Hydrophthalmos is the term used for enlargement of one or both eyes that is due to impaired aqueous humor outflow. Hydrophthalmos is associated with the congenital form of glaucoma. It is treated surgically.
What is hydrophthalmos?
The eye is a part of the central nervous system and enables visual impression through receptors and their connection to the brain. Glaucoma is also known as glaucoma. It is an eye disease that results in nerve fiber loss. Where the optic nerve exits, the optic nerve head progressively hollows out or atrophies as the disease progresses. Visual field loss occurs, which can increase to blindness of the eye. Glaucoma can be acquired or congenital. The congenital form is often accompanied by hydrophthalmos. This is a pathologically enlarged eyeball associated with high intraocular pressure. The condition is also known as buphthalmos or bull’s eye and may be present in one or both eyes. Often, infants with hydrophthalmos are easily shy in addition to the eyeball enlargement. Hydrophthalmos literally means “water eye” and never occurs in isolation. The phenomenon is practically always accompanied by the congenital form of glaucoma, which in this case is necessarily triggered by the increased intraocular pressure.
Causes
Congenital glaucoma is caused by congenital elevation of intraocular pressure. Affected individuals suffer from aqueous humor outflow obstruction due to embryonic developmental abnormalities of the chamber angle. In many cases, the embryonic developmental disorder additionally manifests in other parts of the body. As a primary cause of the developmental disorder, infection with rubella during early pregnancy is most common. Due to increased intraocular pressure, enlargement of the eyeball occurs on one or both sides. As soon as hydrophthalmos is accompanied by corneal opacity or photophobia, the diagnosis of congenital glaucoma applies on a larger scale. Infection with rubella does not always have to be responsible for the developmental disorder in the embryonic period. In some cases, hydrophthalmos and glaucoma also occur hereditarily. Due to the developmental disorder of the chamber angles, the draining paths of the eye close in hydrophthalmos, so that an excessive amount of aqueous humor accumulates. The name of eyeball enlargement as water eye is due to this connection.
Symptoms, complaints, and signs
Patients with hydrophthalmos suffer from “big eyes” with photophobia and eye tearing. In addition, blepharospasm may occur. Often, myopia is also present. Corneal opacification or Descement tears are also characteristic. The same is true for deep anterior chambers and narrowing of the chamber angle. In addition to atrophy of the iris, a de-rounded pupil may be present in the affected eye, which reacts pathologically. The uvea of the eye often shimmers through with a bluish tint. In most cases, intraocular pressures are elevated. Measurements are as high as 60 mmHg. The anterior segment dilates and the increase in pressure usually increases by the first year of life. The sclera and cornea lose their elasticity after the first year of development. For this reason, hydrophthalmos usually shows effects only on the optic nerve. Due to the occurring changes in the optic nerve head, optic atrophy usually occurs. In this atrophy, the optic nerve degrades piece by piece. The complete degradation of the optic nerve results in blindness. The phenomenon is present either on both sides or only on one eye.
Diagnosis and course of the disease
Usually, hydrophthalmos is diagnosed immediately after birth. The unilateral form, in particular, can already be guessed at purely by visual diagnosis. The bilateral form is sometimes not recognized immediately after birth. The diagnosis includes a careful anamnesis and intraocular pressure measurement. Ophthalmologic examination often corresponds to microscopy of the anterior and middle eye segment in combination with gonioscopy, examination of the fundus of the eye or skiascopy. Due to the young age of the patients, the procedures are usually performed under anesthesia. Differential diagnosis is to exclude space-occupying processes and inflammation. Hydrophthalmos is prognostically rather unfavorable. Blindness is likely in the affected eye.
Complications
Hydrophthalmos usually requires surgical treatment to relieve symptoms. In most cases, the disease causes the patient to become myopic. Likewise, the cornea becomes cloudy and the chamber angle is narrowed. This leads to considerable limitations in the patient’s vision, so that the quality of life is also severely restricted by hydrophthalmos. As a rule, vision deteriorates and decreases with age. The optic nerve also recedes in the patient, so that in the worst case complete blindness of the patient occurs. Hydrophthalmos does not necessarily occur in both eyes. In most cases, the disease is diagnosed relatively early and without complications, so that early treatment is possible. In this case, too, no particular complications occur and the symptoms disappear after the procedure. In most cases, the visual acuity can also be restored by the operation, so that the affected person no longer suffers from any complaints. Life expectancy is not reduced by the disease. However, if the treatment is performed too late, permanent blindness may result.
When should you see a doctor?
