( Received
August 26; revision accepted for publication December 10, 1968.)This study was supported in part by a Research Grant from the National Institute of Child Health and
Human
Development
(
USPHS 2-R01-HD-00999-04).P.E.F. is the recipient of Research Career Development Award
# 1K3-HD-34,
547-02
from the National Institute of Child Health and Human Development.ADDRESS: 4800 Sand Point Way N.E., Seattle, Washington 98105.
PEDIATRICS, Vol. 43, No. 5, May 1969
FAMILIAL
PITUITARY
DWARFISM
ASSOCIATED
WITH
AN
ABNORMAL
SELLA
TURCICA
Pierre
E. Ferrier,
M.D.,
and
E. Franklin
Stone,
Jr., M.D.
From the Departnent of Pediatrics, University of Washington, and the Children’s Orthopedic Hospital
and Medical Center (Joan Judson Research Laboratory and Retarded Children’s Clinic),
Seattle, Washington
ABSTRACT. Two non-twin sisters issued from healthy non-consanguineous parents of normal stat-ure demonstrated severe growth failure,
hypoglyce-mia, and evidence of deficiency of growth
hor-monc, thyroid stimulating hormone
( TSH
), andadrenocorticotropic hormone (ACTH
)
; in addition,they both had a very slnall sella turcica located in
a sphenoid bone of abnormal morphology. This
form of familial pituitary dwarfism is different from the genetic forms previously described. Pediatrics,
43:858, 1969, FAMILIAL PITUITARY DWARFISM,
SELLA TURCICA, HYPOPITUITARISM.
S
EVERAL FORMSof
familial
or
genetic
pi-tuitary
dwarfism
now
have
been
recognized.’ They are characterized by
var-ious
pituitary
hormone
deficiencies,
but
in
none
of
them
is the
sella
turcica
strikingly
abnormal.
The
object
of
this
paper
is to
re-port
the
association
of
severe
growth
fail-ure,
inadequate
pituitary
functions,
and
ex-treme
hypoplasia
of the
sella
turcica
in
twosisters. The familial nature of this
associa-tion
is thought
to be
unique.
METHODS
The
charts
of
the
Harvard
School
of
Public
Health
were
used
for
plotting
growth.’
Bone
maturation
was
estimated
by
use
of
the
tables
of
Greulich
and
Pyle’
and
Wilkins
and
co-workers.4
Urinary
17-hydroxvcorticosteroids were measured
according
to
the
method
of Glenn
and
Nel-son.’
The
semiquantitative
method
of
Dorfman
and
Steiness7
was
utilized
for
the
detection
of
acid
mucopolysaccharides
in
the
urine.
Plasma
growth
hormone
was
mea-sured
by
the
radioimmunoassay
technique
of
Schalch
and
Parker.8
Blood
sugar
was
de-termined
by
means
of
a
glucose-oxidase
method.’
Chromosomal
analyses
of
periph-eral
blood
leukocytes
were
performed
by
a
modification
of
the
technique
of
Moorhead
and co-workers.’#{176}
Patient 1
CASE
REPORTS
This 11-year-old, white girl was initially re-ferred to the Retarded Children’s Clinic at 6 years of age because of her slow development. She
was
the
oldest
child
of
the family, whichcon-sisted of the 35-year-old mother, the 39-year-old father, a 10-year-old sister
(
Patient 2) and a3-year-old sister. Parents were third generation
Americans, and unrelated; the father’s ancestors
were French, and the mother’s were Flemish
( Belgium).
There
was no family history ofex-cessively short stature. The mother was 5 ft, 3 in. and the father was 5 ft, 10 in. The youngest sister was growing along the 25th percentile according to the Vickers and Stuart chart.’ The patient was
born at 42 weeks of gestation with a weight of
8 lb, ii oz and a length of 21 in. Labor, delivery,
and immediate neonatal period were
uncompli-cated. However, at 10 weeks of age a physician
suspected a thyroid deficiency because she was
“sleepy” and she was treated with dessicated
thyroid for 8 months. This then was discontinued because she was thought to have received
“maxi-mum
benefit
from
the
treatment.”
