| Newsletter Volume 1 |
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Introducing the Pediatric Keratoplasty Association Welcome to the first issue Of tile Pediatric Keratoplasty Association Newsletter. The rarity of neonatal and pediatric corneal disorders and tile difficulties involved III examining and treating very young patients prevent most ophthalmologists pediatric ophthalmologists and corneal surgeons from developing consistent clinical practice patterns. The goal of the Pediatric Keratoplasty Association (PKA) is to provide pediatric ophthalmologists, corneal surgeons and others with a method for diagnosing and managing pediatric keratoplasty cases through the exchange of ideas and information, Its referral network will enable ophthalmologists to seek advice from their peers oil the technical difficulties and challenges of individual cases. The presence of such all association will facilitate data collection oil the success rate associated with various types Of Surgical procedures. In addition. the PKA newsletter will address the many non-ophthalmic issues (i.e. family counseling genetic analysis, etc.) that arise in the care of these children Patients' families will be able to use the Association as a referral Source ill emergency situations such as when traveling; if they are planning to relocate; or if. for any reason, tile, are in need of another ophthalmologist Tile improvement in technical ability that will surely result from tile opportunity to share information oil a routine basis can only enhance and facilitate patient Care. Please join with us in networking our knowledge and experience so that we may all better serve our patients and their families. Gerald Zaidman. MD Guest Surgeon Interviews A New Paradigm for the Diagnosis of
Congenital Corneal Opacities For the first issue of the Pediatric Keratoplasty Newsletter, I thought it would be helpful to present my approach to the diagnosis of congenital corneal opacities. Hone reviews the literature, one will find several paradigms to assist in this diagnostic problem, however, many of these paradigms are too dependent oil rotea I like memorization or acronyms. They are not based on any consistently logical clinical approach. How does one approach the infant born with a congenital opacity? First, how do we best examine tile infant? If an adult presented with an eye with a dense corneal leukoma in which a cataract might be present, and it was impossible to see the posterior pole, a slit lamp examination, measurement of the intraocular pressure, and A and B ultrasounds would be indicated. To do anything less for an infant because of the patient's small size and poor cooperation would be unacceptable, one should never use shortcuts when performing the infant eye exam. In fact, precisely because there is usually poolcooperation, it is imperative to get as much information as possible; for this reason, all infants with congenital corneal opacities should have an exam under anesthesia - there is no way to adequately ass ess an infant or a young child while he or she is moving around in their parent's arms. Also, it is imperative to rule out glaucoma, and so one must accurately measure the intraocular pressure. With Current anesthetic techniques, the risks of sedating, intubating, and anesthetizing a very small infant are minimal. Moreover, the benefits of a correct ocular diagnosis far outweigh the risks of anesthesia. Therefore, whenever I see a child with a congenital corneal opacity, I do an off-ice exam - but inform the parents that an exam under anesthesia is required. I schedule the EUA as the first case of my surgical day, and I leave a 30-40 Minute break between tile EUA and my second case to allow time to discuss tile findings with tile parents. The goals of the office exam are to obtain an adequate pediatric and obstetric history; to determine if the child is able to fix or follow a light in either eye; and to rule out any acute infectious/inflammatory process that requires urgent medical attention. A comprehensive exam is left for the operating room In the operating room. in addition to a routine examination of the eyelids and ocular adnexa rnicroscopic examination of the cornea and anterior chamber is performed. The corneal diameter is measured and the intraocular pressure checked \% ith a pneumotonometer (a hand-held portable applanation tonometer is also acceptable). A-scan ultrasonic biometry Is then performed. Direct and indirect sibility. If the cornea is smaller than normal, then tile eye may be microphthalmic This is important because microphthalmic eyes frequently have other ocular abnormalities. In cases of suspected microphthalmos, both A- and B-scan ultrasound examinations are helpful. The vast majority of congenital conical opacities occur in quiet normal sized eyes. The next step, therefore, is to determine the location of the opacity. Opacities can be peripheral, central, or diffuse. Isolated opacities involving the peripheral cornea are uncommon I hey include peripheral corneal dermoid; per ipheral Reiger's anomaly, and peripheral conical ulcers. Most corneal opacities involve either the central cornea or are diffuse. These cases are the most important because they are III the visual axis. Lesions involving tile central cornea, and sparing the peripheral corilea, include Peter's anomaly, corneal dermoid, some corneal infections (secondary to rubella. syphilis, gonorrhea, and Herpes simplex), and sclerocornea. Neurotrophic ulcers due to the Riley-Day syndrome or congenital alacrima can also be seen. Sclerocornea is tile most common cause of a diffuse corneal opacity In an otherwise