REVIEW
ARTICLE
HEMIHYPERTROPHY
Robert E. Ringrose, M.D., J. T. Jabbour, M.D., and Doman K. Keele, M.D.
Department of Pediatrics and the Clinical Study Center for Birth Defects, Children’s Memorial Hospital,
University of Oklahoma School of Medicine, Oklahoma City, Oklahoma
(Submitted November 12, 1964; accepted for publication March 28, 1965.)
Supported in pant by Training Grant in Pediatric Neurology, No. 5 TI NB 5387-02 from the
National Institute of Neurological Disease and Blindness and a grant from the National Foundation
supporting the Clinical Study Center for Birth Defects at the Children’s Memorial Hospital.
ADDRESS FOR REPRINTS:
J.
T. Jabbour, Children’s Memorial Hospital, University of Oklahoma Schoolof Medicine, Oklahoma City, Oklahoma.
PEDIATRICS, Vol. 36, No. 3, Pant I, September 1965
434
H
EMIHYPERTROPHY is one of the moreunusual and interesting errors of nature found in the human race. The Greek and Roman sculptors displayed asymmetry of the human body in their art, but
hemi-hypertrophy was first documented by
Meckel8 in 1822 and the first case was recorded by Wagner106 in 1839. Since that time, over 100 cases have been reported. The reported cases have not been tabulated
since 1927. The purpose of this
communi-cation is to report an additional case and
present a review of this interesting problem.
CASE
REPORT
A 16-year-old Negro male was admitted to the
Children’s Memorial Hospital with a history of
asymmetry of the body since birth. The
develop-mental history was remarkable in that he held his
head up at 3 months, stood alone at 18 months,
talked at 3 years, and walked alone at 5 years.
He attended school for 6 years but did not learn
to spell his name, read, write, or count to 10.
He was the second of 1 1 children. All of his
siblings had an area of red hair on the right side
of the head, but were considered to be normal
otherwise. There was no family history of
anatomi-cal asymmetry or mental retardation.
The patient had a left hemihypertrophy which
was more prominent in the face and legs (Fig. 1).
The size of the child was similar to that of a
10-year-old. The facial asymmetry was associated
with an enlarged left tongue, mandible, maxilla,
and auricle of the ear. The hair to the right of
the midline was red, fine and thinner, while the
hair on the left side of the head was black and of
normal texture. Increased pigmentation on the
left side of the face and neck was especially
prominent just beyond the midline, where there
was hypertrophic epidermis. The left nostril was
larger than the right and the nasal septum
devi-ated to the right side. The left side of the tongue
was enlarged and deviated to the right. The teeth
were larger on the left and deviated to the right
side. Gum hypertrophy was noted on the left.
The left thorax was larger than the right and a
thoracic dextroscoliosis was present.
The heart sounds were normal, regular, and of
a normal rhythm except for a grade 1/VI
holosy-stolic, low-pitched murmur at the left sternal
border in the pulmonic area which disappeared on
exercise. Multiple hemangiomata were noted over
the body and extremities, but none were present
over the head. No venous hum or bruit was heard
over the skull or body. There was increased venous
distention throughout the right side of the body
(Fig. 1). The temperature of the right foot and
lower leg was wanner to the touch than the left.
The liver was palpated 2 cm below the right
costal margin. The penis was infantile but was
of adult proportions due to the presence of large
superficial veins resembling vanicosities. The testes
were small and measured 1
x
2 x 1.5 cm; therewas a 3 X 3 hydrocele on the right. There was no
pubic, facial, or axilary hair. On rectal
examina-tion large internal and external hemorrhoids were
noted. The extremities were disproportionately
large for the body size, and the left foot was
noticeably larger than the right (Table I). Edema
was present in the distal portions of the lower
extremities. There was a scal’, sharkskin-like,
darkly pigmented stripe on the dorsum of the left
foot along the distal L-5 nerve distribution and
another on the back of the left leg in the S 1-2
nerve distribution. The neurological examination
was normal except for mental retardation.
Hemograms and urinalysis were normal. A
sickle-cell preparation and a serum Wasserman test
were negative. The BUN was 14 mgI 100 ml, a
fasting blood sugar was 96 mgI 100 nil, and a
TABLE I
COMPARATIVE BODY M}ASUREMENTS
Organ
Ears
Chest Lpper extremity
Biceps Forearm hands lowerextremity
Thigh
Calf
435
REVIEW ARTICLE
Right I.e/I
ieasuremenI
(c,n) (cm)
.5.5 \ertical 6.0
3.5 horizontal 4J
:u.o Ilemicircurnferene :14.0
6 .0 Length 64) .0
15.0 Circumference 17.5
0.0 Circumference 21.0
20.0 Length 18.2
61.5 Length 611.5 29. 3 Circumference 32.0
25.5 Circumference 26.1)
29.3 Length 32.0
gm/100 imml albumin and 3.4 gm/100 ml globulin.
A serum calcium was 4.7 mEq/l and a serum
5. 1 irig/ 100 ml. The protein-bound
iodine was 6.3 icg/ 100 ml and I’’ uptake was
31.5% (normal range 5-40%).
