CAPILLARY
LYMPHANGIOMA
OF
THE
TONGUE
COMPLICATED
BY GLOSSITIS
C. Everett Koop, M.D., Sc.D. (Med.), and Elias A. Moschakis, M.D.
Surgical Clinic, the Children’s Hospital of Philadelphia, and the Harrison Department of
Surgical Research, School of Medicine, University of Pennsylvania
Presented at the Annual Meeting of the American Academy of Pediatrics, October 16, 1960.
ADDRESS: (C.K.) Children’s Hospital, 18th and Bainbridge Streets, Philadelphia 46, Pennsylvania.
Psnwrmcs, May 1961
800
O
NE OF the most perplexing butfortu-nately rare intra-oral lesions that we
have encountered in children is that of
lingual capillary lymphangioma complicated
by glossitis. The eight cases here reported
and seen in a 13-year period of observation
have in each instance been associated witil
a major lymphangioma. One of these was
confined to the tongue, two were subglottic
and the remainder were large cystic
hy-gromas of the neck and face. During this
same period, we have seen two additional
lymphangiomas of tile tongue unassociated
with the eruptive phenomena on the
sur-face of the tongue.
Medical literature contains numerous
ref-erenees to hereditary or familial
hemor-rhagic telangiectasia, with an excellent
re-view and bibliography by Hodgson et al.1
However, little can be found concerning the
lesion here described which has not had a
familial aspect in a single instance.
Several authors,25 in writing about cystic
hygromas of the neck, have alluded to the
concomitant finding of macroglossia or
lymphangiomatous involvement of tile floor
of the mouth. Lierle3 described three cases
of macroglossia associated with cystic
hy-groma and noted superficial ulceration of
the mucous membrane as well as deformity
of the mandible. Fuller et al. found
macro-glossia to be an infrequent finding with
cystic hygroma of the neck, but one of their
illustrative photographs showed a mild form
of the dynamic pathologic process to be
de-scribed ilere. Perzik reported a single case
of macroglossia and described the tongue
as being superficially ulcerated and
crust-covered and continuously exuding a
sero-sanguinous discharge. He suggested that
excision of tile sublingual extension of tile
cervical hygroma causes regression of the
macroglossia. Our experience would
mdi-eate that although removal of sublingual
ex-tensions of the cervical lymphangioma
might reduce the intra-ora! contents, the
macroglossia per se is not altered.
Goetsch,#{176} in his classic paper on cystic
hygromas, considered them to be benign
true neoplastic tumors of lymphatic origin,
excision of which is usually followed by
cure. Harkins and Sabiston, in discussing
the pathology of lymphangiomas, stated that
the cavernous lymphangiomas occur in the
tongue among several sites. They are
de-scribed as diffuse, spongy, compressible
masses, the microscopic appearance of
which consists chiefly of multiple dilated
lymph channels with abundant or scanty
fibrous stroma. The lesions described here,
although similar in histologic appearance to
photomicrographs of Harkins and Sabiston
had lymphatic channels capillary in size,
and the tongues were certainly neither
spongy nor compressible.
CLINICAL CHARACTERISTICS
The clinical history begins with the
spon-taneous eruption of a variable number of
clear, fluid-filled vesicles on tile dorsum of
the tongue, which apparently are not painful
(Fig. 1). These disappear in several days, to
be replaced by others. Indeed, tile process
is quite dynamic, with the surface of the
tongue changing rapidly. As the vesicles
spontaneously disappear, a relatively small
number of them leave a residual
papilloma-tous lesion that in some early instances
re-sembles a papule of granulation tissue and
801
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Fic. 2. (BA.) Late appearance of tongue after vesicles. Note hypertrophic villi, hemorrhagic
carious teeth.
recurrent eruptions of lymphangiectasia and
Tile former bleed easily and are painful to
touch, while the latter seem to have lost their propensity for bleeding but are
never-theless 1)ainful.
The latter two lesions tend to remain,
once they have appeared, although they,
too, seem capable on occasion of sloughing
at the base to leave a normal appearing
tongue surface behind. Indeed, some
tongues subject to this phenomenon look
almost normal for days or even weeks, only to have tile lesions reappear in tile same
order but not necessarily with the same
speed or in tile same quantity.