She
rolled
overat 5 months, sat at 6 months, and crawled at 11
months. From about 12 months of age her rate of
growth and development was noted to slow down.
TABLE I
BLOOD SUGAR AND GRowTH HORMONE DATA
Blood Sugar (my, 100 nil). 36-hr Fast
Pa-lent
8 lg:3O 4
,
n 4 8AM. P.M. P.M. P.M. AM. AM. AM.
71 68 51 47 45 36 19
10 30 45 60 90 110 3.1
I.9 3.0 6.0
9.7
1.5
ii
I
MONTHSliii
LNTI1 ‘rri , 1 I ‘* Is 1* C70 Coo LINTH HIIHT C5O II
h
,j
#{174}OLDU SIlTU
346
8
‘1
Fic. 1. Growth curves of the
two
patients.years of age she had fallen below the third
per-centile for height. At the age of 6 years psycho-logical testing with the Wechsler Intelligence Scale for Children ( WISC) revealed a verbal scale
I.Q. of 65, a performance scale I.Q. of 54, and a full
scale I.Q. of 56. At this time the value of the serum
PBI was 3.1 eg/100 ml, the bone age was read as
2 years, and an electroencephalogram was con-sidered slightly abnormal in view of the predomi-nant frequency of 4 cycles per second ( slow for
this age). The excretion of aminoacids in this
urine was not excessive. A buccal smear showed a normal female pattern.
Her growth pattern continued to follow a curve below the third percentile, as shown in Figure 1.
At 72 years of age, she had a 4 month history of
repeated episodes of fatigue, vomiting, abdominal pains, and headaches at 1- to 2-week intervals. This was not explored further at the time. Repeat
psychological evaluation at 82 years, with
the
WISC,
revealed
a verbal
scale
I.Q. of 57, a
perform-ance scale I.Q. of 39, and a full scale I.Q. of 43, consistent with functioning in the moderately re-tarded range. At 1 1 years of age she was still enu-retic; she was reading at a pre-primer level and
doing
some simple counting. She also was reportedto have experienced several episodes of motor
weakness during the previous 4 months, usually in the afternoon, characterized by an initial pallor
fol-lowed by poor coordination and staggering gait.
Occasionally there were complaints of associated headache and poorly’ localized pain elsewhere. The episodes usually subsided after a brief nap or rest, following which she usually manifested a ravenous hunger. Feeding during the initial stages of an epi-sode often seemed to abort completion of the full cycle.
Physical examination revealed a very small, well
proportioned, well nourished 11-year-old girl with
a height ( 106 cm) and weight ( 17.2 kg)
corre-sponding to the 50th percentile for 4 years ( Fig. 2 )
.
Span was 97 cm and the lower segment was 55cm. Head circumference was 50 cm. Variable left
internal strabismus, slight epicanthal folds, and clinodactyly of the fifth fingers were noted. There
was no evidence of pubertal development, and the
rest of the physical examination was not remark-able. The following laboratory studies were ob-tamed.
Blood Sugar Plasma Growth
(mg,’lO() ml) Hormone (mzg/ml)
Control 61 3.9
18-hrfast 38 3.5
Arginine Stirnul.olion Test
Time Pla:nza Growth B1o4 Sugar
(mm) Hormone (mig/ml) (mg/100 ml)
0 4.1 .5.5
860
‘l’;flLE II
tTulNAuv 17-uI’n1IcxveoltTIcosr:lioIDs (sio/4 iiit)
Metyrajx)ne
. Pre- ,
Post-I atient (.O iiig/kg
iiieti/raJx)ne
j
j.)
metyrapune1 1.47 1.61 1.15
‘z (1.66 1.3’2 1.16
RADIOGRAPHS : Examination of multiple ossifica-tim centers revealed a bone age of 3 to 4 years,
and examination of dental radiographs revealed a
tlental age of 8 to 9 years. Lateral views of the spine showed slight ballooning of most of the in-tervertebral spaces, with some increase in the
ks’-1)hOSi5 of the thoracic region and in the lordosis of
the cervical region. Examination of the skull by
conventional views showed no depression in the
usual location of the sella turcica and an exces-sively thick and prominent dorsum sellae
( Fig.
