Acute corneal hydrops in keratoconus

Acute corneal hydrops is a condition characterized by stromal edema due to leakage of aqueous through a tear in descemet membrane. The patient presents with sudden onset decrease in vision, photophobia, and pain. Corneal thinning and ectasias combined with trivial trauma to the eye mostly by eye rubbing is considered as the underlying cause. With conservative approach self-resolution takes around 2 to 3 months. Surgical intervention is required in cases of non-resolution of corneal edema to avoid complications and for early visual rehabilitation. Intracameral injection of air or gas such as perflouropropane is the most common surgical procedure done. Recent investigative modality such as anterior segment optical coherence tomography is an extremely useful tool for diagnosis, surgical planning, and postoperative follow up. Resolution of hydrops may improve the contact lens tolerance and visual acuity but most cases require keratoplasty for visual rehabilitation.     

Acute corneal hydrops in keratoconus: a national prospective study of incidence and management

Purpose

To determine incidence and management of acute corneal hydrops in the UK.

Methods

We used the BOSU report card system to survey cases of acute corneal hydrops in patients with keratoconus that occurred in the UK between November 2009 and December 2010. Ophthalmologists who reported a case were sent an initial questionnaire, with a follow-up questionnaire after 6 months. We collected information on the demographics, complications, changes in visual acuity, and management. The 2011 National Census was used as a source for population and ethnicity in the UK.

Results

There were 73 incident cases of acute corneal hydrops, with a response to the initial questionnaire for 64 (88%) patients and follow-up data at 6 months for 57 (78%) patients. For the 64 confirmed cases the median (interquartile range) age of onset was 31.9 (23.2, 41.3) years and 48 (75%) of the cases occurred in males. A total of 42 (66%) patients were white, 14 (22%) were South Asian, and 7 (11%) were black. The proportion of South Asian and black patients with acute corneal hydrops was significantly higher than in the general population (P<0.001). The minimum estimated annual incidence of acute corneal hydrops in patients with keratoconus was estimated to be 1.43 (1.10, 1.83) per 1000. At 6 months following acute corneal hydrops a decision to proceed with keratoplasty had been made for 12 (20.3%) patients.

Conclusions

This is the first population-based estimate of the incidence of acute corneal hydrops in keratoconus.     

Late corneal hydrops after penetrating keratoplasty for keratoconus

PURPOSE: To report 2 cases of corneal hydrops occurring in eyes with previously healthy corneal grafts. METHODS: Retrospective case series. A report of 2 presenting cases. Both patients underwent a full ophthalmic clinical examination and anterior segment optical coherence tomography (OCT). RESULTS: Two patients with longstanding corneal grafts for keratoconus presented with an acute painful red eye and reduced vision. They were found to have acute hydrops with breaks in and detachments of Descemet membrane. CONCLUSIONS: Keratoconic patients with longstanding corneal grafts who present acutely with corneal edema must be carefully examined not only for signs of graft rejection or failure but also for acute hydrops. Important differentiating signs may be a Descemet break or detachment and the presence of stromal edema across the donor-host corneal interface.     

Bilateral keratoconus,acute hydrops and unilateral corneal perforation due to Tourette syndrome

A 27-year-old male with a repetitive behavior of eyeball pressing for six months presented with decreasing visual acuity for three months. Upon arrival his best corrected visual acuity was 0.2 on the right and 0.6 on the left eyes. Scheimpflug camera system demonstrated grade 4 and grade 2 keratoconus, respectively. Psychiatric consultation revealed Tourette syndrome and treatment was started. Despite psychiatric treatment, acute hydrops occurred in both eyes decreasing visual acuity to hand motions bilaterally. Left corneal perforation due to ongoing habit of eyeball pressure was experienced which led the loss of light perception and phtisis bulbi. Although Tourette syndrome is rare, it might cause bilateral acute onset keratoconus when the repetitive movements affect periocular region. Psychiatric treatment should be considered as early as possible in these patients in order to prevent unfavorable complications.     