At times there is obvious edema and
pro-trusion of the tongue (which was thought
at birth to he too large in five of the eight
patients ill the present series). After
re-peated episodes of swelling, the tongue fails
to regaul its former size and seems
perma-nently hypertrophied. Acute glossitis with
swelling, excessive salivation, increased
Paffi, fever, and even respiratory distress due to obstruction, may be initiated by
respiratory infection or ingestion of certain
foods, or it may be idiopathic (Fig. 3). In
tile small infant, nursing becomes
impos-sible, fluid intake is difficult and parentera!
fluid therapy must follow. In older children,
Fic. 1. (C.O.) Fluid filled vesicles erupting on surface of tongue. Note smaller hemorrhagic
papillae in lower left corner.
eating is at first difficult, then too painful to
be attempted. Speech, already suffering
from some impediment under the best of
conditions due to the macroglossia, becomes
more difficult because of pain and swelling.
Sleep is possible only with the tongue
ex-truded from the mouth, in which position
4
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Fic. 3 (K.O.) Edematous painful tongue with confluent lesions.
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extension of tile process just described (Fig.
4).
All patients are not affected in the same
degree by this eruptive phenomenon. Oddly
enough, the three patients of this group
re-quiring least attention to the
!ymphangiec-tasias on the surface of the tongue and now
asymptomatic were those in whom the
ma-jor lymphangioma was confined to the
Fic. 4. (B.A.) Subsiding acute exacerbation 1 year after partial glossectomy. This tongue fits well into mouth, when free of glossitis. Note
prominence of mandible.
tongue or subglottic area without extension
into the submaxillary region or other
tn-angles of the neck. Of the remaining five,
three are yet problems in management.
None has reached puberty.
The major visible tumor masses were all
seen at or shortly after birth, while in only
half the patients were the vesieles over the
dorsum of the tongue prominent enough to
be recorded at the same time. Clinical
prob-lems with the lymphangiectasias arose in
the newborn period in only a single infant.
In the remaining patients there was a
pe-nod of 4% to 33 months between our first
en-counter with the major lymphangioma and
the appearance of tile clinical difficulties
due to the ectasias on tue tongue.
TREATM ENT
Treatment in all patients was directed
first toward excision of the major lesion,
whether cervical or intra-oral. Tile single
patient (L.L.) requiring attention to the
sun-face lesions on the tongue may have gone
for some time without a major eruptive
problem had his tongue not been burned by
prior treatment.
Treatment of the tongue itself consisted
of two parts, once the major lymphangioma
was cared for: electrodesiccation of the
1 (GO.) (lays 9 mo Neck (left) yr 6 no No No No
(CA.) Syr 6 mo 6 yr S mo SuLgiottic 7 yr 4 mo No No No
S ((.O.) 6 yr S yr 3 mo Tongue .5 yr 1 no Only with lymphangioma No No
4 (LI,.) wk wk Neck (bilateral), submaxillary I I yr 7 mo Yes Yes Ye
5 (MA.) g uk 1 yr .5 mo Neck (bilateral), suhglottic S yr 6 mo Yes Yes Yes
S (K.1).) 7 mo 1 yr Neck (bilateral), suhglottie I I yr S mo Ye Ye, Yes
7 (KR.) 2 yr 6 no S yr 6 mo Neck (right), face, supraclavicular fossa Syr Yes Yes Yes
8 (BA.) 6wk 6 mo Bilateral, stibmandihular 4 yr Yes Yes Yes
TABLE I
HISTORY OF EIGHT CHILDREN WITh SYNDROME OF LINGUAL CAPILLARY
LYMPH-A NGIOMLYMPH-A COMPLICATED BY GIOSSITIS
Surface Fir.t See,, Leajon., a (a.c (age) P,oblent
(age)
Site of Major Lymphangioma Follow-up (age)
Partial Symplo-Macroglossia Glossec- malic lomy Lale
form, and amputation of a portion of the
tongue in some patients in order to reduce
it to intra-oral size (Table I). No
postopera-tive problems followed partial glosseetomy
but there was variable edema, infection,
pain and inability to eat following
electro-desiccation. Discussion of this aspect of care
would be more profitable after presentation
of the ease material.
Case 1, G.O.
CASE REPORTS
A 2-day-old white male was first seen
with a cystic hygroma involving the entire
left part of the neck from mandible to
clavi-ele and from the angle of the mouth to the
external auditory canal. Within the large
mass, smaller nodular masses were palpable.
On December 31, 1957, the cystic hygroma
was removed.
The child’s second admission in
Septem-ben, 1958, was necessary because of
multi-pie lesions on the surface of the tongue and
an inguinal hernia. The hernia was repaired,
and many of the larger lymphangiectasias
on the dorsal aspect of the tongue were
electrodesiccated.