normal shaped eye, Other Causes of diffuse corneal opacities in tile normal shaped eye include congenital glaucoma birth traurna, congenital corneal dystrophy (Such as congenital hereditary stroma or congenital hereditary endothelial dystrophy). posterior polymorphus dystrophy, and the systemic metabolic disorders that account for congenital Corneal opacities. To separate these various entities, it will help to determine whether the disorders are unilateral or bilateral and whether the disorder tends to change with time. Birth trauma Herpes keratitis, neurotrophic ulcers, and dermoids are usually unilateral. Glaucoma, rubella, metabolic disorders, and hereditary corneal dystrophies are usually bilateral. Sclerocornea, Peter's anomaly and other forms of anterior mesodermal dysgenesis (Rieger, Axenfeld, etc.) can be unilateral or bilateral. As noted above. metabolic disorders are usually not present at birth and worsen with time. Birth trauma Will have I tendency to Improve with time. In birth trauma, one can Usually find evidence idence of forceps marks oil tile forehead and skull Of tile infant Finally, it has been reported that in some patients sclerocornea can also clear with time. HOW DO YOU HANDLE IT? Guest writers are invited to submit to the editor newsletter articles focusing oil corneal diseases in children under eight years. Any corneal disorder that could affect vision, function, or cosmesis will be considered: these disorders might be inflammatory, infectious, congenital, metabolic, traumatic, or genetic in nature. Specific topics might include: preoperative workup; intraoperative surgical approach; post-operative care; type of anesthesia used; frequency of examination under anesthesia; suture type and style; postoperative drug use; treatment of amblyopia, glaucoma; and strabismus; and graft rejection. Non-ophthalmic factors will also be considered such as the risks of anesthesia; family counseling; genetic analysis; and management of other systemic abnormalities. We would like to know how you handled it. Mail your manuscripts to: Gerald W. Zaidman, MD How I do It - Post Operative Course This brief report will summarize my post-operative regimen in children with corneal transplants. This approach is influenced by two parameters - the age of the patient and the diagnostic reason for the corneal transplant. In general most children over 8 years old (and many over 5-6 years of age) can be examined at the slit lamp. In many aspects of their care they can be treated and examined like young adults. Usually one can check the intraocular pressure (especially with the pneumotonometer), remove sutures for astigmatism (though this may occasionally require sedation) and check for rejection or * other complications. They and their parents can be trained and educated as to how to recognize the early signs of graft rejection and many children, even at this young age, are good reporters of sudden changes in visual acuity. Like Many adults, graft prognosis depends on the underlying diagnosis. In my experience the most common causes of corneal transplantation between the ages of 8-17 are scarring secondary to trauma or infections (especially HSV), and keratoconus. Since the corneas in the first two diagnoses are often vascularized and irregular their prognosis is not as good as keratoconus patients. In general however I manage these patients with a therapeutic regimen very similar to adults. It is in the group Of children less than 6-8 years of age or those children with emotional or psychological problems (in whom cooperation is poor) that the post operative course is most difficult. Prior to surgery on any child in this age group I tell the parents that I expect their total and absolute cooperation. They must understand that surgery on children in this age group is like running a marathon. Because of the child's age and general lack of cooperation very frequent office exams and exams under anesthesia are necessary (sometimes at very short notice). The parents must be willing to deal with the psychological ups and downs and stresses associated with this post operative course. Other than the child's age the post operative regimen is strongly influenced by the underlying diagnosis. Essentially these patients can be divided into two groups - children with congenital disease (anterior segment dysgenesis, Peter's anomaly, congenital glaucoma, metabolic or chromosomal disorders, etc.) or children with acquired diseases (primarily trauma and infections). The first group more likely to have had severe ocular pathology requiring more extensive anterior segment surgery (glaucoma, cataract surgery, vitreous surgery, etc.) than the latter. Therefore the post operative course is more complicated, requiring more attention to intraocular pressure control, complications of retinal or vitreous surgery, etc. Despite their differences certain similar principles are followed for both groups. Both groups are similar in terms of very rapid wound healing and high rejection rates. Therefore daily eye exams are performed for the first 3 weeks. Patients at high risk for glaucoma, retinal or vitreous pathology have weekly exams under anesthesia. This is also done to evaluate the status of the corneal sutures. If there is any sign of suture loosening the sutures are removed. After 3 weeks and after the third EUA the sutures are usually out in the younger children. They are then examined twice a week, weekly and then every 2- weeks as the months pass. Chloral hydrate is frequently used in the office to sedate the patient in order to check the intraocular pressure. In the older children (or those with a