The llickev-lIare2’ test revealed normal
anti-(liuresis on the administration of hypertonic
so-ilium chloride. Urine chromatography revealed
100 tcg of glvcine, 50 tcg giutamine, and 50
/Lcg of alanine all per rug of creatinine with traces
of taurine, tvrosine, and cysteic acid; these were
normal levels. The SU-4885 (Methapyrone )
sup-l)ression tests’ showed an increase of the 1
1-des-oxvcorticoid-creatinine ratio from 1.50 to 13.23,
which is a normal response. The urinary
17-keto-steroids were 4.5 mg/day; a urinary gonadotropin
(letermination was greater than 6.5 but less than
13 IllOliSe units in a 24-hour urine collection.
The psychometric examination showed a mental
age of 4 ears ( I.Q. 23 ) indicating severe mental
retardation. Testicular biopsy revealed small
semi-niferous tui)ules showing no spermatogenic
activ-ity, compatible with the diagnosis of atrophy of
the testes due to immaturity. Buccal mucosa
smears were negative for sex chromatin l)Odies
bilaterally. Gel infusion electrophoresis revealed
normal giucose-6-phosphate dehydrogenase
activ-ity and electrophoretic nigration without evideiice
of mosaicism on his X chromosome.”
Chromo-sonic analysis revealed a normal karvotv1x with 40
chromosomes. Electrocardiogram reveale(1 night
atrial hvpertrophv.
X-ray examination revealed consistently greater
size 00 the left of the calvariuni, niaxilla and
adjoining I)011C5, maxillary teeth, and mandible;
the superior orbital fissure; the rib spaces; and the
pelvic osseous structures and soft tissues. The
heart was normal, but with a concave rulmlonary
artery segnient and prolilinence of pulmonary
vessels in the right lung. There was a minor
fusion of a sacral vertebrae. An intravenous
pyelo-grain was normal.
The bone age, 12.5 to 13 years, was retarded.
FIG. 1. Left hemihvpentnophv more I)ron)illint ill
tile face afl(l leg. Promiint V(110115 distention
throughout the right side of the body.
Both hdfl(ls and feet sere unusually large, vith
the size dfl(l length (l11C to overgrowth of the
phalanges. The bones on the right sere snialier
throughout than those on the left (‘Xce1)t in tile
hand, l)ut the fourth lIl(l fifth digits of the left
hand were larger than those of the right.
The electroencephalogram sas mildly .Ll)normai
vith moderate amplitu(le reduction of sled)
spindles iii the right frontal (lli(l tenlporal areas
with normal patterns of drowsiness aixi sleep.
(OM M ENT
It is now commonly accepted that in
normal individuals the left cerebral
hemi-sphere, the left half of the skull, and the
left side of the face are larger than the
right. The left cheek and left half of the
lower jaw are slightly more prominent. In
contrast, the right halves of the vertebral
bodies, as well as the ribs of the right side,
430
IIEMIIIYPERTROPIIY
of the right upper extremity are longer and
slightly thicker than those of the left. The right upper extremity may be as much as 1 or 2 cm longer than the left. In the lower extremities the situation is reversed, the
left side more often being longer and stronger than the right. The pelvis is rarely
symmetrical.#{176} The right ear and right eye
are usually a few millimeters higher than the left. The two nipples are seldom at the
same level or at the same distance from the
median line, the right higher than the left.5#{176}
Normal asymmetry, therefore, is common,
not always apparent and usually must be
established by exact measurements; on the
other hand, gross asymmetry which is at once perceptible to the eye is rare.
\Vard and Lerner109 presented a revised
classification of hypertrophy, dividing it into congenital and acquired. Congenital
hypertrophy is further subdivided into
“total” and “limited” hypertrophy. In total hypertrophy, all systems of the body are
involved and consist of segmental
hyper-trophy, crossed hypertrophy, and
hemi-hypertrophy. Limited hypertrophy affects
only the muscular, vascular, skeletal, or
neurological systems. Total congenital
hemi-hypertrophy includes hypertrophy of the
paired internal organs on the involved
side.72’84 Congenital hypertrophy varies in extent and severity, an almost infinite num-ber of variations being possible.
A review of 104 confirmed cases of
hemi-hypertrophy and 25 cases of segmental
hypertrophy from the English and German
literature is given in Tables III and
IV.
Incidence and associated anomalies in hemihypertrophy are given in Table II.
The case reported here has many of the commonly associated features of hemihy-pertrophy (Table III
)
. Abnormalities not previously recorded include increased skintemperature and venous distention on the
uninvolved side, hydrocele, testicular aLto-phy, and the large penis with sexual
infan-tilism. Although 2 were noted to
have bilateral enlargement of the hands and feet, and many cases of unilateral facial
4247 88, 96 have been reported,
none have recorded the clear-cut facial
hemihypertrophy with the enlargement of
both hands and feet yet with the
hemihy-pertrophy continuing into the trunk, pelvis.
and lower extremity.
Hemiatrophy, thought by some to be
re-lated to scleroderma,7’ #{176} but related to
brain injury by many,15’ 05. 74, 109 may give
difficulty in diagnosis. With hemiatrophy, there is accompanying evidence of neuro-logic disturbances, usually acquired rather than congenital.3’65’ 77,110,115
There are findings in our case which sug-gest that it is one of the congenital neuro-cutaneous syndromes
(
neuroectodermaldy-splasias
)
. Recognized entities of this groupinclude
(
1)
neurofibromatosis(
vonReck-linghausen’s disease
)
, (2)
tuberous sclerosis(
Bourneville’s disease)
, (3)
trigemino-en-cephaloangiomatosis(
Sturge-Weberdis-ease ), and
(
4)
hemangioblastoma of the retina and cerebellum(
Lindau-von Hippel disease) 117, 118 These have the followingfindings in common :
(
1)
developmental anomalies of the brain, skin, retina, skeleton, and viscera,(
2)
heredo-familial incidence, and(
3)
co-existence of other develop-mental stigmata. Commonly recognizedfeatures of these diseases also occurring in
our case are: cafe-au-lait spots, hemangio-mata, edema, sharklike skin, mental retarda-tion, facial nevi, enlargement of one side of the face, organomegaly, and involvement of bones. The presence of a patch of red hair on the right side of the scalp in our
patient and his siblings suggests a
heredi-tary involvement. Yakovlev and Guthrie116 have suggested that the congenital neuro-cutaneous syndromes have a common origin
in a malformation of the ectoblast; the differences are explained by the disturbance
occurring in a different period of
embryo-genesis.