3).Sagittal tomograms of the skull revealed a very
small, round, radiolucent area
(
5 mm in diameter) within tile sphenoid bone, anterior to the bulky (lorsunl sellae( Fig.
4)
. The anterior chinoids only could be identified. A series of frontal planetomo-grams through the sphenoid bone were obtained at
1 mm intervals, using a Massiot-Phillips “poly-tome.” The floor of the sella was demonstrated this way to be very shallow
( Fig.
5),
in keeping with the depth observed on lateral tomograms ( Fig. 4).LABORATORY DATA : Several determinations of
the serum protein-bound iodine gave the following values: 4.6, 3.1, and 3.9 ag/100 ml. Oral
admin-100 cm
1%1
I
I
Fic. 2. Rig/it, Patient 1 (older sister) at the age of 11 years. Left, Patient 2
FIG. 3. Skull radiograph of Patient 1. Chronologi-cal age, 1 1 years. Dental age, 8ll years. istration of a test close of 1131 resulted in a 7.7%
uptake by tile thyroid gland, both at 2 and 24
hours.
Following
two
injections
of 10 units ofthyroid-stimulating hormone
(
TSH ) intramuscu-larly at 24-hour intervals, the 1131 uptake by thethyroid
rose
to 14% at 2 hours and 26% at 24 hours.Scanning of the neck showed the thyroid gland to
he in its normal location. Following an overnight
fast, the blood sugar at 8 AM. was 72 mg/100 ml.
Fast then was continued for 24 hours and the
blood sugar progressively decreased to reach a
nlinimum level of 29 mg/100 ml at 8 AM. the next morning
(
Table I). The pituitary-adrenal axis wastested in the following manner: 30 mg/kg of
metyrapone
( Metopirone
)
was given intravenously in a small volume of saline over a 4-hour period.This did not cause a significant increase in the
urinary excretion of 17-hydroxycorticosteroids
(
Ta-ble II). A test for acid mucopolysaccharides in the urine did not reveal an excessive excretion.Chro-mosome analysis from peripheral blood
leuko-cytes showed a normal 46,XX constitution.
Patient 2
The 10-year-old sister of Patient 1 was seen mi-tially in the Retarded Children’s Clinic along with her sister, also because of slow development. She was the product of the mother’s second pregnancy. She was born at term, weighed 7 lb, 8 oz, and was 21 in. long. Pregnancy, labor, and delivery were free of complications. The longitudinal growth of
this
child
also is represented in Fig. 1. By 1 yearof age her length was below the third percentile,
and by the age of 10 years her height
corre-sponded to the 50th percentile for a 53i-year-old
girl. Early development appeared normal;
how-ever, she did not walk until 19 months, use spoken words until 2 years, or use sentences until 4 years.
At
the
age of 10 years she was enrolled in a“trainable” class in the special education program. She continued to experience nocturnal enuresis and constipation. At 10 years her measurements were:
height, 110 cm; span, 106 cm; lower segment, 52
cm; head circumference, 48.5 cm; and weight,
18.3 kg. Except for internal strabismus and mild myopia, there were no other somatic anomalies. None of the secondary sex characteristics was de-veloped ( Fig. 2 ). On psychological testing with
the
Stanford-Binet
intelligence
scale
(form
L-M)she was attributed an I.Q. of 39. The qualitative
aspects of her performance were thought to
sug-gest “cortical dysfunctioning.”
RADIOGRAPHS : Bone age, as interpreted from
multiple centers, was 4 years. Dental age was 9 to 10 years. Skull radiographs at 43i years and again at 93 years revealed an anomaly similar to that of her older sister: an extremely shallow sella turcica making a slight depression on the upper surface of a sphenoid bone of unusual thickness. The dorsum
sellae was particularly thick and prominent (Fig. 6).
