification and each of the 22 standardized templates including the level of evidence for being a corneal dystrophy [from category 1 (most evidence) to category 4 (least evidence)]. RESULTS: Epithelial recurrent erosion dystrophies now include epithelial recurrent erosion dystrophy, category 1 ( COL17A1 mutations, chromosome 10). Signs and symptoms are similar to Franceschetti corneal dystrophy, dystrophia Smolandiensis, and dystrophia Helsinglandica, category 4. Lisch epithelial corneal dystrophy, previously reported as X-linked, has been discovered to be autosomal dominant ( MCOLN1 mutations, chromosome 19). Classic lattice corneal dystrophy (LCD) results from TGFBI R124C mutation. The LCD variant group has over 80 dystrophies with non-R124C TGFBI mutations, amyloid deposition, and often similar phenotypes to classic LCD. We propose a new nomenclature for specific LCD pathogenic variants by appending the mutation using 1-letter amino acid abbreviations to LCD. Pre-Descemet corneal dystrophies include category 1, autosomal dominant, punctiform and polychromatic pre-Descemet corneal dystrophy (PPPCD) ( PRDX3 mutations, chromosome 10). Typically asymptomatic, it can be distinguished phenotypically from pre-Descemet corneal dystrophy, category 4. We include a corneal dystrophy management table. CONCLUSIONS: The IC3D third edition provides a current summary of corneal dystrophy information. The article is available online at https://corneasociety.org/publications/ic3d .

High reliability of an algorithm for choice of implants in hip fracture patients.

PURPOSE: Hip fracture treatment is controversial, with high complication rates. An algorithm for hip fracture surgery has shown reduced reoperation rates, but choice of implant is based on the commonly used fracture classifications, which were previously evaluated to be unreliable. The purpose of this study was to investigate the reliability of the algorithm. METHODS: From two hospitals, four observers (orthopaedic consultant, fellow, resident and intern) used the algorithm to classify into 15 hip fracture types [Garden type I-IV femoral neck including posterior tilt, vertical femoral neck, basocervical and Arbeitsgemeinschaft für Osteosynthesefragen (AO)-31 A1.1 to A3.3 trochanteric fractures] and to choose between five surgical procedures [parallel implants, prosthesis, two-or four-hole sliding hip screw (SHS) and intermedullary (IM) nail]. After individual assessment, each hospital made a consensus decision. Observations were performed twice, ten weeks apart, on pelvic, anteroposterior (AP) and axial X-rays from 100 consecutive patients. RESULTS: For fracture classification, mean kappa values were 0.60 for intra and 0.62 for interobserver variation, with interobserver variation between hospitals at 0.65. For posterior tilt, mean intraclass correlation coefficient was 0.91 for intra and 0.87 for interobserver variation. For choice of implant type, mean kappa values were 0.86 for both intra and interobserver variation. The two hospital consensus decisions chose same implant in 91 of 100 patients, giving a kappa value at 0.88. CONCLUSION: Although hip fracture classification confirmed to be somewhat unreliable in this study, posterior tilt measurement and subsequent choice of implant type by the algorithm was found to be reliable, which opens up the possibility for a more standardized treatment of hip fracture patients between hospitals.

Fate of microplastic captured in the marine demosponge Halichondria panicea.

Microplastic particles are widespread pollutants in the sea and filter-feeding sponges have recently been suggested as useful monitoring organisms. However, the fate of microplastic particles in sponges is poorly understood, yet crucial for interpreting monitoring data. The present study aims to help develop sponges as more useful monitoring organisms for microplastic in the sea. Here, we describe the fate of inedible (2 and 10 µm) plastic beads compared to that of edible bacteria and algal cells captured in the marine demosponge Halichondria panicea. Small Cyanobium bacillare cells entered the choanocyte chambers and were phagocytized by choanocytes, while larger Rhodomonas salina cells were captured in incurrent canals and phagocytized in the mesohyl. Small 2 µm-beads were captured by choanocytes and subsequently expelled into the excurrent canals after 58 ± 34 min. Larger 10 µm-beads were captured in the incurrent canals and transferred to the mesohyl, where amoeboid cells moved them across the mesohyl before they were expelled into the excurrent canal after 95 ± 36 min. SEM observations further indicated engulfment of plastic beads on the outer sponge surface. This insight provides useful information on how sponges, in general, treat microplastic particles of various sizes. It helps us understand actual measured sizes and concentrations of microplastic particles in sponges in relation to those in the ambient water.

von Hippel-Lindau disease: Updated guideline for diagnosis and surveillance.