The etiology of hemihypertrophy is not known; several theories have been ad-vanced. These include anatomical and/or
functional vascular or lymphatic
abnormali-ties of the hypertrophic 42 63, 72, 75, 80,
88, 90, 11, 113 lesions of the nervous system
Abnornia/ily
Percentage of (‘asex
Reference
80 100
63
37
53
47
I 1 39
47 19
11
6
3
1 4 4 4
5
C)
7
C)
‘5
1 5 3 1
4 3 4 I
1
3 3 1
2
REVIEW
ARTICLE
437TABLE II
IN(’IDENCE OF ABNORMALITIES IN HEMIHYPERTROPHY
I Iemihvpertrophv
Females
Males
Itight side left side Mental defiieney
Negro Mulatto
Noted at birth
Skin abnormalities Xcvi
‘I’elangiectasia Ilemangioma Cafe-au-Iait spots PapiHoma Pigmentation Cutis Marmorata
Skin temperature increased on involved side
Temperature instability
Skiti coarser on involved side Ilypertrichosis
hair thicker on involved side
Al)normal nail growth on involved side
hair different color on two sides
Premature eruption of teeth on involved side l)ifferences in tooth size bilaterally
Excessive secretion of sehaceous and sweat glands
Epicanthal folds
Other Features
Involved side stronger Uninvolved si(IC stronger
Some internal organs eniarge(l on involved side
All internal organs enlarged on involved side
Prominent in face afl(l leg Mac rodaetlv
Syndactyly Polydactyly (left foot Bilateral IWS (ILV(1S
5, 15, 5, 43, 46, 47, 48, .59, 61, 71, 7, 7.5, f), 93, 108, 09, H3, 114
80
3, 5, 15, 5, 37, 4, 43, 47, 48, 59, 7, 9, 93, 98,
108
3,88, 4, 43, 47, 48, 50,69 10, 15, 4, 44,80,83 15, 44, 95
7 4, 46, 49 3, 15,42
4, 47, 59
44, IU 3,43,46,48,69
3,4
15,38, 42, 44,61,66
4, 114
38,61 113
3, 43, 46, 48, 69 44, 66, 114 75
3, 4, 47, 59
37,66, 114 3, 4, 47, 59
97
37, 48, 66, 93, 108
43
15,46, 114
15, 108, 109 15
7:
(Continued on ,aezl jszye)
to
tiit’ ilv)eltropllic area,-” ‘‘ ‘ , #{176},:..:, ::i. 7::. 7:. Ml. I) . I ‘9. 1 59 ell(lOCriHe
abnorrnal-ities,’’ 12. 4. II. ‘I;. 105 Ll)norn1alities of the
ovtlln,41, abnormality of tvinning,7’ 12,43,74
and chromosomal rm926, 87,
102,112 The data in our case does not add any
new iIlfOrlnatiOIl as to etiology. Although io chromosomal abnormalities were observed
ill OU case, the possibility of some minor
chromosomal abnormality occurring cannot
Abnormality
of Cases References
1
I 17
1
1
S 1
I
1
1
1 1 1 1 1 1 3
1
5
7
1 3
5
1 1 1 1
1
1
1 4 1
438
HEMIHYPERTROPHY
TABLE II
INCIDENCE OF ABNORMALITIES IN HEMIRYPERTROPILY (Continued)
Talipes valgus
Congenital dysplasia of the hip Spina bifida occulta
Compensatory seoliosis
Kyphosis
Spondylosis
l)eviation of phalanges Atrophy of 4th metatarsal
Bone age increased on involved side Umbilical hernia
Inguinal hernia
Elevated urinary gonadotropins
Hypospadias
Cryptorchidism Large clitoris
Large penis with hirsutisin Menorrhagia
Virilism secondary to adrenal tumor on involved
side
Wilms’ tumor of involved side
Undifferential sarcoma of lung on uninvolved side
Cephalgia
Otorrhea
Supernunierary nipples Congenital heart disease heart enlarged
Increased superficial vessels Varicose veins
Strabismus Nystagmus Optic atrophy
Normal EEG
Abnormal EEG
Involved cerebral hemisphere larger Internal communicating hydrocephalus
Larger ventricle on involved side
Brain atrophy Poreneephalic cyst Short stature
Diagnosed as eiephantiasis Diagnosed as lymphedema History of rickets
Localized gigantism of leg associated Bronchiectasis
Epilepsy Tuberous sclerosis
93 15
74
5, 15, 19, 4, 46, 66, 72, 74, 80, 93, 97, 1(18, 114 5, 7
15 0, 93 S 43, 7
15, 37, 57, 71, 91 15
94,95 108 91, 108
48
43,91 4,
51, 5
8
46
47
4 50
93, 108
3
15, 47, 69, 9, 109
5,43, 47, 50, 99, 114
48, 51, 48, 75
75
72,95 46,47 4, 44, 48
75 46 75
75
95
41, 48 63 80,99 80 3
0, 46, 48, 50
48
Even though the findings in our case suggest that it should be classified with the congenital neurocutaneous syndromes, it is not suggested that all cases of
hemthyper-trophy have a similar origin. Table II shows
TABLE III
C.&s:s o}’ IIEMIIIYPEItTItOIhI Y
Sex .lf/c
:si io yr
2yr
2yr
Side Pin’ Inrnlrrd’ iIr’iIa1iI
R head, face, ear, Normal
teeth, tongue.
tireiist,chest, UE,
& I.E.
