The Pediatric Glaucomas

113 Pages·2005·10.6 MB·English

by  Anil K. Mandal, Peter A. Netland

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© 2006, Elsevier Inc. All rights reserved.
First published 2006
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Foreword
When I first joined Robert Shaffer, MD and John difficult decision of whether to keep trying. Is the pain and
Hetherington, MD in practice over 30 years ago, Dr Shaffer risk of another operation worth it? A psychologist told me
asked me if I would be interested in focusing on children with many years ago that a child who retains vision till the age
glaucoma. He had worked with Dr Barkan and had already of six or beyond will have visual memories that improve his
developed a large practice in childhood glaucoma, especially later functioning. These points are nicely made in the con-
the developmental form. That started a long-term interest clusion to Chapter 10. Fortunately, these decisions are less
in this fascinating, rewarding and sometimes discouraging common with the advent of antifibrosis agents and drainage
disease. At one point we were seeing as many as one new case implants. At some point however, it may be best to quit. The
a week, which kept us all quite busy. I still see occasional parents will have to be led to this most difficult decision by
patients on whom I operated 30 years ago who are seeing well the physician.
and doing fine. That is very gratifying. Finally, experience counts. It is often the first operation
As most young physicians, I started out focused on that determines the outcome in these children and whenever
managing the disease but it soon became obvious that possible it should be done by an experienced surgeon or team.
managing the family was equally important. Parents of Since these tend to be rare cases and patients cannot always
children with developmental glaucoma are often filled with travel, that will not always be possible. This book will help
guilt, believing that something they did or did not do caused physicians manage the process of doing the right thing for the
this terrible affliction that could blind their child. Solving right diagnosis at the right time.
the problem is, of course, the best solution, but helping the I offer my congratulations and thanks to Drs Mandal and
parents understand that they are blameless and that the most Netland for providing all this information in a wonderfully
important thing they can do for their child is to give them organized and illustrated text that clarifies the diagnosis and
lots of love is critical. Some of these patients will inevitably treatment of the many forms of childhood glaucoma. I wish
end up blind and they will function much better in the world it had been available 35 years ago.
if they grow up in a loving environment.
Occasionally, after multiple surgeries and continued visual
deterioration, the doctor and the parents are faced with the H. Dunbar Hoskins, Jr MD
vii
Preface
The idea for this book originated from patient care. The effort past, but are sufficiently outdated to create a need for this
to manage the clinical problems in children with glaucoma book. Significant advances have occurred in genetics, medical
revealed the need for up-to-date and organized information therapy, surgical management, and other topics included here.
about the topic. Over the years, we have met and discussed This book is intended for clinicians who care for pediatric
these problems at length. Our previous writings provided glaucoma patients, including, in particular, glaucoma and
the backbone for this work. We organized our material for pediatric subspecialists. We hope that other practitioners who
an Instruction Course at the American Academy of have contact with pediatric glaucoma patients will find value
Ophthalmology, which crystallized our thoughts about the in it, and that ophthalmology residents and subspecialty
topic. trainees will benefit from this information.
We have attempted to present evidence-based information
about the topic, while providing perspectives from clinical Anil K. Mandal, MD
experiences. Other excellent textbooks have appeared in the Peter A. Netland, MD, PhD
Drs Mandal and Netland perform Koeppe gonioscopy during an examination under sedation.
ix
Acknowledgments
We are deeply indebted to our patients, as well as our patients’ parents and families. Caring for
these patients has been a group effort, and we appreciate all of the individuals on the ‘team.’
We are also grateful to our families, friends, mentors, and colleagues who provided support and
guidance. Medical Publisher Karen Oberheim provided critical early support for the project,
as did Senior Editor Natasha Andjelkovic and Assistant Editor Andrea P. Sherman. Joseph
Mastellone and Stephen Moser assisted with photography. Jerry Harris at St. Jude Children’s
Research Hospital provided assistance with graphic arts. We are especially thankful for the
expert assistance of Mary E. Smith, Vijaya K. Gothwal, Anita Fernandez and Joyce Solomon.
We thank Richard D. and Gail S. Siegal for their support. We would like to thank the copyeditor,
Alison Woodhouse, the proofreader, J. Ian Ross, the indexer, Liza Furnival, and the illustrator
Richard Tibbitts. Elsevier provided excellent publishing support through the efforts of Senior
Editor Paul Fam, Project Development Manager Amy Head, Project Manager Kathryn Mason,
Designer Andy Chapman, Illustration Manager Mick Ruddy and Product Managers Lisa
Damico and Gaynor Jones.
xi
To
my loving parents, Jayalaxmi and Manik, who instilled in me the desire to learn
and the enjoyment of teaching
and
my wife, Vijaya, for her constant help and encouragement in this endeavour.