von Hippel Lindau disease (vHL) is caused by a hereditary predisposition to multiple neoplasms, especially hemangioblastomas in the retina and CNS, renal cell carcinomas (RCC), pheochromocytomas, neuroendocrine pancreatic tumours (PNET) and endolymphatic sac tumours. Evidence based approaches are needed to ensure an optimal clinical care, while minimizing the burden for the patients and their families. This guideline is based on evidence from the international vHL literature and extensive research of geno- and phenotypic characteristics, disease progression and surveillance effect in the national Danish vHL cohort. We included the views and preferences of the Danish vHL patients, ensured consensus among Danish experts and compared with international recommendations. RECOMMENDATIONS: vHL can be diagnosed on clinical criteria, only; however, in most cases the diagnosis can be supported by identification of a pathogenic or likely pathogenic variant in VHL. Surveillance should be initiated in childhood in persons with, or at risk of, vHL, and include regular examination of the retina, CNS, inner ear, kidneys, neuroendocrine glands, and pancreas. Treatment of vHL manifestations should be planned to optimize the chance of cure, without unnecessary sequelae. Most manifestations are currently treated by surgery. However, belzutifan, that targets HIF-2α was recently approved by the U.S. Food and Drug Administration (FDA) for adult patients with vHL-associated RCC, CNS hemangioblastomas, or PNETs, not requiring immediate surgery. Diagnostics, surveillance, and treatment of vHL can be undertaken successfully by experts collaborating in multidisciplinary teams. Systematic registration, collaboration with patient organisations, and research are fundamental for the continuous improvement of clinical care and optimization of outcome with minimal patient inconvenience.

von Hippel-Lindau disease: surveillance strategy for endolymphatic sac tumors.

PURPOSE: : Up to 16% of patients with the hereditary von Hippel-Lindau disease develop endolymphatic sac tumors of the inner ear. Early diagnosis and treatment of endolymphatic sac tumors can prevent audiovestibular morbidity, but optimal endolymphatic sac tumor surveillance strategy has yet to be determined. We aimed to evaluate endolymphatic sac tumor surveillance to determine the best surveillance strategy. METHODS: : In a national prospective study, 40 VHL mutation carriers were interviewed about audiovestibular symptoms and had audiological examinations and magnetic resonance imaging of the inner ear. Further, we performed a meta-analysis including all reported endolymphatic sac tumor von Hippel-Lindau disease cases in the literature (N = 140 with 156 endolymphatic sac tumors). RESULTS: : In the prospective study, endolymphatic sac tumors were suspected based on audiovestibular symptoms, audiometry, and magnetic resonance imaging in 34%, 30%, and 12.5% of subjects, respectively. In total, more than 90% of radiologically diagnosed endolymphatic sac tumors were associated with abnormal audiometric findings. No endolymphatic sac tumor genotype-phenotype correlations were found. CONCLUSION: : We recommend annual audiometry as a first-line endolymphatic sac tumor screening tool, and in countries where periodic surveillance magnetic resonance imaging of the central nervous system is performed, specific images of the inner ear should be included. Audiometric abnormalities in patients with von Hippel-Lindau disease without magnetic resonance imaging-visible endolymphatic sac tumors could be due to microscopic endolymphatic sac tumors. Determination of audiometric endolymphatic sac tumor characteristics could further target screening and improve endolymphatic sac tumor diagnosis.

Thoracic imaging.

The various techniques encompassed in the term 'Thoracic Imaging' have had a dramatic effect on the practice of respiratory medicine over the last 25 years. One of many examples is the increased precision with which lung cancer can be preoperatively staged using CT and PET imaging. The increasing sophistication of thoracic imaging tests brings many benefits, but there are caveats. This review considers a selection of techniques and contains state-of-the-art commentaries by distinguished experts on current challenges and likely future developments.

Hydrodynamics of the leucon sponge pump.

Leuconoid sponges are filter-feeders with a complex system of branching inhalant and exhalant canals leading to and from the close-packed choanocyte chambers. Each of these choanocyte chambers holds many choanocytes that act as pumping units delivering the relatively high pressure rise needed to overcome the system pressure losses in canals and constrictions. Here, we test the hypothesis that, in order to deliver the high pressures observed, each choanocyte operates as a leaky, positive displacement-type pump owing to the interaction between its beating flagellar vane and the collar, open at the base for inflow but sealed above. The leaking backflow is caused by small gaps between the vaned flagellum and the collar. The choanocyte pumps act in parallel, each delivering the same high pressure, because low-pressure and high-pressure zones in the choanocyte chamber are separated by a seal (secondary reticulum). A simple analytical model is derived for the pump characteristic, and by imposing an estimated system characteristic we obtain the back-pressure characteristic that shows good agreement with available experimental data. Computational fluid dynamics is used to verify a simple model for the dependence of leak flow through gaps in a conceptual collar-vane-flagellum system and then applied to models of a choanocyte tailored to the parameters of the freshwater demosponge Spongilla lacustris to study its flows in detail. It is found that both the impermeable glycocalyx mesh covering the upper part of the collar and the secondary reticulum are indispensable features for the choanocyte pump to deliver the observed high pressures. Finally, the mechanical pump power expended by the beating flagellum is compared with the useful (reversible) pumping power received by the water flow to arrive at a typical mechanical pump efficiency of about 70%.

[Diagnosis, monitoring and treatment of tuberous sclerosis complex].

Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder with highly varying disease manifestations, many of which cause extensive morbidity. There are international consensus criteria for the diagnosis, monitoring and treatment of TSC, and approved medical treatment for some of the most serious disease manifestations. However, organisation of a rational and coordinated care of TSC patients involves many different medical specialities and is only sparsely described. This review describes the interdisciplinary care of TSC patients at Aarhus University Hospital, Denmark.

Retinal hemangioblastoma: prevalence, incidence and frequency of underlying von Hippel-Lindau disease.

BACKGROUND AND AIMS: We aimed to determine the frequency of von Hippel-Lindau disease (vHL) as the underlying cause of retinal hemangioblastoma and to estimate retinal hemangioblastoma incidence and prevalence in a national cohort study. METHODS: Through the national patient register and vHL research database, we identified 81 patients diagnosed with a retinal hemangioblastoma in Denmark between 1977 and 2014. Consent was obtained for 54 living and 10 deceased patients with retinal hemangioblastoma. For each participant, we collected medical records and family information. Almost all (63 of 64) participants were or had previously been tested for mutations in the VHL gene. RESULTS: Overall, 84% of the participants (54 of the 64) had vHL. Compared with the non-vHL patients, the vHL patients had their first retinal hemangioblastoma at a younger age (22.5 vs 40 years), and were more likely to have an asymptomatic first hemangioblastoma (80% vs 20%). Overall, 76% (41 of 54) of the vHL patients had a family history of vHL, while none of the patients without vHL did. Despite the rarity of the disease, on average more than eight new tumours are diagnosed each year due to multiple tumour development in vHL patients. The estimated prevalence of patients with retinal hemangioblastoma was up to 1 in 73 080 individuals. CONCLUSION: In the first national study in which almost all participants were genetically tested, vHL was the underlying cause of retinal hemangioblastoma in 84% of cases; more often than previously reported. We recommend that genetic and clinical vHL screening should be performed in all patients with retinal hemangioblastoma.

Biomechanics and energy cost of the amphipod Corophium volutator filter-pump.

The integrated function of the setal filter-basket and the pleopodal pump in the burrowing amphipod Corophium volutator was studied by video-microscopy in order to evaluate the energy costs of filter feeding. Video-microscope observations indicated that, in general, nine short, water-pumping beat cycles of the pleopods are succeeded by one slow cycle that coincides with cleaning of the setal filter and transient slowdown of inhalant water velocity. The position of the plumose setal filter on the second pair of gnathopods ensures that all water runs through the filter-basket. The fine V-shaped bristles on the setae enlarge the total filter area so that the velocity of water flowing through the filter is relatively slow, about 2.5 mm s(-1), giving rise to a resistance of about 2.9 mm H(2)O, which is the most important contribution to the total pressure drop in the system. In "standard" individuals of C. volutator with lengths of 3 and 6 mm, the normal operating pump pressure and pumping rate were, respectively, 2.6 and 3.1 mm H(2)O, and 18.3 and 85.5 ml h(-1); the overall pump efficiencies were 5.1% and 11.6%, respectively. These results show that the Corophium filter-pump is comparable to other low-pressure biological pumps in filter-feeding marine invertebrates, such as mussels, polychaetes, ascidians, and bryozoans.

Interstitial deletion of the short arm of chromosome 1 (1p13.1p21.1) in a girl with mental retardation, short stature and colobomata.

Interstitial deletions on the short arm of chromosome 1 are rare. We describe a girl with severe mental retardation, short stature and dysmorphic features including colobomata where high-resolution comparative genomic hybridization revealed an interstitial deletion with breakpoints in band 1p13.1 and 1p21.1. The deletion was further characterized by real-time polymerase chain reaction. We hypothesize that haploinsufficiency of WNT2B (wingless-type MMTV integration site family, member 2B) and NTNG1 (Netrin G1) contributed to the patient's phenotype.

ICP Materials Trends in Corrosion, Soiling and Air Pollution (1987-2014).

Results from the international cooperative programme on effects on materials including historic and cultural monuments are presented from the period 1987-2014 and include pollution data (SO2, NO2, O3, HNO3 and PM10), corrosion data (carbon steel, weathering steel, zinc, copper, aluminium and limestone) and data on the soiling of modern glass for nineteen industrial, urban and rural test sites in Europe. Both one-year and four-year corrosion data are presented. Corrosion and pollution have decreased significantly and a shift in the magnitude is generally observed around 1997: from a sharp decrease to a more modest decrease or to a constant level without any decrease. SO2 levels, carbon steel and copper corrosion have decreased even after 1997, which is more pronounced in urban areas, while corrosion of the other materials shows no decrease after 1997, when looking at one-year values. When looking at four-year values, however, there is a significant decrease after 1997 for zinc, which is not evident when looking at the one-year values. This paper also presents results on corrosion kinetics by comparison of one- and four-year values. For carbon steel and copper, kinetics is relatively independent of sites while other materials, especially zinc, show substantial variation in kinetics for the first four years, which needs to be considered when producing new and possibly improved models for corrosion.

Tooth loss treatment in the anterior region: autotransplantation of premolars and cryopreservation.

Avulsed and lost anterior teeth are common in young people. Using autotransplantation, it is possible to move problems in dental arches to regions where they are more easy to solve orthodontically. Transplantation of premolars with three-quarter root formation or full root formation with wide-open apical foramina provides the best prognosis for long-term survival. This article describes the use of autotransplantation and orthodontic treatment, together with cryopreservation, in connection with complicated trauma in the anterior region of an 8-year-old girl.

Transoral robotic surgery for the management of head and neck squamous cell carcinoma of unknown primary.

CONCLUSION: The addition of transoral robotic surgery (TORS) in the diagnostic management of patients classified with head and neck squamous cell carcinoma of unknown primary (SCCUP) is promising and appears to improve detection rates of the primary tumour. The approach presented in this first Scandinavian study could potentially minimize the radiation field to the pharyngeal axis in patients with identified primary tumours. OBJECTIVES: The aim of the study was to investigate whether bilateral lingual tonsillectomy performed with TORS is feasible, and whether it could improve the detection rates of primary tumours in patients diagnosed and classified as having SCCUP. METHODS: The study was retrospective and included 13 patients with SCCUP who were referred to TORS between October 2013 and January 2015. All 13 patients had previously undergone a full investigation programme following the national guidelines including whole-body PET/CT, examination in general anaesthesia, including random biopsies of the base of the tongue and bilateral palatine tonsillectomy without identification of the primary tumour. RESULTS: The primary tumour was identified by TORS in seven of the 13 patients (54%) at the lingual tonsils. Human papillomavirus DNA and p16 were positive in all identified primary tumour specimens and in the corresponding lymph node metastases.

IC3D classification of corneal dystrophies--edition 2.

PURPOSE: To update the 2008 International Classification of Corneal Dystrophies (IC3D) incorporating new clinical, histopathologic, and genetic information. METHODS: The IC3D reviewed worldwide peer-reviewed articles for new information on corneal dystrophies published between 2008 and 2014. Using this information, corneal dystrophy templates and anatomic classification were updated. New clinical, histopathologic, and confocal photographs were added. RESULTS: On the basis of revisiting the cellular origin of corneal dystrophy, a modified anatomic classification is proposed consisting of (1) epithelial and subepithelial dystrophies, (2) epithelial-stromal TGFBI dystrophies, (3) stromal dystrophies, and (4) endothelial dystrophies. Most of the dystrophy templates are updated. The entity "Epithelial recurrent erosion dystrophies" actually includes a number of potentially distinct epithelial dystrophies (Franceschetti corneal dystrophy, Dystrophia Smolandiensis, and Dystrophia Helsinglandica) but must be differentiated from dystrophies such as TGFBI-induced dystrophies, which are also often associated with recurrent epithelial erosions. The chromosome locus of Thiel-Behnke corneal dystrophy is only located on 5q31. The entity previously designated as a variant of Thiel-Behnke corneal dystrophy on chromosome 10q24 may represent a novel corneal dystrophy. Congenital hereditary endothelial dystrophy (CHED, formerly CHED2) is most likely only an autosomal recessive disorder. The so-called autosomal dominant inherited CHED (formerly CHED1) is insufficiently distinct to continue to be considered a unique corneal dystrophy. On review of almost all of the published cases, the description appeared most similar to a type of posterior polymorphous corneal dystrophy linked to the same chromosome 20 locus (PPCD1). Confocal microscopy also has emerged as a helpful tool to reveal in vivo features of several corneal dystrophies that previously required histopathologic examination to definitively diagnose. CONCLUSIONS: This revision of the IC3D classification includes an updated anatomic classification of corneal dystrophies more accurately classifying TGFBI dystrophies that affect multiple layers rather than are confined to one corneal layer. Typical histopathologic and confocal images have been added to the corneal dystrophy templates.