R Entire side .
It Nose. ear, tongue, .
teeth, UE & l.I.
4\oled Birth Birth Birth Birth As a child Smu Birth 2 mo Birth Dirt hi Dirt hi Dirt hi Birthi 5mu 19mo Dirt Ii Sliii) F 1’ F F 51 F F F I” F F F 1’ F F F F I., I.’ .lulhor Adams, 1894’ Ahilfeld, 18822 Arnhieim, 189hz Dahionniex iiil Biiizzard, 1922’ Burr, 19295 IhiiMN()e, 1912’ lhjorklund, 1955’ Jhiiisqtiet, 1921’ flowers, 1950#{176} Droca, 1859#{176} Driining. 1904”
Bryan, Lipscomb. &
Chatterton, 1958”
Bryan, h.ipsinih, &
( h,itterton 1 958”
(contintiel)
11,11, 1875” I’ . hhirthi
4 mu 12yr 22 yr 16yr 4yr 4 nb :tyr 14yr 1 yr 21 yr 15yr 18yr 14yr i:t yr 31)sr 4yr 4yr 4yr : i,, 11 yr
.-kii Oth,r Fealar.’
Ilair on It tipper lip (‘onipensatory stohio-darker & longer. ‘tel- sis
angectati( sjsits flOilfi-l over I side.
Nevus pilosis hiypertri- 11teeth ti(hiilt in size;
chosis, skin on trunk II si(Iestrongest; heart thicker, reddish-blue, enlarged;
Bronchi,-&with, cold cracked & ectasis; Necrojisy
turned violet.
‘reIirigi-ectasis in middle line
of chest & hack.
Extensive iievi oil I. \larkeil traiisverjc leg. iiisal vein
. . Obese, scohiosis
Irregulir red hiliitdies .Strophiy if #{149}tth metsi-on extremitie, tiit iiiit tarsal 1. f’sit 011 the face.
hlvpertrichiiisis. it’nieiited nevi.
. . 55iln,’s tiinior I.,died
it 4 yr
. . lateral (Ieviation of it ;ihialaiiges
hlemarigionia at I.
iii-gle of the mouth & 1
forearm
Nevi, pigmented I lypertrophiy of 1 2nd angii)flia finger
. . Severe scohiosis; No uvula; increased nuni-her of superficial veins
of chest & abdomen
Cutis niarniorata &
strawhierrv nexi of L
forehead kCliNk. Skin texture different in I side
(hoanal occlusion (if
Itfiosteri(ir
Ilair ilenser in 1. 11 iIii_’iiiiICil &unibihical SVehihied tics i)i( hi hieriiia
feet; ileniangionia (ifl back
(‘urnjiensatory
scohiosis Cafe’-ai,-lait 51)(its (in
trunk
Accent(iate(l riitis
niarmiirat
I lair curly un I.side (if ( ‘onigenit al hysplasia tieail 1k st raihit
on
H of 1 tip. (iiXisuit siiindylosis of Iril hurutiar sertehira
- . (‘hiiefly in siihii’iit
sine-(ilis tissues; cleft foot
& I Ities it 1. I It 1 1 it R It 1. it I. 1. 1. L I 1. IQ 81 Normal Siihniornia I itetarihed Noririal Nitrnial Nitrnial Nornrial Normal Niirnial Normal Niirrrial Normal Nornia I i;,itire side
Face. ear, fE, & ii:.
Both fingers, feet,
&ankles large, hut
I.greater than R, 1(5 iS lureast &
Ia-huiuitn.Nii face
in-volsenient. I’ntirc si(1C Entire siule Entire siile 1’:uitire siihe Entire side including tongue Entire side Total ‘Iuital Total Iiutil ruth ‘I’ottih
‘Fit a I
Total
‘roth left &it foot larger ‘Fot aI
1’,uttil
Entire side
* 1]- tier ext renuity; + iuui’r,.tuseih - utiul; I.E hiisver ixt reniiity: - uheureaseth
Author Sex Age NO(eVI Sid6 Parts lnrolred’ Mentality Skin Other Fea!ure
Burlet, 18O” & Oilier. 186276
Cagiati, 1907”
Carter & Dockeray, 195320
(o,toni, 192021
Cozculino, 192322 Crosby, 1933 Davidson, 1930” Demme, 1891” Devounges, 1836” Dodge. I92’ Eaton, l8862i Fatichi, 1895” FinIayson, 1884” Fortescuic-Brickdale, 1915” Fowler, 1900” Fr#{225}nkel, 1868” Gesell, 1927” (,ianier, 193344 Gordineir, 1918”
Gorhini & Sleskini, 1962” F F SI SI SI F F F SI SI F F SI F F F SI F F 27 yr 15 18 mo 22 mu 6 mo 30yr 5yr 18 yr Iwk 17 yr SI yr 10yr 53 yr 18 no 9 10 mo 12 yr 4 yr 25 yr 3yr 63 yr 9 yr Birth Birth Birth Birth 6 no Birth Birth Birth 2mo Birth Birth 2yr 2 yr Birth hhirth R I L It 1. It R It L It It I. L It It R R It It I I’ Entire side Entire side Entire side
Head, ear,
mcmli-ble,chest, UE, &
LE
Entire side
Pupils, ears,
tongue, UE, & LE
LE, UE, & slight
of face
Entire side
Entire side
Entire side except 3rd & 4thfingers & toes.