Anil K. Mandal
xiii
To
my patients and their families,
my colleagues and trainees,
and
my supportive family and friends.
Peter A. Netland
xv
Light, my light, the world-filling light, the eye-kissing light, heart-sweetening
light!
Ah, the light dances at the center of my life... The butterflies spread their sails on
the sea of light. Lilies and jasmines surge up on the crest of the waves of light.
The light is shattered into gold on every cloud and it scatters gems in profusion.
Mirth spreads from leaf to leaf and gladness without measure.
The heaven’s river has drowned its banks and the flood of joy is abroad.
From Gitanjali, Number 57
Rabindranath Tagore, Nobel Laureate 1913
xvii
Chapter 1
Historical perspective of developmental
glaucomas
The poor prognosis of infantile glaucoma changed dramati-
Introduction
cally in 19382 with the introduction of goniotomy (Greek:
Goniotomy gonio = angle + tomein = to cut) by Otto Barkan (Fig.1.1)
Description of the clinical entity who revived the Italian surgeon de Vincentis’ operation
Microsurgery and trabeculotomy (1892), which ‘incised the angle of the iris in glaucoma.’ Otto
Barkan modified de Vincentis’ operation by using a specially
designed glass contact lens to visualize angle structures while
Introduction
using a knife to create an internal cleft in the trabecular
Congenital enlargement of the eye has been recognized since tissue.3 He called the operation goniotomy and reported
the time of Hippocrates (460–377BC), Celsus (1st century AD), several successfully treated cases in congenital glaucoma.4,5
and Galen (130–201AD), although buphthalmos or hydro- Although instrumentation has since been refined and the
phthalmos were not related to elevated intraocular pressure operating microscope now permits more precise visualization
until the middle of the 18th century. Increased intraocular of the angle structures, the operation has remained
pressure was mentioned by Berger (1744), but was grouped essentially unchanged.
together with a variety of heterogenous conditions varying In 1949, Barkan described a persisting fetal membrane
from high myopia to anterior staphyloma and anterior mega- overlying the trabecular meshwork.5 This was confirmed by
ophthalmos. In 1869, Von Muralt (1869) established the Worst (1966) who termed it ‘Barkan’s membrane.’6 Recent
classical type of buphthalmos within the family of glaucoma. pathological studies by Anderson,7–9 Hansson,10 Maul and
Both he and Von Graefe (1869) considered that the enlarge- co-workers,11 and Maumenee12 could find no evidence of a
ment of the cornea was the primary phenomenon, but believed membrane in any of the specimens they examined by light
that the clinical picture with its rise of tension was due to a or electron microscopy. Despite this evidence, Worst stated
primary intraocular inflammation. that ‘though histopathological proof of this structure is
Pathological studies of the late 1800s and early 1900s almost completely lacking ... this has little influence on the
had detected congenital anomalies in the anterior chamber probability that this concept is valid.’13
angle or the absence of Schlemm’s canal. These anomalies
were confirmed by Von Hippel (1897), Parsons (1904), and
Siegrist (1905). Exhaustive anatomical descriptions appeared
in the early to middle 1900s by Gros (1897), Reis (1905–11),
Seefelder (1906–1920), and others who demonstrated a
number of different malformations of the angle structures
as the primary abnormality, with inflammation playing a
secondary role.
Goniotomy
As late as 1939, Anderson1saw little hope for preservation of
useful vision in these patients. Despite a detailed evaluation
of all known treatment modalities available at that time,
he stated that ‘one seeks in vain for a best operation in the
treatment of hydrophthalmia.’ He further wrote:
The future of patients with hydrophthalmia is dark.
Little hope of preserving sufficient sight to permit the
earning of a livelihood can be held out to them. It
progresses, as a rule, in a relentless fashion until the best
setting for the patient is some institution that caters for
Figure 1.1 Otto Barkan (1887–1958). Reprinted with permission from
the blind. Cordes FC, Otto Barkan, MD. Trans Am Ophthalmol Soc 1958; 56:3–4.