LABORATORY DATA: A buccal smear was
posi-tive for sex chromatin, and chromosome analysis showed a normal 46,XX complement. The serum
protein-bound iodine was 4.5 eg/100 ml on
two
occasions. J131 uptake by the thyroid was 9% at 2
and 11% at 24 hours. After intramuscular admin-istration of 10 units of TSH on two consecutive days, the uptake values were 15% and 21, respec-tively. A scan of the neck showed the gland to be in
its
normal location. Urinary17-hydroxycorti-FIG. 4. Sagittal tomogram of the skull of Patient 1, Note the very small sella and the massive bony
862
FIG. 5. Frontal tomograms of skull of Patient 1.
‘fop, section through tile middle of the seila,
sIloss’-ing a slight depression of the floor ( arrows ).
Bot-toni, Se(’tiOll more anteriorly’ tilall top, showing the
elevation of the floor ( arrosvs ). The anterior
clinoicls, ill section, are visible laterally.
costeroi(i excretion \vis 0.66 nig per 24 hrs. Nh’-tvrapolle stillRhlatioll, 5tithS 30 mg/kg of the drug
(t(hluinistered intravenously in a small volume of sahille over a 4-hour period, failed to produce a significailt incredse in this excretion ( Table II). After au overnight fast, the blood sugar value was (ii mg/100 ml; at this time the 1)lasma growth hor-IT1OI1C l(’V(i was 3.9 niag/nli. Fasting then was
prolonged for 18 more hours, at which time the
1)100(1 sugar was 38 mg/100 ml and the plasma
growth hormoiie was 3.1 mug/mi. Thus, there was
110 rise iii tile I)lasna growth hormone despite a renlarkal)iv lOW 1)100(1 sugar. The -apacitv to re-ledsc growth hormone was tested further by argi-ICICle stinuilation i)y’ using 0.5 gm/kg of arginine lllOnoChlOride#{176} as a 10% solution, administered O%P :30 millilteS. The values for blood sugar and plasma growth hormone during and after stimula-tion are shown Ill Table I. A peak growth
hor-mone ‘alue of 9.7 m,zg/ml was observed at 90
minutes. A semiquantitative determination did not
* Kindl’ 5Il)plied for investigative purposes by
11.
J.
Prentice, M.D., Cutter Laboratories, Berkeley, California.reveal an excess of acid Illtlcopohvsacchlarides ill the urine.
Skull Radiographs of Other Family Members
‘Fhe niother and tile tllird sistcr each had a nor-hid1 ap)earing sella turdca. lhlc father had a nor-Illai-5iZe(l
sella
svith i)riclging of the clinoid pro-((55e5.DISCUSSION
Evidence of Hypopituitarism
The
tvo
sisters
are
l)elievcd
to
(lemon-strate evidence of hypopituitarism. Their
l)irth weights and lengths were normal, l)ut
growth
failure became evident early inin-fancy and Persiste(l during the following ‘ears. Lal)oratory CVi(lCflCC of liypoglvcc-cilia OIl fastilig s’as ol)taiIlc(l in 1)0th; ifl
ad-dition, one had symptoms suggestive of
spontaneous
hypoglycemia.
Poor
ability
to
maintain
a normal
blood
sugar level is notuncommon in pituitary (lwarfism.’1 In the
younger sister, prolonged fasting and
subse-quent hypoglycemia failed to cause an
in-crease in plasma growth hormone
concen-tration.
An
increase
is expected
in
normal
subjects,- and its absence is consi(lcred
by
some investigators as a good criterion for
ppril3
Arginine
stimulation
of
growth hormone release has not been fully
evaluated in
children
vet.
The
highest
plasma value of 9.7 m.g/ml observed after
arginine infusion in the younger sister is
relatively
low,l36
hut it clearly indicatesthat some growth hormone could be
re-leased into the circulation following this
I)artic1lar
type
of stimulation.
The
PBI
values
were
within normallim-its
or
just
below,
as
they
most
often
are
in1)ituitary dvarfism. The values for 1131 up_
take
by
the
thyroid
were
low
and
werecor-rected
by
administration
of
TSH,
a finding
suggestive
of
TSH
deficiency.
The
marked
retardation
in bone
maturation
was
in
keep-ing
with
hypopituitarism.