Entire side
Entire side
Entire side
Entire side
Face, ear, tongue,
lower jaw &
cx-tremities
Face, pupil, cheek,
ear, tongue,
lab,-urn,UE, & LE
Entire side
Entire side except tongue & teeth
Ear, nose, palpe-hralfissure,cheeka, lips, teeth, penis,
IJE, & LE
Face, ear, thorax,
UE, & LE
Entire side
Entire side pupil.
face, togue,
teeth, UK, & I.E
Subnormal Temperature + on It
side
. . Nevi, pigmented keratosis
Subnormal Skin coarser on L
Nuirnual
Normal
Retarded Fine nex-i
Normal
Nevi on hand & leg
Subnormal Museum of nevi
hipo-mata
. . Dark red on R side,
blue on crying
Norml Nevi
Multiple telangiectasia
onIt side; nails on it
dry, rough & ridged
Subnormal -.
IQ-65
Mental age Port-wine
telangiecta-tic patches of R hand
&leg
Normal Hair thicker on
violet red hemiangioma
over most ofR arm &
upper lip; when crying I side of face & trunk
were discolored &
pro-fuse sweating limited tothe I side was noted
Normal Nevi
hQ 66 Pigmentation of it &
I arms; large
cafe-au-lait area in sacral
region
Vasculurity increased,
CephaIaia &
menor-rhagia. More
pro-nounced in leg
Frequent petit mci
at-tacks; compensatory scoliosis; hone age on
I 21 yr, on R 18 no;
necropsy--h kidney & lung larger; cerebral hemisphere, thyroid,
adrenal, tonguic &
testes were =.
Parents of lw
nieni-tahity
Scoliosis. 2 mu
lire-matuire
Decreased muscle
tone, kyphosis,
epi-physeal development
advanced
Necropsy - I
cere-bral hemisphere +
it side stronger, van-cose veins, 2 htees +
Macrodactyhy. I)e-formity of I knee,
varicose veins.
Chiefly in sulicutanc-oils tissues Otorrhea. Tempera
tore + on It side
Mostnoticeablein face
& hE; umbilical
lien-nizi. I handed
Shuistachie fuller on it
I side relatively rn-mobile in smiling
Reversed asymmetry
of epiglottis, varicosi-ties of R leg
At autopsy It kidney
&thyroid were larger but ovaries & adrenals were = ; bilateral
en-largement of brain
more pronounced on
R; microscopic, R
conium thicker, it
brain more cellular; also, instability of
temperature control
Neeropsy - I,
eere-bral hemisphere +
Smuliosis; 1.eye bnuuwni
& it eye blue;
syndiu--tylism tuihiterally; I,
ventricle larger thaii
R on PiG; seizure, alunormal FiG;
un-differentiated sarcoma of It lung removed
440
HEMIHYPERTROPHY
tide PurL, I,,uolred 11en1(IlzIy Ski,, 01/ic,’ Feature-v
Skull, teeth,
tongue, epiglottis,
maxillary process
& LE
Forehiead, face, Retarded parietal eminence,
ear, jaw, 1.E, &
IJE
Chieek,ear, tonguie,
breast, labia
maj-jora, UE, & LE
Head, (-heck, li;1, Normal ear, jaw, tuunguie,
LE & UE
Face. tiunguie, Ui, Normal
& I.E
Face. ear Nornial
Entire side
Euitire side
Ftce. hie:id,
tuinguie, LE, & FE
Fu-e, tongue,
i’hiee ,chest,
pcI-‘is, FE, & LE
-- Sei: .lge
Gross & Iil)errak, SI 52 yr .
lt)’5548
F 10yr
hall, 11)21#{176} I” 7 uuiui Birth
Ilaljonini, h91l’ SI - llirthi
Iharwood, 11)32” SI 8or Dirhi
1larwood arid
O’Flynn, If)35’’
hhenchi, \\‘akefield 1’ 21 yr 23 yr inil (itniiju, 1912”
Ilornstein, 1893” F’ 2 mo
lIut-hiinson, 19ItP SI S yr
.Ji’iihui, 1871” . 1 mo Birth,
Korting arid F- 17 yr
Riitt,er, 1953”
haniglet, l882’#{176} . Auhiiht Lisser, 1914” F- 45 yr
I-ugn, 1868” F’ 4 yr Birth
Mann, 1955#{176}’ F 6yr
-Slasmejean, I 888” I yr -
-\I;i’ers, 19295’ F 9yr birth
SIeGregor, ISt)4’ SI It) yr
ShIne, 1895’s F’ 19 mu Birth,
it
It
It
it
it
It
It
It Entire side
It lIeuI, face,
tongue, trunk,
CE, & LE
It l’nitire side
I, F’ac-e, arm,
hint-t(uck & thigh,
it Entire side
It Head, face. e:ir,
neck, chest, t’E,
& LE
R Entire side
it head, face, clue
nasi, J)alate,
tonguie, teeth, ton-sil, trunk, Iabii,m,
CE, & 1.1’
Numerous
telangiec-tasis (in I back
Extensive
hyperkera-totic, dark brown
ne-ylis of 1 neck;
streak-ing of I nipper arm and
enlargement of under
arm with horny skin
with pale grey-brown discoloration
(lear
Supernuirner,ry nipple
on it; ahudonien, both
LE’s & back have J)1(tch(es of port-wine teltngiect,sis
Nevi
Itetardeil Skiii disorder on cheek;
nexus fhammeuis R
chest, abdomen & face
more pronounced in
midline; revuis venosuis
on It hand, foot, and
tees; teniperatuire + un It
Depressed Finigerni,ils, it hand
dry, rough, ridged;
mustache fuller on R
side; hair coarse & dry
Skin & subcutaneous
tissue thickened on I
Normal i hair coarser on it; skin
Ithicker on R; R side
perspires profusely &
emits a peculiar odor
- - Dark skin
. . Cutaneous congestion
greater on It
Elephantiasis of I.