Hydrophthalmos is usually diagnosed immediately after birth. Whether medical treatment is needed depends, among other things, on whether the child is experiencing discomfort. For example, pain or visual disturbances require medical clarification in any case. Externally visible changes in the eyes should also be examined to ensure that there are no vascular injuries. In most cases, drug treatment is sufficient to reverse the enlargement of one or both eyes. However, because young children are sometimes sensitive to medications, parents should pay close attention to any side effects or drug interactions. If problems develop, the child must be taken to the pediatrician immediately. Therapeutic treatment is often necessary for chronic conditions. In the long term, hydrophthalmia not only leads to blindness of the affected eye, but also causes mental discomfort. To avoid serious complaints such as depression or inferiority complexes, the child should talk to a psychologist. Attending a support group may also be useful in some circumstances.
Treatment and therapy
For therapy, some conservative drug treatments that are applied locally are available for patients with hydrophthalmos. However, such therapies often remain ineffective and are inferior to surgical interventions. In invasive treatment procedures such as trabeculotomy or goniotomy, the greatest success can be achieved in hydrophthalmos. Surgical therapy is indicated when intraocular pressure exceeds 19mmHg. In addition, intervention is indicated for corneal diameters greater than twelve millimeters within the first year of life. There is also an indication for surgery with increasing pupil excavation, increasing corneal diameter or axial length increase of the bulb. The time of diagnosis and the regularity of control are all-important for the success of therapy. Purely medicinal therapies are sometimes suggested, but more often result in blindness of the affected eye than the surgical approach. The procedure requires hospitalization of the patient. If both eyes are affected, surgery often takes place on only one eye initially and must be repeated on the other eye after a period of time.
Outlook and prognosis
Without medical care and surgical intervention, the prognosis of hydrophthalmos is unfavorable. Improvement of symptoms is not expected in these cases. As the condition progresses, most patients complain of a successive increase in existing symptoms or the onset of additional sequelae. In severe cases, the patient gradually goes blind. This condition is experienced as highly stressful and can trigger psychological sequelae in particular. If a treatment plan is drawn up and implemented, there is a better prognosis. The prospect of alleviating the symptoms depends on when the therapy is started. The sooner surgery or drug treatment can begin, the better the prognosis.If no complications occur, the patient’s vision can be almost completely regenerated with early treatment. In these cases, the patient is discharged from treatment as recovered. Despite the favorable prospect of freedom from symptoms, regular follow-up visits to a doctor are necessary. The internal pressure of the eye as well as the general vision must be controlled and observed in the long term. The likelihood of a relapse and thus a new development of the underlying disease is possible at any time, even if freedom from symptoms has been achieved, and must therefore be monitored continuously.
Prevention
Hydrophthalmos can be prevented to some extent by rubella vaccination. With rubella vaccination, women are safe from disease during pregnancy so that rubella-induced maldevelopment of their fetus does not occur. However, since hydrophthalmos can also be due to genetic factors, this does not completely exclude the possibility of the child becoming ill.
Follow-up
In the case of hydrophthalmos, affected individuals are primarily dependent on a rapid diagnosis and subsequent treatment to prevent further complications and discomfort. This is also the only way to prevent further worsening of the symptoms. Self-healing is not possible with this disease, so that those affected are always dependent on treatment. The measures of an aftercare are thereby strongly limited, so that in the foreground with this illness an early recognition and diagnosis of the Hydrophthalmus stands. In most cases, hydrophthalmos is treated by surgical intervention. This usually leads to success and is also without complications. Affected patients should take care of themselves and rest their bodies after the operation. Efforts or stressful physical activities should be avoided in any case, in order not to put unnecessary strain on the body. In most cases, further examinations of the eyes are necessary even after a successful procedure. The procedure must then also be performed on the second eye. As a rule, this disease does not reduce the life expectancy of the affected person. Since hydrophthalmos can also cause depression or psychological upset, psychological treatment should also be performed in this case.
What you can do yourself
Hydrophthalmos always requires treatment by a physician. Options for self-help are not available to the affected person for this reason. However, the symptoms can usually be alleviated by surgical intervention. Possibly, the disease can be prevented by a rubella vaccination, although the effect of this vaccination on the disease has not yet been fully researched. If the disease occurs due to genetic determinations, it cannot be prevented. The rubella vaccination should be administered directly during pregnancy. Thus, an infection of the unborn child can be avoided. Those affected must take care of their bodies after the surgical procedure. High hygiene must also be observed to avoid further inflammations or infections. If the hydrophthalmos also affects the second eye, the procedure usually has to be repeated on the other eye as well. Regular check-ups by a doctor are also necessary after the treatment to prevent complications. Especially in children, the controls should be observed. If patients suffer from visual complaints, these are compensated for with the help of visual aids. The visual aids should be worn at all times, as vision may be reduced even further.