Only rarely did vesicles appear afterward,
and they were not symptomatic.
Case 2, C.A.
A 3%-year-old white male was first seen
because, 1 month before, the father had
noticed a small lump under the right side
of the child’s tongue. At the time of
admis-sion, the tongue was enlarged so that it
pro-truded from the mouth about 1.5 cm. A
large cystic mass was found on the floor of
the mouth beneath the tongue. On February
11, 1953, the cystic mass was removed and
the anterior 2 cm of the tongue was excised.
Histologic diagnosis was lymphangioma.
The child’s second admission in May,
1956, was necessary because areas of
vesi-des and bleeding papillary lesions had
ap-peared on the surface of the tongue and
were painful. These were electrodesiceated.
The child had no recurrence of the
erup-tive phenomenon, and speech remained
normal, although the tongue was slightly
deformed and all teeth were carious.
Case 3, CO.
A 6-year-old white female was first seen
with swelling of the tongue that had been
present since birth. Three years before she
bit her tongue, which bled profusely. Since
then the lesions had been asymptomatic but
had increased gradually in size. At the time
of her admission to the Children’s Hospital,
a blue nontender mass was present on the
left lateral surface of the tongue anteriorly.
A few vesieles were asymptomatic.
In May, 1955, an elliptical incision was
made about the lesion through normal
tonque tissue, and by sharp dissection the
entire tumor mass was resected. Histologic
diagnosis was lymphangioma. A smaller
le-sion posterior to the first was excised in
January, 1960.
Speech was very poor, but the child is
804
tongue later occurred only with respiratory
infections and were not painful. The teeth
were very earious.
Case 4, L.L.
A 2-week-old white male infant was
ad-mitted because of respiratory difficulty
as-sociated with swelling of the tongue. At the
time of birth two small firm swellings were
noted beneath the mandible, and the tongue
was thought to be too large. There were
small lesions which, in retrospect, may be
described as typical vesicles treated by local
caustic applications. A burn of the tongue
produced by the medication had resulted in
secondary infection and a cellulitis leading
to swelling of the tongue and difficulty in
respiration. At the time of admission the
child’s mouth was open and from it
pro-truded a purple, swollen tongue.
Surround-ing the bilateral neck mass there was
obvi-ous edema and inflammatory change. The
temperature was 101.6#{176}F (38.7#{176}C).
Following subsidence of tile infection and
edema on September 8, 1948, a
multi!ocu-lated lymphangioma extending back to the
angles of the jaw bilaterally and well
around to the posterior triangle of the neck
was excised except for some small locules
which extended to the base of the tongue.
Histologic diagnosis was lymphangioma
in-volving the submaxillary gland.
On November 17, 1948, four radon seeds
were inserted through the tongue into the
areas of remaining lymphangioma.
Addi-tional admissions in March, 1949; April,
1949; November, 1950; January, 1951;
Feb-ruary, 1952; and September, 1959, were
necessitated because of an enlarged tongue
with acute inflammatory change and pain
on eating. On these occasions
electrodesic-cation of vesicles and papillary hemorrhagic
lesions was performed. At times this child
was almost completely free of lesions on the
dorsum of the tongue and at times he was
completely asymptomatic. His exacerbations
were triggered usually by acute
upper-re-spiratory infections, although ingestion of
some foods, especially salty ones, produced
painful episodes of several days.
Case 5, M.A.
A 2-week-old white female was seen with a mass under her tongue and bilateral cystic
hygromas present since birth. At the time
of admission the tongue was pushed
cephalad, causing difficulty in feeding and
excessive salivation that tile patient found
difficult to swallow. On January 7, 1952, a
sublingual incision was made and the mass
between the tongue and tile floor of the
mouth was removed; tnacheostomy was
pen-formed because of tile considerable
respir-atory difficulty. Histologic diagnosis was
cavernous lymphangioma.
The cervical mass on the left was
ne-moved in January, 1952, and the mass on
the right, 14 days later, each by radical
ap-proach to the appropriate side of the neck.
Histologic diagnosis was similar to that of
the subglottic lesion.
The child was admitted again in July,
1953, to undergo direct ianvngoscopy before
the tracheostomy tube was removed. This
examination revealed considerable
involve-ment of tile larynx and epiglottic folds with
lymphangioma. These areas were treated by
electrodesiccation, and tile tracheostomy
tube was left in place to be removed finally
in September, 1953.