lower leg; autopsy
- inner organs =,
cerebrum, medulla,
and cerebellum larger
on L
Tumor of cheek
covered with a thick
horny layer of skin &
dirty brown
pigmen-tation; I upper 1ip ful-her & knotty; large cli-tons; alternating
coii-x’ergent strabismus &
rotatory nystabmuis;
tuberouis sclerosis; epi-leptic; h cerehiniini is larger
Epileptic; veins (Ii-lated on L arm & It
leg
Ilyperplasia in both
testes & peais with it
testis largest; many
seizures terminally,
iuitopsy - kidney &
liver larger on R; It
adrenal replaced by
tumor; I convergeat
strt,bismus
Postural sciatica (t
tnibiited to scoliosis;
Sacral spina bifida
oc-culta
Ne-ropsy-connective
tissue Iiyperplasia
Necropsy-internal,
organs of It, especial-ly suprarenal,
en-larged
Puilse more feeble on
I; umbilical hernia
Rside stronger; EEG
- increased activity
oil I.
‘I’iuuiguie,lip. & J)u(pil
all larger on It
l arm grossly en-larged, heavy &
ede-matous; thought to lie
congenital
lymph-edema of L arm
Better motor function
on I; scohiosis; hands
are; difference great
eat in face & LE
Necropsv - R optic
nerve t’Iualamuis en larged
6teeth on R, I on I
REVIEW ARTICLE 441
J-IEMIHYPERTROPHY
TABLE III (Continued)
F
F
F 9 mo
40yr
t yr
SIuinunier’, 1907’
-;z I lye Noted Side
F’ h2yr -. It
SI 1 1yr Birth, I,
F’ io sr
F
It) sr 442
1uI/mr
SIuuhuiu,s, h81)0#{176}
Slohir, 1t)25
Slorris and (A) SIarGilhivray, 1955’
(11)
((‘)
(I))
Nue unul Berman,,
1962
Patersuuni & F
Iteyniolds, h923
h’euhuuul’, 11)3(1’ F,
SI
F’
F
I5iuhlosson, 1884” ‘uI
Itedarul, IS9IP’ 51
hti,geh, 1916#{176}
Su-huiller, 1t)i(t’
SrliwCurtzniani,
Grossman, &
l)raguutsky, I441”
SI
F Birth
I
I.
I
It
I.
it
I.
It
It
I.
1.
It
it
l
I
I
Parts lnrolred Mentality ‘kiuu ()Iher
Entire side -, ‘l’elun,giectasis It forefinger & thuuunihi nuuurnsal
Entire side with, - ‘l’hiick skin; telangu- Pains ui I. urni
with tongue & estatic marbling,
((lute especially in it
I’iee, cur. tongue. Retarded -- Slight u,mhuilk’al
thorax. i-F:, & Iuerniu
F’ace, cur, & urnn IQ 42 -- Compensatuury
scu)Iiuu-Entire suhe IQ 38 1.ankle & wile thiiu’k- Extreme kyphuoscolio
Mental age enrol & huornu’ sis of the upper
thor-(,f8 yr ucic reguon ; huilateral iies coims osithi valguis
uleforniity (if the smuull
toes uI huuuthu feet, lEG
normal; sister of (A)
family has 10children
another sister has hQ
of 43; materniul
grand-mother had I facial
enlargement
Entire side IQ 36 SmuuII uigmenited Slight scuuluuusis;
vu1-pitches gus defiurmuty of the terminal PhiuhanX of
the ring fingers of buuthi
hands; EEG normal Entire side IQ 41 Papilloma on I calf Biluuteruul hiC5 cuis’uuS
daughter of (‘; father
not known, sister hius
IQ of 37
Entire side Normal I side uppurenthy
grew fiuster in 2 yr;
then followed Iuur 18
yr & practically us-uppeured
F’ace. huand, &leg IQ 33 Epicunithual folds, uufutie
utrophiy & haurizontiul
niystagnuuis; PEG
shuowed internal
con,-ii,u,iuiu’uting
huyulroee-1uhuulius with,
general-ized brain utrophiy &
Cu Iuorencephiuli(’ (‘yst in us(ei1uitu1 & -erehuel-lar regnon
(‘hieek, cur, tru,nik, Norrnul Skull uyniniet ru-a I
CF & LE
‘l’uutul Nuirniul l)iffu,e huiluterul her- Su ‘uliusis
niah ngiuunuu
Fuu’e, arm, & leg - - - Negruu
Skull, trunk, hE, Nuurmul , , S-uuliu,sis; SIuuluttuu
& I.E
Fee, UE, & I.E Nuurniul (‘uunaiulereul as
hienui-atrophy but proluat)ly
huemihypertrophuy
Fuuce, UE, I.E & Nuurmul Chondrooscous
hyper-jels’is trophy of I patella;
rickets at 18 mo;
lii-caulized giguntism uuf
greater trochanter uuf
femuir; multiple
suir-gery on grossly longer
L leg