Finally,
the
low
excretion
of
17-hydroxycorticosteroids
and
the
lack
of
response
to
metyrapone
block-ade
in both
patients
constituted
evidence
of
Fie. 6. Skull radiograph of Patient 2. Very shal-low sella turcica and thick clorsulll seilae. Chrono-logical age, 10 years. Dental age, 9 to 10 cars.
Small Sella Turcica
The
difficulties
of
estimating
the
volume
of the sella turcica from radiographs have
been
demonstrated
in
the
studies
of
Silverman’8 in children and of Fisher and
Di
Chiro’#{176}in
adults
and
children.
The
pi-tuitary fossae
of tile
twO patients presented here had a sagittal area well below thenor-mal values established by Silverman’8 for
girls of this age or even of this height age.
On
the
basis
of radiographic
measurements,
Fisher and Di Chiro’#{176} estimated that the sella turcica was small in 18 of 28 hypopi-tuitary children less than 15 years of age.
The
finding
was
thought
to
be
significant,
for
no
example
of
a small
sella
was
found
by
these authors in patients with dwarfism associated with achondroplasia, hypothy-roidism or gonadal dysgenesis. A small sellawas
found
in
10 of 74 patients
with
“dwarf-ism” of unknown etiology. The finding of a
small sella in dwarfed individuals is
there-fore
suggestive,
but
not
specific,
of pituitary
dysfunction. An apparently small sella
tur-cica associated with growth hormone
defi-ciency has been observed in two unrelated patients
by
Frances, et 20 These patientshad
fusion defects of the face(
cleftlip-pal-ate
)
.The
combination
of an
abnormal
sella
with midline facial defects has been
ob-served in cases of D1 trisomy.’1 These
pa-tients had a shallow and ill-formed sella but
a
normal )ituitary. By contrast, illother
pa-tielits with fusion defects of the face’
and
in
certain
cases of iioloprosencephal,’ Cthe
l)itllitarv gland
was
ai)sent, hut the 1)ituitarv fossa was normally developed.Familial Pituitary Deficiency
Different sorts of familial or genetic
pi-Rotary dwarfism flOW
have
been
isolated.Rimoin, et 25
and
Seip,
et a!.’ havestud-ied a form of familial pituitary dwarfism
characterized I)y isolated growth
hor-Iflolle deficiency
(
sexual ateliosis”)
. Thesedwarfs eventually develop sexually, and re-produce; and, postpartum lactation occurs
in the females. The sella turcica is of nor-ma! size in proportion to the skull.’”
Pedi-gree studies support an autosomal recessive
inheritance.
In
other
instances
of
probably
recessive pituitary dwarfism,’ ‘‘ sexual
de-velopment does not take place and a
defi-ciency of several trophic hormones of the
pituitary seems to IJe llresent.
The
sella
tur-cica appeared normal radiologically in the
members of at least one such family.2
Iii
yet another form of genetic dwarfism, the
affected subjects seem to be able to
pro-duce immunoreactive growth hormone but
either suffer from a peripheral, non-respon-siveness to this product or tile product itself
is devoid
of
growth
promoting
activity,
perhaps due to a molecular mutation.
End-organ non-responsiveness seems to he
responsible for the short stature of
the
African
pygmies.’1
Apituitarism
(
absent
gland)
has l)ee:1verified
at
autopsy
in
One patient whosenon-twin sister seemed to be similarly
affected.” Dwarfism and hypoplasia of all
existing endocrine glands were extreme in
this patieiit, who died at 17 years of age.
The sella turcica
vas
of
normal
size
aIl(lshape, despite the complete absence of tile hypophysis.
CONCLUSION
The
syndrome
exhibited
by
the
two
one
of the
known
familial
forms
of pituitary
dwarfism
as
listed
here.
It
is characterized
by
an
excessively
small
sella
turcica
located
in
a sphenoid
bone
of
abnormal
morphol-ogy
and
by
deficient
production
of multiple
pituitary
hormones,
including
growth
hor-mone.
Mental
retardation,
perhaps
secon-dary
to
recurrent
hypoglycemia,
is
also
present.
An
autosomal
recessive
inheritance
is possible.