One-step thermokeratoplasty for pain alleviating and pre-treatment of severe acute corneal hydrops in keratoconus

AIM:To observe the efficacy of simplified one-step thermokeratoplasty for the treatment of severe acute corneal hydrops in keratoconus.METHODS:In this retrospective,noncomparative clinical study,10 patients(10 eyes) with acute hydrops in keratoconus were treated with simplified one-step thermokeratoplasty.Pain,changes in the corneal curvature,thickness,and size and morphology of the Descemet membrane breaks were detected before and after surgery.Eight patients were successfully treated using modified deep anterior lamellar keratoplasty(DALK).Graft transparency,visual acuity,and immunological rejection were evaluated for 6 to 12mo.RESULTS:Pain and corneal oedema were promptly alleviated,and the intrastromal ruptures diminished within 3 to 6wk after thermokeratoplasty.At 3 to 6wk after corneal oedema was faded,Descemet membrane breaks and intrastromal ruptures were healed.Two patients did not undergo subsequent corneal transplantation after thermokeratoplasty.Eight patients underwent DALK successfully and safely after thermokeratoplasty,without corneal perforation.Central corneal opacity faded or disappeared within 6mo.The mean best-corrected visual acuity was increased to 20/30 at 12mo after DALK.No one was observed with any immune rejection.CONCLUSION:One-step thermokeratoplasty can successfully and efficiently accelerate the absorption of prominent corneal oedema in severe acute hydrops patients.This simple procedure with no complications can be performed in the emergency department by residents.This method can improve the safety of DALK and obtain good postoperative vision.Long-term management of acute corneal hydrops using simplified one-step thermokeratoplasty seems promising.     

角膜移植术后免疫排斥反应的药物治疗

目的 探讨治疗角膜移植术后排斥反应的有效药物.方法 我院近2年全身及局部应用抗排斥反应药物的方法治疗角膜移植术后的排斥反应成功者34例(48眼),进行回顾性研究分析,总结药物治疗的经验.结果 48眼成功病例均为局部应用皮质类固醇者.其中21眼配合全身应用皮质类固醇及免疫抑制剂;27眼局部滴用环胞霉素A滴眼液;4眼应用FKS06滴眼液.全部病例角膜植片恢复透明或半透明.46眼视力恢复至排斥反应发生前水平.结论 在诸多的抗排斥反应药物中,皮质类固醇仍然是首选及非常有效的药物.尤以局部应用为抗排斥反应必不可少的治疗措施.     

Acute corneal edema in pellucid marginal degeneration or acute marginal keratoconus

This article reports a case of bilateral corneal pellucid marginal degeneration. The right cornea had an acute hydrops. Both eyes underwent penetrating keratoplasty. A histopathological study of the corneal specimens was performed by light and electron microscopy. The histological changes observed on the right cornea showed breaks on Bowman's layer, edema and disorganization of the stromal collagen, and break of Descemet's membrane. The ultrastructural changes were similar to those observed in acute keratoconus, leading to the belief that these two corneal diseases are closely related.     

Intracameral air injection for acute hydrops in keratoconus

PURPOSE: To evaluate the efficacy and safety of intracameral air injection in treating acute hydrops in keratoconus. DESIGN: Retrospective, nonrandomized, comparative trial. METHODS: PATIENTS: Thirty eyes (30 patients) with acute hydrops secondary to keratoconus. INTERVENTION: Nine eyes (nine patients) with acute hydrops in keratoconus were treated with intracameral injection of 0.1 ml filtered air. Additional 0.1 ml filtered air was injected if corneal edema persisted when air disappeared from the anterior chamber. Twenty-one eyes (21 patients) with acute hydrops that received no therapy or conventional therapy not likely to shorten the duration of hydrops served as controls. MAIN OUTCOME MEASURES: The period of persistence of corneal edema, the interval between the onset of acute hydrops, and the time when the eye could begin to wear a hard-contact lens, and best spectacle-corrected and hard-contact lens-corrected visual acuity after corneal edema subsided were used as criteria to evaluate any differences between the two groups. RESULTS: The average period of persistence of corneal edema was 20.1 +/- 9.0 days (+/- SD) in the intracameral air injection group and 64.7 +/- 34.6 days in the control (P =.0008). The average interval between the onset of acute hydrops and the time when the eye could begin to wear a hard-contact lens, was 33.4 +/- 5.6 days in the air injection group and 128.9 +/- 85.8 days in the control group (P =.0058). The best-corrected visual acuity after corneal edema subsided was similar between the two groups. Intracameral air injection induced no complications. CONCLUSIONS: The results suggest that the intracameral air injection is a safe and useful therapy to shorten the period of corneal edema in acute hydrops secondary to keratoconus.     