less complicated course) after the first few weeks, office exams are done slightly less often (initially weekly and then no less often than every 2-3 weeks). This continues until all sutures are removed - : (usually within 3-4 months). Again exams under anesthesia in the O.R. or chloral hydrate in the office are used as necessary to follow the clinical course in these patients. Finally during the post-operative period I have the parents obtain a penlight and I train them to examine the eye. Motivated parents can be taught to recognize changes in corneal clarity, collection of mucus around potentially loose sutures and increased conjunctival discharge or redness. I tell the parents that other signs of ocular pathology include changes in the child's normal attitude or temperament, rubbing of the eyes or decreased ability to follow a bright, silent, object. I expect the parents to immediately call my office if there is a problem. The last Newsletter included survey forms regarding the pre- and post-operative management of infants and children with corneal opacities. Certain clinical practice patterns were observed in the responses. Regarding the initial pre-operative evaluation, all of the respondents would attempt an office visit consisting of a penlight exam, slit lamp exam, tonometry and funduscopy. Two-thirds of the respondents would then perform ultrasound. About 75% of the respondents routinely perform an exam under anesthesia after the office exam, some as soon as possible. The other 25% generally do not perform an EUA. Twenty percent of the respondents simultaneously have a pediatric ophthalmologist check the patient. Most respondents were relatively aggressive regarding the timing of surgery. Nearly 70% would perform a penetrating keratoplasty on unilateral opacities soon after the diagnosis was made. Nearly 90% would do urgent surgery on bilateral cases. The 10% that wouldn't perform rapid surgery on bilateral cases felt that surgery would be easier on slightly older children. They also felt that since unilateral amblyopia is less of a concern in bilateral cases, surgery could be delayed. In general, the post-operative regimen consisted of many months (6-12) of topical corticosteroids, supplemented by occasional periocular steroids. The preferred treatment for glaucoma consisted of topical agents only, usually a beta blocker. A significant minority used oral agents (DIAMOX®) only. The intraocular pressure is usually checked in the office without sedation using either a Perkins tonometer, tonopen, or pneumotonometer. (The next Newsletter will concentrate on this topic.) Visits are usually daily for the first 7-21 days. This frequency is extremely dependent on the presence or absence of sutures. In general, patients are examined very often until all sutures are out. The frequency of visits is then decreased. Once sutures are out, visits usually become more routine, occurring once every week or two and quickly being decreased to monthly, etc. Suture removal is relatively independent of the diagnosis but is relatively dependent on the age of the recipient. In infants (patients less than one year of age), about halt of the respondents removed sutures in less than one month, while the other halt removed them in 1-3 months. In older children (up to six years of age), all sutures were usually out 3-6 months after surgery (half the respondents removing them closer to three months and the other half closer to five months). Children over the age of 6-8 have sutures removed similar to adults. To summarize, the usual approach to a pre-verbal child with corneal opacities is an office visit followed by an EUA generally leading to rather rapid surgery. Afterwards, close monitoring of the child with early suture removal is performed. I would like to thank the members of the PKA for their excellent responses.r Despite their differences certain similar principles are followed for both groups. Both groups are similar in terms of very rapid wound heating and high rejection rates. Therefore daily eye exams are performed for the first 3 weeks. Patients at high risk for glaucoma, retinal or vitreous pathology have weekly exams under anesthesia. This is also done to evaluate the status of the corneal sutures. If there is any sign of suture loosening the sutures are removed. After 3 weeks and after the third EUA the sutures are usually out in the younger children. They are then examined twice a week, weekly and then every 2- weeks as the months pass. ..Chloral hydrate. is frequently used in the office. to sedate the patient in order to check the intraocular pressure. In the older children (or those with a less complicated course) after the first few weeks, office exams are done slightly less often (initially weekly and then no_less often than every 2-3 weeks). This continues until all sutures are removed (usually within 3-4 months). Again exams under anesthesia in the O.R. or chloral hydrate in the office are used as necessary to follow the clinical course-in these patients. Finally-during the post.-operative period I have the parents obtain a penlight.. and I train them to examine the eye. Motivated parents can be taught to recognize: changes in corneal clarity, collection of mucus around potentially. loose sutures and increased conjunctival discharge or redness. I tell the parents that other signs of ocular pathology include changes in the child's normal attitude or temperament, rubbing of the eyes or decreased ability to follow a bright, silent, object. I expect the parents to immediately call my office if there is a problem. SPA-4867A |
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