Entire side -- (?)Elephiantiasis
Entire side Normal Nes’i hlypertrophy It fusut
s’ery marked;
temjuer-utiire + on It
Fuice & leg
Skull, face, tongue, , , - Congenital
omphalo-trunk LE, & UE cele; cryptorchidism
Face, cur, tonguie. Suibnormul Erythienuutuuuis & pig- Large penis;
promi-pupil. thorax, IF:. niented hulotchues noted nent superficial vein
& I.E on 1, jaw, arm & leg on L
Syr
I;nniuu
13 yr
hutyr
Ilyr
Syr
It) yr 5
%uluult .‘uuluilt Birth,
yr
F, 2yr
SI Ilyr
SI 7yr
Aut/ior Sex
Su’utt, 1935” F
Silver uI ui,, I95i’
Stunuhuun unuuh Tufts,
1t)1t
Telford, lt)ll”
‘I’huonuson, I89I’#{176}#{176}
‘rilunuis, 1893”
Tuirtuil, 1896”’ \uumuderweiult, I921’#{176}
Syr
6 yr
16
Age Noted Side Paris Inrolred Mentality Skin Other Features
I yr , - it Skull, urn, & leg Niurmal Smuill nexus uun it
shiouilder
Talipes valgus (if I
foot, Congenital huean-t
(prohu,uble pulmonic
stenosis), bone uuge
normal
F 26 yr 9 yr I Faec & leg Normal . Compensat ory scolio
sis; mother of the
pre-ceding
I” I I no,) . It Body, f,uce, &
cx-tremities
Retaruleul Bone age on It 4 yr
on I 2 yr
I” ‘2yr I Entire side N,urnial (‘afe’-a,i-huit spot on
I, luuwer ahuul,uii:eii
EEG niurm,iI; shuu,rt
stature; elevated
un-ii.ry gonadotruu)iiis
SI Ihirt hi It Enit ir(’ siule Nuurni:uI - , Shuuurt stutu,re;
dc-s’zited
guuiiaduutruup,nu-(urine)
F, Birth, I hle,ud, face, bunguue
trunk, CE, & I.E
N,ormul Scoliosis; It siule
stronger
F, 7 yr -- It Skull, face, IF,
pelvis & LE
-- Extensis-e port-wine
lies-ui, on lower trunk
on R
Itickets equi’il oni Iuuuthu
sides; vanicosities over
uihudomen & thigh
I” 18 no - , R Entire side Suihunormal , , ?thyroid absent
F’ 10yr -- It Entire side Nuurmal Telangiectiutic
mar-huling
It side stronger
SI 2 n-k Birth I Entire side
-‘uI I I vs-k it Entire side Retarded It side more c(ungested
suhuen infant cries
Asymmetry more
pro-n(uumnced in LE’s
SI I 1yr - 1. Fntire si(le ‘relangiectusis; Iuluue
pignient
SI 7 yr Iyr I lIe,ud, fa-e, & leg Nuurmal -,
IIy[s)spadiuis,cr5’pt(ur-(-hidism;? congenital heart (Iise,ise
F’ 9 yr ‘1 yr It l”ace, leg, & arm Nuurmal , , Iliurkeul scohiosis
‘uI 11yr - 1. F’au-e, u’hiest, arm,
& leg
Nuurm,ul , , Congenital heart
(his-case; sulydaetyhisni
SI 18 yr -- it h”iuee, arm, & leg Noruml
F’ -13yr Ihurthu It I”aee, brast,
labia, ,urm, & leg Retarded
Slental age
(if 1)yrs,
Extensive nexus over
(‘best fl(I upper
him-huur region
SI 8 mu) Birth It Skull &
extremi-ties
S,,hunuurnual Suuperficiud hulood
yes-sels on It n,uure
prom-ruent
Suipernunnerary toe iuni It. It skull larger;
net’-ruups5’ after deuitb due
1(0 meningitis shiossmI
onuly proniiflefl(’e uuf
hulxd se&sels in the
suuhu(’uitaneuuuiu4 I mu It (hue tuu nieuhiah hu-perplasia
F’ t; yr Birth, it F’,uu’c,tuungiie.
gums, truunk, CE,
& l1’
Returdeul - ‘I’eethu uun it it 4 no uuno
I at 7 mu; large lynn-phiangioma on the
huiuck ‘u’uagner, 1887”
%‘u’akefield unul
Iliuues, 1933”’
‘uVard ,unul l.erner,
1947i09
Williams, 1951”
REVIEW
ARTICLE
TABLE III (Continued)
443
rule out the possibility that the same
mccli-anism is involved in the formation of the
hemihypertrophy.
SUMMARY
(Please turn the page for Table IV) A case of hemihypertrophy with many
manifestations of a congenital neurocu-taneous syndrome is presented; the
pre-‘iously reported cases of hemihypertrophy
are reviewed and tabulated with many of
Author
I
Sex Age Noted aide Parts Invotvedt Mentality Ski,, Other Features
- - Partial sucrulizut ion
(of I-S
Arm, breast, & leg
Arm & leg
Arm, shoulder, &
leg Arm & leg
Arm & leg
Arm, leg, & I.