In January, 1955, a lymphangiorna was
removed from the submental area. In
Feb-ruary of the same year electrodesiccation
was performed on the dorsum of the tongue
because many of the vesieles had ruptured
spontaneously and had left behind tiny
blood-filled cystic areas which were painful.
In May, 1958, the child was admitted again
because of an acute glossitis, a
tempera-tune of 105#{176}F(40.6#{176}C) and inability to close
the mouth because of swelling of the
tongue.
Final admission took place in June, 1958,
when a partial glossectomy was carried out.
Speech remained difficult to understand;
there was deformity of the tongue from
scarring and prominence of the mandible
in reference to the other structures of the
face.
The youngster had very bad dentition;
crusty foods were difficult to eat because
they could not be placed properly on the
tongue. Exacerbations of the eruptions on
the surface of the tongue were occasioned
usually by upper-respiratory infections.
Case 6, K.O.
A 7-month-old white female was noted
at the time of delivery to have a swollen
tongue that interfered somewhat with
respi-ration and necessitated the use of oxygen.
From then until time of admission there had
been recurrent swelling of both sides of the
neck involving the region of the angle of the
jaw as well as the floor of the mouth. The
size of the swelling was inconstant, and
before she had been seen by us the
diag-nosis of cavernous hemangioma had been
entertained and three x-ray treatments had
been given without beneficial result.
At the time of admission she had a short,
fat neck and gross swelling over the angle
of the left jaw. A single, large, freely
mov-able mass in the right anterior cervical
tn-angle and a large mass below the ramus of
the right jaw were prominent findings. The
floor of the mouth was irregular, and the
buccal mucosa, swollen. Tile tongue was
covered over its anterior surface with
yes-ides and papillomatous lesions.
In March, 1949, tile tumor mass was
cx-posed in the neck, and the right half of the
cervical lesion was excised. Tile histologic
diagnosis was lymphangioma. Tile second
admission, in April, resulted in excision of
tile mass on tile left side of the neck, and it
was clear from this dissection that the lesion
there was contiguous with tile one in the
floor of the mouth. Tile postoperative course
was stormy, and on the evening of the
op-erative day tile patient became cyanotic and
was in status epilepticus for several hours.
Convulsions were finally controlled with
intravenously given sodium pentothal, and
there were no sequelae to this episode.
On the third admission, in May, the
tongue was enlarged and protruded from
the mouth, with painful papillary eruptions
over its inferior surface. The oral cavity
ap-peared to be almost completely filled by the
tongue. A large cystic mass was excised
from the base of the left side of the tongue.
This lesion was vell encapsulated and did
not extend into the adjacent tissues of the
floor of the mouth. Although the procedure
reduced substantially the size of tile tongue,
postoperative edema caused the tongue to
swell remarkably and protrude from the
mouth by the next day. The color became
deeply cyanotic, but there was no
obstrue-tion to the respiratory tract. Edema had
completely subsided in 6 days to permit
taking food by mouth.
In October, the child fell and struck her
chin on the floor. Shortly thereafter the
tongue began to swell, and on admission
the next day tile tongue was protruding
be-yond the tips of tile teeth approximately 4
em. It was dark purple in color and dry.
Final admission to the hospital was for a
partial glossectomy which was carried out
in September, 1950, when the anterior half
of the tongue was excised with wedging
and construction of a reasonably pointed
tongue that fit the oral cavity.
With subsequent scarring the tongue
be-came quite deformed and still had a
num-her of ectasias and papillomatous lesions
over its dorsal surface. However, speech
be-came quite good, except for some difficulty
in tile sounds of “s” and “1”, but the child
progressed well in school. She was not
troubled with pain even when tile eruptive
lesions were prominent.
Case 7, K.R.
This 23-year-old white female was first
seen with Il enlarged tongue wilich had
been asymptomatic until tile time of the
initial consultation. The child had been 6
weeks premature and weighed 5 lb 8 oz
(2,495 gm). At the time of admission,
the
tongue was enlarged so that it protruded
from the mouth about 2.5 cm and was
covered with numerous clear lymphatic
ectasias. There was a cystic hygroma
in-volving tile entire right side of the neck
cx-tending from the angle of the jaw anteriorly
and downward to the supraclavicular space.
the floor of the mouth or in the buccal
mueosa.