SUMMARY
An
11-year-old
girl
and
her
10-year-old
sister
presented
the
following
characteris-tics: severe growth
failure
since
early
in-fancy;
a
tendency
to
hypoglycemia;
poor
production
of
growth
hormone,
TSH,
and
ACTH;
marked
retardation
in skeletal
mat-uration;
and
an
excessively
small
sella
turcica
with
abnormal
sphenoid
bone
mor-phology.
Mental
retardation,
moderately
se-vere,
was
perhaps
secondary
to
recurrent
hypoglycemia.
This
syndrome
is believed
to
represent
a hitherto
unrecognized
form
of
genetically determined pituitary dwarfism.
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0. : Hereditary pituitary dwarfism with
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J.
Pediat., 72:81, 1968. 25. Rimoin, D. L., Merimee, T.J., and
McKusick,V. A. : Growth-hormone deficiency in man: an isolated, recessively inherited defect. Sci-ence, 152:1635, 1966.
26. McKusick, V. A. : Primordial dwarfism and ec-topia ientis. Amer.
J.
Hum. Genet., 7:189, 1955.27. Seip, NI. : Personal communication, 1968. 28. Trygstad, 0., and Seip, M. : Hereditary
pitui-tary dwarfism treated with human growth
hormone. Acta Paediat. Scand., 53:527, 1964. 29. Bierich,
J.
R. : On genetically determinedpitui-tary dwarfism. (Abst. ) Acta Endocr.
(Suppl. 45), 89:27, 1964.
30. 1.aron, Z., Pertselan, A., and Mannlieimer, S.: Genetic pituitary (lWarflsm with high sertlin concentration of growth hormone: a new
in-born error of metabolism? Israel
J.
Med.
Sci., 2:152, 1966.
31. Merimee, T.
J.,
Rimoin, D. L., Cavalli-Sforza,L. C., Rabinowitz, D., and McKusick, V. A.:
Metabolic effects of human growth hormone in the African pygmy. Lancet, 2: 194, 1968. 32. Steiner, M. M., and Boggs,
J.
D. : Absence ofpituitary
gland,
hypothyroidism,hypoadre-nalism, and hypogonadism in a 17-year-old dwarf.
J. Clin.
Endocr.,
25: 1591, 1965.Acknowledgment
We are indebted to Dr. Charles C. Gale, Dc-partment of Physiology and Biophysics, for the growth hormone immunoassays, and to Dr. Byron H. Ward, Children’s Orthopedic Hospital and Medical Center, and Dr. Paul S. Paulson, Provi-dence Hospital, Seattle, who performed the radio-logical studies.
JACOB
ABBOTF
(I 803- I 879), AUTHOR
OF THE MOST
FAMOUS
BOOK
ON
CHILD
REARING
OF THE
1870’S, DISCUSSES
CORPORAL
PUNISHMENT
OF
CHILDREN
Jacob Abbott wrote
the
most
widely
readbook
about
child
nurture
in
the
decade
just
after
the
Civil
War.
His
two
principal
themes
were:
the
absolute
need
for
parental
authority
over the child “without violence or anger,” and “right development of the child’s character . .
irs harmony with the structure and characteris-tics of the juvenile mind.” If the child did not bow to parental authority, Abbott advised par-ents of the need for corporal punishment:
The parental authority must, therefore, be es-tablished-by gentle means, if possible-but it must by all means be established, and be firmly main-tamed. If you cannot govern your child without corporal punishment, it is better to resort to it than not to govern him at all. Taking a wide view of the field, I think there may be several cases in which a resort to the infliction of physical pain as
the only available means of establishing authority may be the only alternative.
. . .
Complete,
abso-lute, unquestioned authority can often be obtainedmost forcefully by this form of punishment
.
manoeuvre and artifice and reason and affection ( are ) a sandy foundation for establishing parental authority that came from
God
and nature.1As harsh
as this
advice
may
seem
today,
Ab-bott
was
convinced
that
his
suggestions
about
“gentler” controls offered proof and
strength-ened
belief
in the
child’s
rationality
or
tracta-bility.
NOTED BY T.E.C., JR., M.D.
REFERENCE