Acute hydrops in pellucid marginal corneal degeneration

Three patients had pellucid marginal corneal degeneration complicated by corneal edema. The corneal edema appeared to be a result of a break or detachment of Descemet's membrane as a result of increasing corneal ectasia. The disruption in Descemet's membrane began just above the inferior, crescent-shaped area of stromal thinning. Therapeutic modalities initially included hypertonic solution to determine whether corneal edema would resolve spontaneously, apparently by endothelial migration with healing over the break in Descemet's membrane. One patient required thermokeratoplasty and another penetrating keratoplasty for persistent stromal edema. Acute hydrops can occur with pellucid marginal corneal degeneration by a pathogenesis similar to other noninflammatory corneal thinning disorders such as keratoconus.     

Spontaneous corneal hydrops and perforation in keratoconus and pellucid marginal degeneration

PURPOSE: To report two cases of pellucid marginal degeneration and one case of keratoconus associated with spontaneous corneal hydrops leading either to perforation or imminent perforation, requiring urgent keratoplasty. METHOD: Retrospective interventional case series of three patients with noninflammatory peripheral corneal degenerations. A retrospective review was done of the clinical courses, surgical interventions, and pathologic specimens, development of spontaneous hydrops, perforation, need for surgical intervention, and final visual outcome. RESULTS: Two patients with pellucid marginal degeneration and one with keratoconus developed spontaneous hydrops followed by aqueous leakage through markedly thinned anterior stroma. In one case, the leak site was successfully sealed after three separate applications of tissue adhesive, although the remaining two cases required penetrating keratoplasty. CONCLUSIONS: These cases document the very unusual occurrence of corneal hydrops leading to spontaneous corneal perforation in patients with keratoconus and pellucid marginal degeneration.     

Keratoconus with acute corneal hydrops

Acute corneal hydrops, a condition secondary to keratoconus, can be difficult to diagnose unless examination is also performed on the fellow eye. Keratoconic signs in the fellow eye will aid in the differential diagnosis of corneal hydrops. Treatment depends on the severity and includes spectacles, contact lenses, hypertonic saline, and ultimately penetrating keratoplasty or epikeratophakia.     

Atypical hydrops in keratoconus

To report the clinical presentation, progress and management of atypical acute hydrops. A retrospective case study of three patients with keratoconus, two of whom had previously undergone penetrating keratoplasty. The patients underwent full ophthalmological examination and digital slit-lamp imaging of the cornea throughout the course of the condition. The two patients who had previously undergone keratoplasty had spontaneous hydrops primarily affecting the host bed but in one case extended to the graft inferiorly; however, in the third patient it was traumatic in origin. The Descemet’s tear affected the host rim in only one patient, which resolved spontaneously. In another patient, the hydrops was related to an internal dehiscence of the graft–host junction and had to be managed by an endothelial transplant covering the dehisced graft–host junction. In the third patient, hydrops secondary to trauma was also associated with acute haemops. Progression of keratoconus post keratoplasty can occur exclusively in the recipient bed leading to acute hydrops in the host sparing the transplanted cornea. The progressive thinning and ectasia of the recipient bed can also result in internal graft–host dehiscence leading to chronic oedema. Rapid entry of aqueous or blood cells into the corneal stroma following acute rupture of the Descemet’s membrane suggests that the abnormal stroma of the eye with keratoconus may have an important role to play in the pathogenesis of acute hydrops/haemops.