vulva
Arm, leg, & tongue
Arm & leg
Arm & leg
It leg longer, L
thigh & calf larger, I arm larger & longer
Trunk &
extremi-ties
Arm & leg
Cheek, lips, chin, ear, tonsil, tongue, &teeth
Facial, cheek,
tongue & teeth
UE, IE & trunk
Arm & leg
Teeth,, nose, maxilla, & tongue
Normal Normal Normal Normal ‘ormal Normal Normal Normal Normal Normal Normal Normal Normal Normal Nornuul -It) Birth 9yr 3 Birth, Birth Birth Birth 2no 9yr Birth, Birth 15 yr 11 no 2 no Birth 4 yr I nuo
Bryan, Lipseomb, F
& Chuutterton, 1958”
F F SI SI F ‘ui
I)umnhop, 190130 M
Fischer, 1880” F
Friedreichu, 1863’#{176} F
Pussauier, 1866’ SI
Reed, 1923” F
Riedel, 1952” M
Rudolph & F
Norvold, I944’
Sab,unius & ‘ti
Chmtterton, 1955’#{176}
Scott, i935 SI
Silver &
Gruuskay, 1957#{176}’ SI
Stafne & F
Lovsstedt, 1962” F It it It I, I R I-’ R I I-’ L It It L It I I It it I. I It 35 Fl. 29 yr 21 yr 17 yr 12 yr 26 yr 3 6 yr 1yr 112yr 9 yr 9 yr 16 yr 11yr 15 yr 11 mo 9yr t)sr 8yr Syr 3); yr hiemangioinu, It tongue Suiperficiuil
huem,uiugi-((ma on 1. trunk & thigh
I)ernioid cyst of fure-head
Sweat glands more
uc-tis’e on I &
tempera-tune lower on it;
CCII)-ihlary nes’i chiefly pres-ent on affected side
Venous ectuisia
Brown, rough &
huuir5-Diffuse areas of passis’e
congestion of reddish
coloration on it IE
Slight pigmentation uun
1 neck; more hair on I lip & cheek; scalp
hairline lower on I;
sweat gland activity
+ on left fiuce
h,iponuta of it neck &
kick; small
telangi-rn’tiasis (in it luck
(if#{233}-au-lait spots on,
I, lower hack & 2nd
small capillary
hem-angiomas on the It
np-per lid & giuhellu
Pronated feet
Reuhuiphiu-atiuun I
kiul-n)e5- & elvis
I)orsuI huuniuhuurScOhlo
S’s
Grip strunger on I.
I Iuanuheul
it convergent straluis-miis, Suhivutionu + ml
it
it breast larger &
more PenduilouS
Penis & scrotum
eni-lunged; hirsuitism; uudenomyosiurcoma of
it adreaal cortex
Mother & maternul gr,un(Imothuer has-c facial ,usvmmetrv;
Pre(’OCiO,i5 eruiptiono uuf
teeth on I., larger & more cariouus
Diluterah ingiuinul
her-nius; it foot 6 tu.es
with 2nd and 3rd
wehhed; shoes R ,ire
15 with, width, 70 1’, I
vaie /7, ssidthu 75 I-;
luoneage in feet isei)uul
(‘uunupenusuturs’
uu,hiu,-S’s
Elevated gu)niCuduutru-pins; bone age 6 yr un
l & St yr on R; sh,urt stutiure
Prenout mire eruption
‘uf
teeth mmii
Both, feet & legs, IQ it ear & tongue
Ihip, leg, & foot Normal
leg
Nuuse, lips. jaw, Retarded
cheek, tongue, &
eur
Cheek, lip, nose, .
-tongue, ear, fron-tal bone, anterior Iof parietal bone.
maxilla, &
man-dible
,
This talule is incomplete and contains only those cases of segmental henuihuypertrophuy, pres-iomisly reported mothe literature asIueniu-ypertrophuy. , ‘
t UE upper extremity; LE lower extremity; +increased.
444
HEMIHYPERTROPHY
TABLE IV
,‘t’oted Side Parts Invoiced Mentality
Birth it CE & IF
-,-tuulhor ‘ez Age
‘l’ruJlut & SIonod,
I
tI 19 yr
\\ iseherg, I931’’ F’ 11 ni(u 1 nio h
I’ueend ( (MC SI I6 yr Birth I.
Total 1)13 37 Si
61F
55 R
301,
Fiu-e, ear, ted ho,
tongue, trunk,
CE, & LE
I lead, (mu-c, ear,
tongue, teeth,, noanditule, aluic
10151, trunk, pels’is & l,E
Retard&’uI
IQ 23
Mental age
3yr 10 mu
Skin
Extensive cutaneouis
nevi
Iluuir duurk brown on
I.; medusa-like venous
channel in It groin;
mottled & bronze-like
& is thin, elastic all
os-er; It silky-like, 1,
dry & scaly;
perspira-tion + on R
Increased
pigmentiu-tion and hypertrophuic
epidermis to the left of
the midline; multiple, vascular-appearing
reddish areas are noted
os-er the body, hands, arms & leg, but none
on the head; scaly
Iurown pigmented
stripes on anterior &
posterior of I, leg;
hair on It reddish,,
finer & sparser
Other Features
Temperature + on It
Scohiosis;
huyperexten-sibihity &
hyj,erflexi-I)ility of IF’s;
tenu-perature is unstalule;
1st & 2nd toes weluhued
Negro; skin tenipera-time + &venous
duuu-tension throughout the R side of the body;
skin temperatuire on
it leg +. Ihydrocele;
testicular atrophy;
hemorrhoids; s-cry
large hands & feet h,u,t
1 foot notably lunger
than R; abnormuul
EEG; ,compensatumry
seoluosis
19
Re-I turuhed
50with, skin
cumphica-tiuuns
REVIEW
ARTICLE
TABLE IV (Continued)
445
etiological hypotheses for hemihypertrophy
that have been postulated in the literature
are listed.
The views expressed herein are those of
the authors and do not necessarily reflect
the views of the U. S. Navy nor the
Depart-ment of Defense.
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