1
Historical perspective of developmental glaucomas
Description of the clinical entity
trabeculotomy ab externo.22At about the same time (1960) in
A few classic textbooks that have been written on the subject London, Redmond Smith, an early microsurgeon, developed
include Hydrophthalmia or Congenital Glaucoma(Anderson, an operation that he called ‘nylon filament trabeculotomy.’23
1939),1 Congenital and Pediatric Glaucomas (Shaffer and This involved cannulating Schlemm’s canal with a nylon
Weiss, 1970),14 and Glaucoma in Infants and Children suture at one site, threading the suture circumferentially,
(Kwitko, 1973).15 withdrawing it at another site, and pulling it tight like a bow-
Sir Stewart Duke-Elder (1963) wrote: string. The surgical technique of trabeculotomy ab externo
is basically a combination of that originally evoked by Burian
Buphthalmos (hydrophthalmos) is the condition wherein
and Smith and modified by Harms (1969),24,25 Dannheim
developmental abnormalities offer an obstruction to the
(1971)26,27and McPherson (1973).28–30
drainage of the intra-ocular fluids so that the pressure of
Following World War II, the Zeiss Optical Instrument
the eye is raised and a condition of congenital glaucoma
Company relocated to southern Germany near the ancient
results. The essential clinical feature of the anomaly is
university town of Tubingen. Seeking to develop new markets
that the coats of the eye are of sufficient plasticity to
and products, Zeiss approached Harms, who told him ideas
stretch under this increment of pressure with the results
for an ophthalmic operating microscope. A prototype was
that the whole globe enlarges, producing an appearance
produced, and the era of ophthalmic microsurgery began.
which is said to resemble the eye of an ox.16
In 1966 Harms organized the First International
Primary congenital glaucoma was described by Shaffer and Symposium of the Microsurgery Study Group in Tubingen.
Weiss (1970) as a specific syndrome as follows: Among the ophthalmologists in attendance was Samuel D.
McPherson, Jr., of Durham, NC. Impressed by the excellent
The most common hereditary glaucoma of childhood,
results being claimed for external trabeculotomy, McPherson
inherited as an autosomal recessive pattern, with a
remained after the symposium to observe Harms in surgery
specific angle anomaly consisting of absence of angle
and learn the procedure. McPherson then became the
recess with iris insertion directly into the trabecular
ophthalmologist most associated with the procedure in
surface. There are no other major abnormalities of
the United States and its most prolific proponent in the
development. Corneal enlargement, clouding, and tears
American ophthalmic literature.28–31
in Descemet’s membrane result from elevated intraocular
Throughout the 1960s, the popularity of external trabec-
pressure.14
ulotomy grew in Europe. By the Second International
Now it is firmly established that developmental glaucoma Symposium of the Microsurgery Study Group in Burgenstock
has as its hallmark fetal maldevelopment of the iridocorneal in 1968, the procedure was widely used throughout Europe.
angle or goniodysgenesis.17 The anomalies of the angle When Harms and Allen eventually met, Allen was the first
include trabeculodysgenesis, iridodysgenesis, and corneo- to tell Harms of the Iowa City work. Although astonished,
dysgenesis, either singly or in some combination. The classic Harms thereafter gave Burian and Allen credit for the first
defect found in primary congenital glaucoma is isolated description of the procedure.
trabeculodysgenesis without any evidence of other iris or The introduction of the microsurgical techniques as ex-
corneal malformation. emplified by trabeculotomy revolutionized the prognosis for
Initial efforts at classification were directed toward eponyms patients with primary congenital glaucoma, with most studies
and syndrome names, and many of these terms are now citing an initial success rate of 80–90%.24,28–37Trabeculotomy
widely employed and recognized. The Shaffer–Weiss (1970) ab externo38–39and goniotomy40remain as the preferred initial
disease classification divides the developmental glaucomas procedure in the surgical management of primary infantile
into primary congenital glaucoma, glaucomas associated with glaucoma.
developmental anomalies of the eye or the body, and acquired The need for ‘glaucoma enucleations’ has markedly de-
glaucomas.14,18 Recently, an excellent classification system creased over the last 50 years, with enucleation for open-angle
has been described by Hoskins, Shaffer, and Hetherington glaucoma (including congenital glaucoma) now almost fallen
(1984), which uses clinically identifiable anatomical defects into oblivion.41 During the last 50 years, ophthalmological
of the eye as the basis of classification.19,20 care has improved, various pressure-lowering and anti-
inflammatory drugs have been developed, new surgical
techniques have been introduced, and, probably most impor-
Microsurgery and trabeculotomy
tantly, the operating microscope has been incorporated into
The classic operation for the treatment of primary congenital clinical practice. These advances have enhanced the efficacy
glaucoma was Barkan’s goniotomy,2 although there has been of treatment while minimizing complications, which has
increasing use of a newer approach, trabeculotomy ab externo. improved greatly the prognosis for congenital glaucoma.
This procedure was simultaneously and independently
described by Burian21,22and Smith23in 1960.
In March, 1960, without the aid of an operating microscope, References
the first external trabeculotomy was performed by Burian
1. Anderson JR. Hydrophthalmia or congenital glaucoma: its causes, treatment,
on a young girl with Marfan’s syndrome and glaucoma.21
and outlook. Cambridge University Press: London; 1939.
After 2 years, Allen and Burian published another paper on 2. Barkan O. Technique of goniotomy. Arch Ophthalmol 1938; 19:217–221.
2