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Hyperthyroidism of the juvenile familial type
HYPERTHYROIDISM
OF THE JUVENILE
FAMILIAL
TYPE*
S. THOMAS GLASSER, M.D.
WILLIAM P. ECKES, M.D., Associ:rtc Attending Surgeon, Flower and Fifth Avenue Hospital; Attending Surgeon, Xletropolitan HospitaI
Associate Assistant
Attending Surgeon, Metropolitan Surgeon, Flower and Fifth Awnuc
Ilospital; 1 luspit;J
AND WALTER L. MERSHEIMER, M.D. Assistant
Attending NEW
Surgeon,
YORK,
Metropolitan
NEW
vergence. normally
URING the past two years our interest in the problem of the juvenile hyperthyroid patient, particuIarIy of the famiIia1 type, has been stimulated by observation of two patients in our hospita1. The famiIia1 aspects and the aIIied endocrine dysfunctions have been of particuIar interest. AIthough these cases may no Ionger be considered rare, they are of such relative infrequent occur-
D
sounds intense; murmurs absent; respiration 24. Recta1 temperature was normal. Breasts mere undeveloped and signs of beginning
survey of this subject has been furnished 63 hlooIten and Bruger’ which makes further summary redundant. \Ve wiI1, therefore, limit our discussion to the saIient features as illustrated by our two patients. KEPOKTS
C.\SE I. hl. h:I. Chart No. 129171. The patient, age seven years, was first admitted on the pediatric service January 31, 1941, at which time there was a history of extreme nervousness, increasing prominence of the eyes, recent nreight loss and excessive appetite for the past file years. Both parents were living and w-ell. One sister (N. I\‘I.) age thirteen, was a diabetic of five years’ duration; (I. M.) age eleven, had rheumatic heart disease; (K. hf.) age ten had been previously hospitalized for a symptomfree benign gorter not operated upon; A. M. (Case II) age six, had a subtotal thyroidectomy for diffuse hyperthyroidism. PhysicaI examination reveaIed a seven-year old white female, well developed but apparently underweight. Marked emotiona instabitity xvas obvious. There \vas definite esophthaImos -eequaI bilaterally, Lid-lag and lack of con-
*
FI-onl
the Dcpartmcnt
of Surgcrv,
New York
The pupils ‘~erc equal and reacted to light and accommodation. The
tonsiIs u-erc slightly hypertrophied and the anterior cervical lymph nodes were moderateI> enlarged. The thyroid was diffusely enlarged, soft and noduIar on palpation, and a marked bruit was present. The heart rate n-as 136,
rence as to merit presentation. A recent excellent comprehensive
CASE
Hospital
YORK
puberty were absent. A fine tremor of the extended hands was noted. At the time of this first admission the pediatric service decided to continue medical therapy which consisted of absoIute bed rest, sedatron, and high caloric diet. On March I, 1041, Lugol’s solution (m 2 T.I.D.) was instituted, and later increased to ( m 5 T.I.D.). This resulted in a slight lowcring of the pulse rate. Surgical consultation a month later noted no improvement. The pulse rate was still elevated (ISO), and the th,yroid gland had increased in size. On April Tth, the basal metabolic rate \vas plus 7. Lugol’s solution was discontinued on April 10th. The pulse rate one week Iater had increased to 170 but dropped to 130 after the patient was placed
on bed rest. At this time Lugol’s solution (m 2 T.I.D.) was again started and one \veek
later was increased to m 3 T.I.D. Thyroid cstract t 5 \vas given daily until June 0, 1941. During this period the basal metabolic rate dropped to minus 18 and it was noted that the thyroid gland had aImost doubled in size and was symmetrical. The patient’s \zeight increased from 43! ,k pounds on admission to
gr.
63 pounds on discharge July 3,
1941,
antI
\V:IS
apparently in good condition. On readmission, October I 7, 194.1, she presented the following symptoms of hyperthyroidism: Exophthalmos \vith confirrnator?
L\sledicaI College, politun I lospital.
291
Flowx
and Fifth
Avcnuc
1lospitals,
:tnd Xlct 10-
292
AmericanJournal of Surgery
Eckes
eye signs, soft diffuse thyroid enIargement, p&e 132 at rest, accentuated heart sounds, bIood pressure 145/65, marked tremor of
FIG.
I. Case I. Preoperative
Avcusr. 1943
et aI.-Hyperthyroidism
appear-
ance.
fingers and tongue, and weight 514/4 pounds. Following consuItation she was accepted for thyroidectomy and transferred to the surgica1 service on November 4, 1941. Preoperative preparation consisted of bed rest, high ca1oric diet, phenobarbital Lugol’s soIution (m IO T.I.D.), vitamin B compIex and increased protein intake oraIIy. The basaI metaboIic rate on November I Ith was pIus 15. Despite continued therapy the pulse rate remained However, the pare1ativeIy high (I 10-130). tient’s weight increased five pounds. On December 1st there was definite signs of improvement. The emotionabIe stabiIity was better and the puIse rate more stabIe. AIthough the maximum puIse rate was 130, a tendency toward a sIower rate was apparent and she continued to gain weight. The puIse pressure showed no tendency to increase, and iodinization had been adequate. By December 3rd the basal metaboIic rate had dropped to pIus g. Cardiac consu1tation at this time showed evidence of marked toxicity but this was thought not to contraindicate surgery. (Fig. I .) On the foIlowing day, cyclopropane-oxygen
anesthesia was administered by way of an intratracheal catheter and a routine coIIar-type incision was empIoyed through which both
FIG. 2. Case
I. Postoperative
appear-
ance.
superior poIes were ligated with silk. This type of incision was decided upon since it was beIieved that it wouId offer a more rapid and thorough means of approach to the problem of Iigating the poles in this highIy toxic patient. The tota eIapsed operating time of eleven minutes apparentIy justified the decision to employ this means of approach. On return from the operating room the pulse rate was I~O--170, of good quaIity and regular. The chiId reacted from anesthesia and became restless, which was controIIed by paraldehyde given per rectum. The oxygen mask was we11 toIerated and aIthough the respirations were shaIIow and thoracic in type there was no evidence of respiratory obstruction or anoxia. Ergotamine $6 cc. was given at q-hour interva1s with LugoI’s soIution m 15. However, the puIse rate, temperature and respiration continued eIevated. The child was motionless, faiIed to respond to stimuIi and the cornea1 reffexes were absent. A venocIysis of 1,000 cc. of IO per cent dextrose in saIine and IO cc. of IO per cent sodium iodide was given. The respirations improved and
Nru
SI.I
OL.
LSI.
t\‘r~
I
Eckes
et al.-Hyperthyroidism
the temperature was controlled with ice packs. On the following morning the patient was given I ,000 cc of I o per cent dextrose in norma saline and IO cc. of IO per cent sodium iodide. Her condition remained unchanged except that she now reacted to stimuli. In the evening a continuation of the present rkgime resulted in a lightening of the coma although the puIse remained very rapid. The patient was placed in an oxygen tent. On December 6th the patient \vas much improved, fully conscious, took fluids we11 by mouth, sat up in bed and ate ice cream and IoIIy-pops. The pulse rate decreased to 130. Marked improvement continued; she took a soft diet, candy, and fluids; the puIse was steadier and slower. The drain was withdrawn and alternate skin clips removed; the incision was healing well. On December 9th all clips were removed; the Lvound nas healing well, and the patient’s general condition was excellent. On December 13th the basal metabolic rate was minus 9. Pulse rate xraried between IOO and 130 and the patient’s condition was satisfactory for further surgery to be limited to hemithyroidectomy. Two days later a right hemithyroidectomy was performed. Microscopic examination of tissue showed marked connective tissue proliferation and numerous focal areas of round cell infiltration. Some of the acini presented Iow papillary projections. The pathologica diagnosis \vas toxic goiter. The patient’s convalescence folIowing this operation was uneventful. Her pulse rate never exceeded 140. The maximum temperaturc was 102’F. on the first postoperative day. On the following day the pulse was 120 and the temperature IOO’F. A striking point was the improvement in the patient’s former surfg diwosition. Her aDDearance became much Iess apprehensive. She’ L,as apparentIy happy and had gained seven pounds. This represented a 20 per cent gain In total body weight since her admission to the surgical service, and a net gain of about 12 per cent since her operation three weeks before. The wound healed well except for a smaI1 granuIating area in the center. AppIication of a IO per cent solution of silver nrtrate removed the exuberant tissue. On January 10, 1942, a small tIuctuant mass about 2 cm. in diameter was noted to the right of the midline. This was assumed to be a sterile abscess due to silk suture irritation,
Incision culture
American
and drainage taken which
Journal
of Surprrv
293
was performed and a proved negative for
micro-organisms. Drainage subsided following the application of suIfathiazole powder. The patient was discharged on February 25th. Further observation of the patient was continued in the out-patient department. On April rlth, she was re-admitted exhibiting little evidence of thyrotoxicosis and in good general health. There was only slight tremor, pulse 106, no apparent improvement in the esophthalmos, marked lid-lag, freely moveabIe scar and several areas of keIoid-like scarring. The Ieft thyroid lobe WIS f)areIy palpable. A tender fluctuant area about I cm. in diameter was noted at the site of the original sterile abscess and on April 23rd this mass was opened under local anesthesia. About 5 cc. of purulent fluid was evacuated along with two pieces of black silk suture material. Despite this procedure the wound continued to drain and tn-o additional pieces of black silk suture were removed on April 29th. On May I rth, sinus tract drainage continued and a small amount of seropurulent and granulation tissue was removed. On May r&th, dkbridement, drainage and curetting of the sinus tract leas performed under general anesthesia. Despite these operative procedures a persistent sinus reformed with continued seropurulent discharge and exuberant granulations. The sinus was probed and found to extend toward the right superior ths_roid pole. Additional black silk suture material 1~8s removed. Th e patrent ’ was discharged to the clinic June I Ith with a persistent silk sinus. (Fig. 2.) Her weight on admission was 643 pounds; bIood pressure 96/70. Her weight on discharge was 6815 pounds; blood pressure rogi$3. A
sIight but steady w-eight gain was noted, and the blood pressure showed little variation. Continued observation of this patient is being carlied out in the endocrine clinic. She has also been examined on repeated occasions at the thyroid committee conference. Despite the complete subsidence of thyrotoxic symptoms, it was the concensus of opinion that further surgerv will be necessar? because of the probable danger of reactivation of the remaining intact lobe. However, in view of the considerable recent operative trauma, it was decided
that
further
intervention
should
be
294
American
Journal
of Surgery
Eckes
et aI.-Hyperthyroidism
deferred and that conservative measures be continued especiahy since the patient and famiIy are co-operative and continued observation will be possible.
AU<.USl, IV45
sinus tachycardia; blood chemistry was normal. After two weeks’ observation the chiId was discharged and referred to the endocrine chnic for foIIow-up. WhiIe under observation
FIG. 3. Case II. Three sisters, ages five and one-haIf years, seven and one-haIf years and thirteen and one-haIf years, respectively.
Five months folIowing operation this patient without further surgery can be considered a normal chiId. She shows a significant improvement in behavior, has lost her former surIy and presents a attitude, weighs 74 pounds marked recession of her original exophthalmos. BasaI metaboIism at this time is minus 17. CASE JI. A. M. Chart No. 112958. The patient, age six years, was admitted on May 8, complaints of tremor, increased with 194% excitability, buIging of the eyes, intolerance to heat, and sweIIing of the neck. She was first admitted October 17, 1941, for a swehing of the neck which was present for three weeks. On this admission there was no evidence of exophthaImos or other symptoms of thyrotoxicosis. There was a diffuse hyperpIasia of the thyroid, the heart was not hyperactive, basaI metabohsm o, eIectrocardiogram showed
during the six months prior to the present admission there had been a gradual development of toxic signs which became progressively worse, and hospitaIization for surgical removal of the thyroid was advised. (Fig. 3.) The patient was thin, pale, very active and an extremely co-operative female. No abnormalities of the head were noted; pupils were equa1 and reacted to light and accommodation. There was slight internal deviation of the left eye (Fig. 4) ; palpebra1 fissures were widened; marked exophthalmos; lid-lag definite; no wrinkIing of the forehead. Ears and nose were normal. The thyroid was enlarged biIaterally (Fig. 5), the right Iobe was Iarger than the left; the isthmus was readiIy outlined. A thriI1 was palpabIe and a bruit was audible over the thyroid. Lung expansion was equal and resonant to percussion; there was vesicular
N, \\ St K,,s\G,,
I.Xl.N<,
L
Eckes
et aI.- -Hyperthvroidism
breathing; the heart rate was 158; bIood pressure 150,/50; the apex was in the sixth intercostal space in the midcIavicuIar line. Cardiac consultation reveaIed heart sounds
FIG. 4. Case II. Preoperative ance.
appear-
hyperactive; no murmurs were present. The abdomen was soft and symmetrical. Liver and spIeen were not paIpabIe. Tremors of the hands were present and a11 reflexes hyperactive. FoIIowing admission, the patient was examined in endocrine and pediatric consultation. The thyroid was diffuseIy enIarged with irregular noduIes throughout. On a four plus scale, the thyroid was enIarged to two or three plus. It was concIuded that the chiId had diffuse t hyrotoxicosis with superimposed toxic adenoma. Surgery was recommended and routine preoperative preparation was instituted. Under this regime the pulse rate ranged 120 and 140, whereas on admission between the puIse rate was over 140. Basal metabolism fel1 from pIus 28, to pIus 4; the patient gained weight, the tremor was less pronounced and the genera1 condition improved markedIy. May z&h, after two weeks of preparation, subtotal thyroidectomy was performed through a coIIar incision. ApproximateIy 36 of the Ieft lobe, 3/4 of the right lobe and the entire isthmus were removed. The child was observed every hour during the day folIowing operation and at no time
A,r,cric;,n.I<,,11 llill(IIS,,I-~C, y
295
any respiratory difiiculty. ‘l‘h( \vas there oxygen mask was we11 tolerated. She reacted from anesthesia in norma time and \vas able to phonate without difficulty. Her temperature
FIG. 5. Cast II. Preoperative ante.
appenr-
rose to IO~‘F., but was readiIy controhed by aIcoho1 sponge baths. The puIse was maintained at about I 16 and respiration 30. On the second postoperative day the temperature was 99.4%., puIse 140, and respirations 40. On the third postoperative day the temperature rose to 104.4%. and remained eIevated in spite of repeated sponge baths. Postoperative atelectasis or pneumonia of the left base was suspected by the pediatric consultant. X-ray examination did not confirm this opinion. Ice bags were pIaced on the extremities and head. There was no evidence of respiratory difficulty. The patient continued to receive 90 per cent oxygen by mask. She was able to take small amounts of fIuid by mouth, incIuding Lugol’s soIution. Ergotomine $1 cc. was administered every three hours. The dressing was removed and the wound inspected. Oxygen inhalations were continued. The child continued to become more aIert and the pulse was of better quality and slower. Fluids were well taken. Dressings were changed and the drain removed; the wound was clean. The further postoperative course was entireIy satisfactory. It was the opinion of the
296
American
Journd
of Surgery
Eckes
AUGUST,1943
et aI.-Hyperthyroidism
endocrine consultant that the patient had suffered a mild postoperative thyroid crisis which apparently was attributable to anoxemia. The surgical service did not completely concur in this opinion. AIthough conceding that there may have been a mild hyperthyroid crisis, this certainly was not attributabIe to an anoxemia since at no time had there been any respiratory difficulty and the patient had been continuousIy receiving go per cent oxygen by mask. The further postoperative course was entirely uneventful; the maximum puIse was 132 with an average puIse of I IO to 120; the temperature was beIow IOO’F., blood pressure I 16/58. The wound heaIed by primary union and the patient was out of bed on the fourth postoperative day and was ambuIatory on the fifth day. On the sixth postoperative day the basal metabolism was minus 4. She was discharged on the fourteenth postoperative day and was referred to the endocrine cIinic for foIIow-up. The pathologica report was as foIIows: ProIiferation of acini HIed with degenerated coJIoid. Some of the acini show low papillary projection. The connective tissue is increased in amount and there are many areas of smaI1 round ceI1 infiltration. Diagnosis: Toxic adenoma of the thyroid. The patient has been closely observed for the past five months. She has gained weight, her puIse averages 80 to 84 and her basa1 metabolism at present is minus 4. She has shown no tendency toward recurrence of her former toxic state. REMARKS
The familial aspect of juvenile hyperthvroidism is illustrated by our two cases, four members of one wfiich incIuded family, three sisters and one cousin. Three
members presented symptoms of hyperthyroidism and one of benign goiter. Of additiona interest is the presence of diabetes meIIitus in another sister and aIso the tendency toward the diabetic state was noted in Case II. This endocrine reIationship has been demonstrated by Steener and Newcomb.* The sugar toIerance curve (in mg. per cent, fasting 8-5, $5 hour 143, I hour 160, I 45 hours 170, 2 hours I IO) in Case II does not appear typical of the thyroid type but is proIonged, apparentIy tending toward the diabetic variety aIthough the fasting bIood sugars are Iow. Case I presented the history of exophthaImos at the age of two years. According to the Iiterature, this is a rare finding. EIIiott3 reported the presence of exophthaImic goiter in his case before one year of age as being “a rea1 medica rarity.” We are in accord with the concensus of opinion that the juveniIe hyperthyroid case is a definite surgical problem for which proIonged medical therapy shouId not be undertaken. This is borne out in our experience with the management of our two patients. REFERENCES I. MOOLTEN, R. R. and BRUGER, M. ExophthaImic goitre of the juveniIe type: a survey of the Iiterature on the famiIia1 aspects of this disease and a report of two addition;1 cases. Arch. Surg., 45: 623-632, 1942. 2. STEINER, M. M. and NEWCOMB, A. L. EnIargement of the thyroid gIand in juvenile patients with diabetes mellitus. Am. J. Dis. Gild., 61: 458-470, 1941. 3. ELLIOTT, P. C. Exophthalmic goitre before one year of age. J. Medial., 6: 204, 1935.
OF THE JUVENILE
FAMILIAL
TYPE*
S. THOMAS GLASSER, M.D.
WILLIAM P. ECKES, M.D., Associ:rtc Attending Surgeon, Flower and Fifth Avenue Hospital; Attending Surgeon, Xletropolitan HospitaI
Associate Assistant
Attending Surgeon, Metropolitan Surgeon, Flower and Fifth Awnuc
Ilospital; 1 luspit;J
AND WALTER L. MERSHEIMER, M.D. Assistant
Attending NEW
Surgeon,
YORK,
Metropolitan
NEW
vergence. normally
URING the past two years our interest in the problem of the juvenile hyperthyroid patient, particuIarIy of the famiIia1 type, has been stimulated by observation of two patients in our hospita1. The famiIia1 aspects and the aIIied endocrine dysfunctions have been of particuIar interest. AIthough these cases may no Ionger be considered rare, they are of such relative infrequent occur-
D
sounds intense; murmurs absent; respiration 24. Recta1 temperature was normal. Breasts mere undeveloped and signs of beginning
survey of this subject has been furnished 63 hlooIten and Bruger’ which makes further summary redundant. \Ve wiI1, therefore, limit our discussion to the saIient features as illustrated by our two patients. KEPOKTS
C.\SE I. hl. h:I. Chart No. 129171. The patient, age seven years, was first admitted on the pediatric service January 31, 1941, at which time there was a history of extreme nervousness, increasing prominence of the eyes, recent nreight loss and excessive appetite for the past file years. Both parents were living and w-ell. One sister (N. I\‘I.) age thirteen, was a diabetic of five years’ duration; (I. M.) age eleven, had rheumatic heart disease; (K. hf.) age ten had been previously hospitalized for a symptomfree benign gorter not operated upon; A. M. (Case II) age six, had a subtotal thyroidectomy for diffuse hyperthyroidism. PhysicaI examination reveaIed a seven-year old white female, well developed but apparently underweight. Marked emotiona instabitity xvas obvious. There \vas definite esophthaImos -eequaI bilaterally, Lid-lag and lack of con-
*
FI-onl
the Dcpartmcnt
of Surgcrv,
New York
The pupils ‘~erc equal and reacted to light and accommodation. The
tonsiIs u-erc slightly hypertrophied and the anterior cervical lymph nodes were moderateI> enlarged. The thyroid was diffusely enlarged, soft and noduIar on palpation, and a marked bruit was present. The heart rate n-as 136,
rence as to merit presentation. A recent excellent comprehensive
CASE
Hospital
YORK
puberty were absent. A fine tremor of the extended hands was noted. At the time of this first admission the pediatric service decided to continue medical therapy which consisted of absoIute bed rest, sedatron, and high caloric diet. On March I, 1041, Lugol’s solution (m 2 T.I.D.) was instituted, and later increased to ( m 5 T.I.D.). This resulted in a slight lowcring of the pulse rate. Surgical consultation a month later noted no improvement. The pulse rate was still elevated (ISO), and the th,yroid gland had increased in size. On April Tth, the basal metabolic rate \vas plus 7. Lugol’s solution was discontinued on April 10th. The pulse rate one week Iater had increased to 170 but dropped to 130 after the patient was placed
on bed rest. At this time Lugol’s solution (m 2 T.I.D.) was again started and one \veek
later was increased to m 3 T.I.D. Thyroid cstract t 5 \vas given daily until June 0, 1941. During this period the basal metabolic rate dropped to minus 18 and it was noted that the thyroid gland had aImost doubled in size and was symmetrical. The patient’s \zeight increased from 43! ,k pounds on admission to
gr.
63 pounds on discharge July 3,
1941,
antI
\V:IS
apparently in good condition. On readmission, October I 7, 194.1, she presented the following symptoms of hyperthyroidism: Exophthalmos \vith confirrnator?
L\sledicaI College, politun I lospital.
291
Flowx
and Fifth
Avcnuc
1lospitals,
:tnd Xlct 10-
292
AmericanJournal of Surgery
Eckes
eye signs, soft diffuse thyroid enIargement, p&e 132 at rest, accentuated heart sounds, bIood pressure 145/65, marked tremor of
FIG.
I. Case I. Preoperative
Avcusr. 1943
et aI.-Hyperthyroidism
appear-
ance.
fingers and tongue, and weight 514/4 pounds. Following consuItation she was accepted for thyroidectomy and transferred to the surgica1 service on November 4, 1941. Preoperative preparation consisted of bed rest, high ca1oric diet, phenobarbital Lugol’s soIution (m IO T.I.D.), vitamin B compIex and increased protein intake oraIIy. The basaI metaboIic rate on November I Ith was pIus 15. Despite continued therapy the pulse rate remained However, the pare1ativeIy high (I 10-130). tient’s weight increased five pounds. On December 1st there was definite signs of improvement. The emotionabIe stabiIity was better and the puIse rate more stabIe. AIthough the maximum puIse rate was 130, a tendency toward a sIower rate was apparent and she continued to gain weight. The puIse pressure showed no tendency to increase, and iodinization had been adequate. By December 3rd the basal metaboIic rate had dropped to pIus g. Cardiac consu1tation at this time showed evidence of marked toxicity but this was thought not to contraindicate surgery. (Fig. I .) On the foIlowing day, cyclopropane-oxygen
anesthesia was administered by way of an intratracheal catheter and a routine coIIar-type incision was empIoyed through which both
FIG. 2. Case
I. Postoperative
appear-
ance.
superior poIes were ligated with silk. This type of incision was decided upon since it was beIieved that it wouId offer a more rapid and thorough means of approach to the problem of Iigating the poles in this highIy toxic patient. The tota eIapsed operating time of eleven minutes apparentIy justified the decision to employ this means of approach. On return from the operating room the pulse rate was I~O--170, of good quaIity and regular. The chiId reacted from anesthesia and became restless, which was controIIed by paraldehyde given per rectum. The oxygen mask was we11 toIerated and aIthough the respirations were shaIIow and thoracic in type there was no evidence of respiratory obstruction or anoxia. Ergotamine $6 cc. was given at q-hour interva1s with LugoI’s soIution m 15. However, the puIse rate, temperature and respiration continued eIevated. The child was motionless, faiIed to respond to stimuIi and the cornea1 reffexes were absent. A venocIysis of 1,000 cc. of IO per cent dextrose in saIine and IO cc. of IO per cent sodium iodide was given. The respirations improved and
Nru
SI.I
OL.
LSI.
t\‘r~
I
Eckes
et al.-Hyperthyroidism
the temperature was controlled with ice packs. On the following morning the patient was given I ,000 cc of I o per cent dextrose in norma saline and IO cc. of IO per cent sodium iodide. Her condition remained unchanged except that she now reacted to stimuli. In the evening a continuation of the present rkgime resulted in a lightening of the coma although the puIse remained very rapid. The patient was placed in an oxygen tent. On December 6th the patient \vas much improved, fully conscious, took fluids we11 by mouth, sat up in bed and ate ice cream and IoIIy-pops. The pulse rate decreased to 130. Marked improvement continued; she took a soft diet, candy, and fluids; the puIse was steadier and slower. The drain was withdrawn and alternate skin clips removed; the incision was healing well. On December 9th all clips were removed; the Lvound nas healing well, and the patient’s general condition was excellent. On December 13th the basal metabolic rate was minus 9. Pulse rate xraried between IOO and 130 and the patient’s condition was satisfactory for further surgery to be limited to hemithyroidectomy. Two days later a right hemithyroidectomy was performed. Microscopic examination of tissue showed marked connective tissue proliferation and numerous focal areas of round cell infiltration. Some of the acini presented Iow papillary projections. The pathologica diagnosis \vas toxic goiter. The patient’s convalescence folIowing this operation was uneventful. Her pulse rate never exceeded 140. The maximum temperaturc was 102’F. on the first postoperative day. On the following day the pulse was 120 and the temperature IOO’F. A striking point was the improvement in the patient’s former surfg diwosition. Her aDDearance became much Iess apprehensive. She’ L,as apparentIy happy and had gained seven pounds. This represented a 20 per cent gain In total body weight since her admission to the surgical service, and a net gain of about 12 per cent since her operation three weeks before. The wound healed well except for a smaI1 granuIating area in the center. AppIication of a IO per cent solution of silver nrtrate removed the exuberant tissue. On January 10, 1942, a small tIuctuant mass about 2 cm. in diameter was noted to the right of the midline. This was assumed to be a sterile abscess due to silk suture irritation,
Incision culture
American
and drainage taken which
Journal
of Surprrv
293
was performed and a proved negative for
micro-organisms. Drainage subsided following the application of suIfathiazole powder. The patient was discharged on February 25th. Further observation of the patient was continued in the out-patient department. On April rlth, she was re-admitted exhibiting little evidence of thyrotoxicosis and in good general health. There was only slight tremor, pulse 106, no apparent improvement in the esophthalmos, marked lid-lag, freely moveabIe scar and several areas of keIoid-like scarring. The Ieft thyroid lobe WIS f)areIy palpable. A tender fluctuant area about I cm. in diameter was noted at the site of the original sterile abscess and on April 23rd this mass was opened under local anesthesia. About 5 cc. of purulent fluid was evacuated along with two pieces of black silk suture material. Despite this procedure the wound continued to drain and tn-o additional pieces of black silk suture were removed on April 29th. On May I rth, sinus tract drainage continued and a small amount of seropurulent and granulation tissue was removed. On May r&th, dkbridement, drainage and curetting of the sinus tract leas performed under general anesthesia. Despite these operative procedures a persistent sinus reformed with continued seropurulent discharge and exuberant granulations. The sinus was probed and found to extend toward the right superior ths_roid pole. Additional black silk suture material 1~8s removed. Th e patrent ’ was discharged to the clinic June I Ith with a persistent silk sinus. (Fig. 2.) Her weight on admission was 643 pounds; bIood pressure 96/70. Her weight on discharge was 6815 pounds; blood pressure rogi$3. A
sIight but steady w-eight gain was noted, and the blood pressure showed little variation. Continued observation of this patient is being carlied out in the endocrine clinic. She has also been examined on repeated occasions at the thyroid committee conference. Despite the complete subsidence of thyrotoxic symptoms, it was the concensus of opinion that further surgerv will be necessar? because of the probable danger of reactivation of the remaining intact lobe. However, in view of the considerable recent operative trauma, it was decided
that
further
intervention
should
be
294
American
Journal
of Surgery
Eckes
et aI.-Hyperthyroidism
deferred and that conservative measures be continued especiahy since the patient and famiIy are co-operative and continued observation will be possible.
AU<.USl, IV45
sinus tachycardia; blood chemistry was normal. After two weeks’ observation the chiId was discharged and referred to the endocrine chnic for foIIow-up. WhiIe under observation
FIG. 3. Case II. Three sisters, ages five and one-haIf years, seven and one-haIf years and thirteen and one-haIf years, respectively.
Five months folIowing operation this patient without further surgery can be considered a normal chiId. She shows a significant improvement in behavior, has lost her former surIy and presents a attitude, weighs 74 pounds marked recession of her original exophthalmos. BasaI metaboIism at this time is minus 17. CASE JI. A. M. Chart No. 112958. The patient, age six years, was admitted on May 8, complaints of tremor, increased with 194% excitability, buIging of the eyes, intolerance to heat, and sweIIing of the neck. She was first admitted October 17, 1941, for a swehing of the neck which was present for three weeks. On this admission there was no evidence of exophthaImos or other symptoms of thyrotoxicosis. There was a diffuse hyperpIasia of the thyroid, the heart was not hyperactive, basaI metabohsm o, eIectrocardiogram showed
during the six months prior to the present admission there had been a gradual development of toxic signs which became progressively worse, and hospitaIization for surgical removal of the thyroid was advised. (Fig. 3.) The patient was thin, pale, very active and an extremely co-operative female. No abnormalities of the head were noted; pupils were equa1 and reacted to light and accommodation. There was slight internal deviation of the left eye (Fig. 4) ; palpebra1 fissures were widened; marked exophthalmos; lid-lag definite; no wrinkIing of the forehead. Ears and nose were normal. The thyroid was enlarged biIaterally (Fig. 5), the right Iobe was Iarger than the left; the isthmus was readiIy outlined. A thriI1 was palpabIe and a bruit was audible over the thyroid. Lung expansion was equal and resonant to percussion; there was vesicular
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breathing; the heart rate was 158; bIood pressure 150,/50; the apex was in the sixth intercostal space in the midcIavicuIar line. Cardiac consultation reveaIed heart sounds
FIG. 4. Case II. Preoperative ance.
appear-
hyperactive; no murmurs were present. The abdomen was soft and symmetrical. Liver and spIeen were not paIpabIe. Tremors of the hands were present and a11 reflexes hyperactive. FoIIowing admission, the patient was examined in endocrine and pediatric consultation. The thyroid was diffuseIy enIarged with irregular noduIes throughout. On a four plus scale, the thyroid was enIarged to two or three plus. It was concIuded that the chiId had diffuse t hyrotoxicosis with superimposed toxic adenoma. Surgery was recommended and routine preoperative preparation was instituted. Under this regime the pulse rate ranged 120 and 140, whereas on admission between the puIse rate was over 140. Basal metabolism fel1 from pIus 28, to pIus 4; the patient gained weight, the tremor was less pronounced and the genera1 condition improved markedIy. May z&h, after two weeks of preparation, subtotal thyroidectomy was performed through a coIIar incision. ApproximateIy 36 of the Ieft lobe, 3/4 of the right lobe and the entire isthmus were removed. The child was observed every hour during the day folIowing operation and at no time
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any respiratory difiiculty. ‘l‘h( \vas there oxygen mask was we11 tolerated. She reacted from anesthesia in norma time and \vas able to phonate without difficulty. Her temperature
FIG. 5. Cast II. Preoperative ante.
appenr-
rose to IO~‘F., but was readiIy controhed by aIcoho1 sponge baths. The puIse was maintained at about I 16 and respiration 30. On the second postoperative day the temperature was 99.4%., puIse 140, and respirations 40. On the third postoperative day the temperature rose to 104.4%. and remained eIevated in spite of repeated sponge baths. Postoperative atelectasis or pneumonia of the left base was suspected by the pediatric consultant. X-ray examination did not confirm this opinion. Ice bags were pIaced on the extremities and head. There was no evidence of respiratory difficulty. The patient continued to receive 90 per cent oxygen by mask. She was able to take small amounts of fIuid by mouth, incIuding Lugol’s soIution. Ergotomine $1 cc. was administered every three hours. The dressing was removed and the wound inspected. Oxygen inhalations were continued. The child continued to become more aIert and the pulse was of better quality and slower. Fluids were well taken. Dressings were changed and the drain removed; the wound was clean. The further postoperative course was entireIy satisfactory. It was the opinion of the
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endocrine consultant that the patient had suffered a mild postoperative thyroid crisis which apparently was attributable to anoxemia. The surgical service did not completely concur in this opinion. AIthough conceding that there may have been a mild hyperthyroid crisis, this certainly was not attributabIe to an anoxemia since at no time had there been any respiratory difficulty and the patient had been continuousIy receiving go per cent oxygen by mask. The further postoperative course was entirely uneventful; the maximum puIse was 132 with an average puIse of I IO to 120; the temperature was beIow IOO’F., blood pressure I 16/58. The wound heaIed by primary union and the patient was out of bed on the fourth postoperative day and was ambuIatory on the fifth day. On the sixth postoperative day the basal metabolism was minus 4. She was discharged on the fourteenth postoperative day and was referred to the endocrine cIinic for foIIow-up. The pathologica report was as foIIows: ProIiferation of acini HIed with degenerated coJIoid. Some of the acini show low papillary projection. The connective tissue is increased in amount and there are many areas of smaI1 round ceI1 infiltration. Diagnosis: Toxic adenoma of the thyroid. The patient has been closely observed for the past five months. She has gained weight, her puIse averages 80 to 84 and her basa1 metabolism at present is minus 4. She has shown no tendency toward recurrence of her former toxic state. REMARKS
The familial aspect of juvenile hyperthvroidism is illustrated by our two cases, four members of one wfiich incIuded family, three sisters and one cousin. Three
members presented symptoms of hyperthyroidism and one of benign goiter. Of additiona interest is the presence of diabetes meIIitus in another sister and aIso the tendency toward the diabetic state was noted in Case II. This endocrine reIationship has been demonstrated by Steener and Newcomb.* The sugar toIerance curve (in mg. per cent, fasting 8-5, $5 hour 143, I hour 160, I 45 hours 170, 2 hours I IO) in Case II does not appear typical of the thyroid type but is proIonged, apparentIy tending toward the diabetic variety aIthough the fasting bIood sugars are Iow. Case I presented the history of exophthaImos at the age of two years. According to the Iiterature, this is a rare finding. EIIiott3 reported the presence of exophthaImic goiter in his case before one year of age as being “a rea1 medica rarity.” We are in accord with the concensus of opinion that the juveniIe hyperthyroid case is a definite surgical problem for which proIonged medical therapy shouId not be undertaken. This is borne out in our experience with the management of our two patients. REFERENCES I. MOOLTEN, R. R. and BRUGER, M. ExophthaImic goitre of the juveniIe type: a survey of the Iiterature on the famiIia1 aspects of this disease and a report of two addition;1 cases. Arch. Surg., 45: 623-632, 1942. 2. STEINER, M. M. and NEWCOMB, A. L. EnIargement of the thyroid gIand in juvenile patients with diabetes mellitus. Am. J. Dis. Gild., 61: 458-470, 1941. 3. ELLIOTT, P. C. Exophthalmic goitre before one year of age. J. Medial., 6: 204, 1935.