On June 2, 1952, under general
endo-tracheal anesthesia, the hygroma was
re-moved; it was somewhat difficult to
cx-tirpate completely because it extended
be-tween the esophagus and trachea and also
was media! and posterior to the carotid
sheath. Following extirpation of the cervical
mass, the vesieles along the periphery of the
tongue were electrodesiecated. Histologic
diagnosis of the cervical mass was
lym-phangioma.
A second admission, in May, 1953, was
necessitated because many of the vesicles
which had ruptured spontaneously left
be-hind tiny blood-filled cystic areas that were
painful and prevented adequate chewing
and ingestion of food.
Additional admissions for
electrodesicea-tion of painful areas occurred in January,
1954; December, 1954; December, 1955;
October, 1956; and November, 1956. There
were times when the child was completely
free of these hemorrhagic excrescences on
the tongue and was periodically completely
asymptomatic. On other occasions there
would be swelling of the entire tongue with
temperatures up to 102.6#{176}F (39.2#{176}C).
Po-tato chips and certain soft drinks were the
only foods associated with exacerbations.
On the last of these admission nine radon
seeds were implanted into the tongue to
give approximately 500 r throughout the
an-tenor two-thirds of that organ.
By April, 1958, the tongue was so
en-larged that speech was difficult, and the
portion of the tongue that extruded from
the mouth under normal circumstances
be-came so dried out during sleep that it
cracked and bled. A partial glossectomy was
carried out, consisting of excision of the end
of the tongue that protruded beyond the
teeth as well as the excision of a wedge of
tongue in the longitudinal axis. Histologic
diagnosis was lymphangioma of the tongue
with chronic inflammation.
Following glossectomy the child’s speech
improved, there were fewer surface
erup-tions, the occasions of swelling and pain in
eating were fewer, and the remissions
be-tween such painful episodes were longer.
However, the youngster sti!! had some very
difficult times, but, with the knowledge that
subsidence follows, eieetrodesieeation has
been avoided.
Case 8, BA.
A 6-week-old white male was first seen
with asymptomatic bilateral submandibular
masses and macroglossia present since birth.
At the time of admission the tongue was not
only enlarged but covered with numerous
clear vesieles. On December 10, 1955, the
hygroma was removed and biopsy of the
tongue was performed. Histologic diagnosis
was lymphangioma involving tile
submaxil-lary glands and a diffuse capillary angioma
involving the tongue. A second admission,
in July, 1956, was an elective one for the
insertion of eight radon seeds. Additional
admissions in November, 1956; December, 1956; and May, 1957, were necessitated by
acute inflammatory episodes with
tempera-tures as high as 105#{176}F(40.6#{176}C), painful
eat-ing and swelling of the tongue, sufficient on
one occasion to embarrass respiration.
On August 3, 1957, fulguration of the
tongue was carried out; on November 27,
under general endotraeheai anesthesia,
par-tial glosseetomy was performed.
In April, 1958, the tongue was improved
and excision of a lymphangioma from the
left neck and marsupialization of a left
buc-cal cyst were carried out.
This child had episodes of complete
free-dom from lesions on the surface of the
tongue; most exacerbations were produced
by
respiratory infections, which requiredhospitalization on several occasions.
PATHOLOGY
The basic histologic lesion is a collection
of vascular spaces (Fig. 5), dilated and
bloodless, lying not only between muscle
fibers but extending to the mucosal surface.
The stroma surrounding some of these
spaces is quite fibrous (Fig. 6).
An inflammatory exudate is seen (Fig. 7)
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Fic. 5. Vascular spaces, dilated and bloodless, with extension to mucosal
surface (upper left). (x25)
Frc. 6. Fibrous stroma surrounding dilated lymphatics. (x 100)
of fungiform papilla is involved by the
lymphangiomatous lesion associated with
in-flammation and hemorrhage (Fig. 8). Focal
hemorrhage and inflammatory exudate is
seen in other areas (Fig. 9).
Thrombosis in small vessels is common,
and occasionally an organized thrombus
with focal calcification and recanalization
may be identified (Fig. 10).
It was the consensus of the several
pathol-ogists who worked with this material that
all lesions were pure lymphangioma and
that they did not represent tumors of mixed
cellular origin.
COMMENT
The children whose tongue difficulties
have been described here have other
stig-mata seen elsewhere with extensive
!ym-phangioma: overgrowth of adjacent
struc-tures and destruction of tissue. The four
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mandibles visibly out of proportion to their
maxillary development (Fig. 4). All the
children have carious teeth (Fig. 2), four
having almost complete destruction of the
first teeth and multiple cavities in their
permanent teeth. One child has had partial
tipper and lower dentures since the age of
8. Presumably the canious teeth result from
changes produced in the tooth buds by the
iymphangiomata.
