Ectasia

Corneal crosslinking (CXL) represents a paradigm shift in the management of corneal ectatic disorders. Before CXL was introduced, patients would need specialty contact lenses and possible corneal transplantation. CXL involves a biochemical reaction in which ultraviolet A light is used in conjunction with Riboflavin to form crosslinks in between corneal stromal collagen. This leads to strengthening and stabilizing of the collagen lamellae, resulting in mechanical stiffening of the cornea. Multiple protocols have been proposed including epithelium on versus off and varying light intensity and duration of treatment...

PURPOSE: Lamellar surgeries with SMILE lenticules are an evolving field of refractive surgery. This chapter intends to discuss the reported clinical results of using SMILE derived lenticules in terms of feasibility, safety and predictability; or the potential management of hyperopia, keratoconus, SMILE ectasia and presbyopia. MATERIALS AND METHODS: Donor SMILE lenticules were prepared under microscope tocreate doughnut shaped lenticules. For hyperopia, this tissue was then inserted into afemtosecond laser enabled pocket created using VisuMax FS Laser at a depth of 160µm...

We report the use of computerized tomography (CT)-guided navigation for complex spinal deformity correction (anterior and posterior) in an 8-year-old patient with neurofibromatosis complicated by dystrophic pedicles, dural ectasia, and extensive vertebral scalloping. A retrospective review was conducted of the patient's medical records for the past 3 years, including the patient's office visit notes, operative reports, pre- and 2-year postoperative imaging studies. The patient successfully underwent anterior lumbar interbody fusion from L3-S1 using CT-guided navigation to negotiate the challenges posed by dural ectasia and vertebral body scalloping...

Neurofibromatosis-1 (NF1) presents complex challenges due to its multisystemic effects, including kyphoscoliosis, dural ectasia, and arteriovenous fistulas (AVF). We present a case of a 31-year-old male with NF1 exhibiting severe cervical kyphoscoliosis, dural ectasia, a bisected cervical cord, and an arteriovenous fistula, highlighting the intricacies of managing such intricate cases. Rapid weakening in the patient's right arm and leg prompted imaging revealing severe cervical kyphotic deformity and a dural fold dividing the spinal cord...

Tubular ectasia of the rete testis (TERT) presents as multiple cystic structures within the rete testis, often incidentally detected on ultrasound as echo-free intratesticular cystic lesions. Despite its benign nature, assessing testicular cystic lesions can sometimes be challenging. The primary importance of identifying this uncommon condition lies in its distinction from cystic testicular malignancies and thus avoiding further radical procedures. We report an instance of TERT within the right testis discovered incidentally in a patient with a medical background of epididymitis, presenting for management of left testis cryptorchidism, and bilateral inguinal hernia...

INTRODUCTION: Gastric antral vascular ectasia (GAVE) is a rare cause of chronic or acute gastrointestinal bleeding. This condition accounts for ∼4% of upper gastrointestinal bleeding cases. This disease is often associated with systemic diseases, such as liver cirrhosis, chronic kidney failure, autoimmune conditions, diabetes mellitus, hypothyroidism, and cardiovascular diseases. However, its etiopathogenesis remains controversial. MATERIALS AND METHOD: We retrospectively reviewed the cases of GAVE treated at our digestive surgery unit...

BACKGROUND: Keratoconus (KC) is degenerative corneal disorder, with central and paracentral thinning and corneal ectasia. For KC progressive cases, primary treatment included corneal collagen cross linking (CXL) to stabilize coning and intracorneal rings segment (ICRS) to correct visual acuity. AIM: The aim of the study is to assess efficacy and safety of ICRS and CXL on one session (Simultaneous) or two sessions (sequential) with maximum of 1 month apart. PATIENTS AND METHODS: This Prospective Intervention Comparative research made at Armed forces hospital, Cairo, Egypt from January 2017 to December 2019...

Coronary artery ectasia (CAE), defined as a 1.5-fold or greater enlargement of a coronary artery segment compared to the adjacent normal coronary artery, is frequently associated with atherosclerotic coronary artery disease (CAD). Membrane-bound endothelin converting enzyme-1 (ECE-1) is involved in the maturation process of the most potent vasoconstrictor ET-1. Polymorphisms in the endothelin (ET) gene family have been shown associated with the development of atherosclerosis. This study aims to investigate the effects of rs213045 and rs2038089 polymorphisms in the ECE-1 gene which have been previously shown to be associated with atherosclerosis and hypertension (HT), in CAE patients...

Keratoglobus is a rare subset of noninflammatory corneal ectasia, which is a group of disorders characterized by corneal thinning, projection, and scarring. Patients with keratoglobus commonly present with poor vision. A case of advanced keratoglobus was managed by a modified phacoemulsification surgical technique using endoillumination and capsular staining with trypan blue. In this case, we present a 54-year-old man with keratoglobus. In January 2023, a modified phacoemulsification surgical technique using endoillumination was described with a video in a patient with bilateral corneal opacification, neovascularization, significant peripheral thinning, and moderate to severe corneal opacity in which cataract surgery had to be performed alone without considering penetrating keratoplasty...

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PURPOSE: Dural ectasia (DE) may significantly impact Marfan syndrome (MFS) patients' quality of life due to chronic lower back pain, postural headache and urinary disorders. We aimed to evaluate the association of quantitative measurements of DE, and their evolution over time, with demographic, clinical and genetic characteristics in a cohort of MFS patients. METHODS: We retrospectively included 88 consecutive patients (39% females, mean age 37.1 ± 14...

Keratoconus (KC) is the most common corneal ectasia. Optical coherence tomography angiography (OCT-A) is a relatively new non-invasive imaging technique that allows the visualization and quantification of retinal and choriocapillary blood vessels. The aim of this study is to assess retinal and choriocapillary vessel density (VD) differences between KC patients and healthy controls and to investigate correlations between VD and KC severity. Fifty-two eyes were included in this exploratory study: twenty-six eyes from 26 KC patients and twenty-six eyes from 26 age- and gender-matched healthy controls...

(1) Background: Our previous data indicated that disturbance of the Transforming Growth Factor beta (TGFB) signaling pathway via its Type-2 Receptor (TGFBR2) can cause a Corneal Ectasia (CE)-like phenotype. The purpose of this study is to elucidate whether the SMAD4-dependent signaling pathway is involved in the TGFBR2-related CE-like pathogenesis. (2) Methods: Smad4 was designed to be conditionally knocked out from keratocytes. Novel triple transgenic mice, KerartTA ; Tet-O-Cre; Smad4flox/flox ( Smad4kera-cko ), were administered with doxycycline (Dox)...

INTRODUCTION: Keratoconus is a progressive disorder of the cornea that causes thinning (Sedaghat et al. in Sci Rep 11(1):11971, 2021), ectasia, and irregular astigmatism, resulting in poor visual acuity that cannot be corrected with standard sphero-cylindrical spectacle lenses. One feature distinguishing keratoconic corneas is ocular aberrations, manifesting up to five or six times the amount of higher-order aberrations than a normal, healthy eye. These aberrations can cause visual disturbances even at the very early stages of the disease...

BACKGROUND: Keratoconus (KCN) is a progressive corneal ectasia that manifests at a young age and significantly impacts vision and quality of life. Early diagnosis allows for effective treatment with corneal collagen crosslinking, yet there is a lack of screening methods. This research aims to screen adolescents and young adults for this sight-threatening disease using quick corneal tomography mapping. METHODS: This prospective cross-sectional study is being conducted at Johns Hopkins Aramco Healthcare in Saudi Arabia, focusing on subjects aged 13-23...

BACKGROUND: Although the implication of receptor of advanced glycation endproducts (RAGE) has been reported in coronary artery disease, its roles in coronary artery ectasia (CAE) have remained undetermined. Furthermore, the effect of RAGE polymorfisms were not well-defined in scope of soluble RAGE (sRAGE) levels. Thus, we aimed to investigate the influence of the functional polymorphisms of RAGE -374T > A (rs1800624) and G82S (rs2070600) in CAE development. METHODS: This prospective observational study was conducted in 2 groups selected of 2452 patients who underwent elective coronary angiography (CAG) for evaluation after positive noninvasive heart tests...

This report presents a case of a 16-year-old male with severe upper gastrointestinal bleeding caused by a Dieulafoy lesion (DL). A DL is a rare but life-threatening condition characterized by sudden and massive bleeding from a small arterial vessel in the gastrointestinal (GI) tract. Diagnosis is often made through esophagogastroduodenoscopy (EGD), which reveals an enlarged submucosal blood vessel. The patient was successfully treated with adrenaline injection and hemoclipping during EGD. This case highlights the importance of considering a DL as a potential cause of severe upper GI bleeding in pediatric patients and emphasizes the significance of early recognition and intervention to achieve favorable outcomes...

Purpose: To evaluate the change in corneal biomechanics in patients with postoperative ectasia risk when combining two common laser vision correction procedures (tPRK and FS-LASIK) with cross-linking (in tPRK Xtra and FS-LASIK Xtra). Methods: The study included 143 eyes of 143 myopic, astigmatic patients that were divided into non-cross-linked refractive surgery groups (non-Xtra groups, tPRK and FS-LASIK) and cross-linked groups (Xtra groups, tPRK Xtra and FS-LASIK Xtra) according to an ectasia risk scoring system...

While the typical patient with idiopathic intracranial hypertension (IIH) is an obese female of childbearing age, there are unique patient populations, such as non-obese females, that have not been well studied. Characterizing this subpopulation may increase awareness our of it, which may prevent underdiagnosis and improve our understanding of IIH's underlying pathophysiology. We retrospectively reviewed electronic medical records and compared the clinical and radiological characteristics of non-obese (BMI < 30) and obese (BMI > 30) female patients with IIH...

Infectious aortitis is a rare disease process which can be of fungal, viral or bacterial aetiology. This disease process is often incidentally found during concomitant infectious processes, likely due to haematogenous spread. Common sources are from cardiac, genitourinary and gastroenterologic sources. CT imaging of the aorta is essential in identifying physiological changes-wall thickness changes, ectasia and stenosis. We present a case of a female in her early 60s with a medical history of cardiomyopathy with heart failure and reduced ejection fraction, who was initially admitted for acute cholecystitis complicated by the development of gallstone pancreatitis...