The effects of partial glosseetomy have
brought an extension of time intervals
be-tween painful exacerbations that seem
greater than that to be expected from
re-moving the affected tissue alone. The
an-tenor portion of the tongue is most altered
by the eruptive phenomena, and anterior
resection would, therefore, be expected to
be helpful. However, one wonders whether
postoperative scarring might not also play a
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-Fic. 8 Lymphangiomatous involvement of the lamina propria of
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Several of tile children had radon seed
implantation in the early years of our
cx-perienee in hope that scarring might
pre-vent the continued eruption of the vesicles
on the surface of the tongue. Unfortunately
no beneficial effect can be attributed to this
therapy.
Electrodesiccation of the hemorrhagic
lymphangiectasias seem to be followed on
each occasion by a progressively more
seni-- 9
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Fic. 9. Focal hemorrhage and inflammatory infiltrate associated with
numerous lymphangiectatic spaces. (x 100)
role in the apparent improvement.
No explanation is obvious for the severity
of the pain experienced by some and not by
others. Although emotional differences in
personality may be partially responsible, it
did
not seem to us that it was a major factorin these eilildnen who learned early to live
with their difficulties and impressed all who
eared for them with their rather stoical
ac-ceptance of tile situation.
FIG. 11. (L.L.) Pre-adolescent boy, several years following routine glossectomy, well adjusted, and at this time asymptomatic in spite of peripheral
lesions.
oils reaction of the tongue as exemplified by
the edema, pain and systemic evidence of
inflammatory change. For this reason such
treatment is withheld as long as possible,
even though the end-result after the
im-mediate postoperative reaction has
sub-sided is very good. As the patients have
grown older, they have tended to select the
timing of desiccation, weighing the eventual
fresh start they have against the immediate
postoperative pain and discomfort.
The initiating role of a respiratory
infec-tion producing a painful exacerbation has
been uniform in all patients. Potato chips,
salted nuts and other salty foods were not
only very painful to two of the children
but produced a painful reaction for several
days after the insult. One child on several
occasions experienced a reaction similar to
that produced by salt when she ingested an
artificially flavored and colored grape
bever-age.
Obviously we have not found an
cmi-nently satisfactory method of management
in four of the patients. The other four are
quite well and suffer no impairment of usual
behavior. In the present state of our
know!-edge, partial glossectomy followed by
pen-odie eleetrodesiceation of the painful
hem-orrhagic lymphangiectasias provides an
ac-ceptable means of keeping these children
socially acceptable, speaking, attending
school and at times completely free of pain
(Fig.
11).SUMMARY
The eight children in this series had a
syndrome of lingual capillary
lymphangi-oma complicated by glossitis. Three with
major lymphangiomas of the tongue and
subglottie areas became symptom-free
fol-lowing surgical removal of the major lesion
and electrodesiccation of the hemorrhagic
lymphangiectasias on the tongue. Four of
the remaining five, all of whom had major
cystic hygromas of the neck, had partial
glossectomy; one of these became
asympto-matic. The four symptomatic children
pre-sented a recurrent syndrome of a painful
edematous tongue characterized by the
eruption on its surface of vesieles and
hemorrhagic papillary lesions that could be
relieved by electrodesiccation, which would
be immediately followed by glossitis, edema
and pain. Changes in the growth of the
mandible and dentition also occurred.
REFERENCES
1. Hodgson, C. H., et a!.: Hereditary hemorrhagic telangiectasia and pulmonary arteriovellous
fistula. New Engl.
J.
Mccl., 261:626, 1959.2. Singleton, A. 0.: Congenital lymphatic diseases: lymphangiomata. Ann. Surg., 105:952, 1937. 3. Lierle, D. M. : Congenital lymphangiomatous
macroglossia vith cystic hygroma of the neck. Ann. Otol., 53:574, 1944.
4. Fuller, F. W., and Conway, H. : Cystic livgrorna.
Surg., Gynec. Obstet., 108:457, 1959. 5. Perzik, S. L. : Early management in extensive
cervical cystic hvgroma and macroglossia. Arch. Surg., 80:460, 1960.
6. Goetsch, E. : Hygroma colli cysticum and hygroma axillare. Arch. Surg., 36:394, 1938.
7. Harkins, C. A., and Sabiston, D. C. : Lympban-gioma in infancy and childhood. Surgery, 47: