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Sheehan’s Syndrome Following Proved and Treated Afibrinogenemia*
SHEEHA..l\.~'S SW~DROME
FOLLOWING PROVED AND TREATED AFIBRINOGENEMIA* A Case Report EDWIN
M.
GOLD,
M.D.,
AND SAMUEL LIBRACH,
M.D.,
N. Y.
BROOKLYN,
(From the Department of Obstetrics and Gynecology, Jeu'ish Hospital of Brooklyn and the State University of New York, College of Medicine at New York City)
P
OSTPARTUM hemorrhage and shock have been described preceding the development of postpartum panhypopituitarism. Simmonds/ in 1914, re-
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Sheehan • • in a series of papers beginning in 1937 greatly clarified this subject. From observations made in a large maternity hospital in Glasgow, he showed that areas of pituitary necrosis of varying size are commonly found in women who die during the puerperium after a delivery associated with postpartum hemorrhage. He was satisfied that infarction was due to marked ischemia of the pituitary with resultant necrosis. A recent monograph by Farquharson5 gives an excellent discussion of the clinical aspects of insufficiency of the adenohypophysis following complications of pregnancy and other conditions. Review of the literature, to the best of our knowledge, has failed to disclose any report of the development of Sheehan's syndrome following an episode of proved and successfully treated afibrinogenemia. The following case report, we feel, exemplifies such a chain of events. 2
8
Case Report The patient, a 31-year-old white para ii-o-o-ii, made five visits to the prenatal clinic of the Jewish Hospital of Brooklyn, after the twentieth week of gestation. Her antepartum course and laboratory findings were within normal limits. At term the patient was admitted to the hospital at 10:30 P.M., on June 17, 1955, in early labor with mild contractions occurring every eight minutes. The cervix was effaced, 2 em. dilated, and the vertex presented at minus one station. Fifteen minutes after admission, while the enema was being expelled, the membranes ruptured spontaneously and strong uterine contractions ensued at two- to three-minute intervals; the patient was now given sedation with Demerol, 50 mg., scopolamine, 0.2 mg., and Benadryl, 50 mg., intramuscularly. By 11:00 P.M. (one-half hour after admission) the cervix was 7 em. dilated. At 11:10 P.M. a tetanic uterine contraction which lasted almost five minutes was noted, and the uterus seemed to be hypertonic until delivery at 11:20 P.M. During this ten-minute interval the patient was transferred to the delivery room, a pudendal block was performed with 50 c.c. of 1 per cent procaine, and, because of difficulty in obtaining the fetal heart tones, delivery was easily effected by low •Presented at a meeting of the Brooklyn Gynecological Society on May lG, 1956.
190
Volume 74 Number I
SHEEHAN'S SYNDROME FOLLOWING AFIBRINOGENEMIA
191
forceps under supplemental nitrous oxide-oxygen inhalation anesthesia. A moderately de· pressed 3,090 gram female infant, born with two tight loops of cord about the neck, responded to simple resuscitative measures within three minutes. The placenta was delivered spontaneously after a three-minute third stage and showed no abnormalities or evidences of premature separation. Methergine, 0.2 mg., was given intramuscularly at this time. Ten minutes after delivery, or one hour after admission, when the fundus was apparently well contracted, a gush of blood and small clots, estimated at 300 c.c., issued forth from the birth canal. Pitocin was administered intramuscularly and by intravenous drip. Examination of the birth canal showed that the blood was coming from the uterine cavity. By midnight, 40 minutes following delivery, because vaginal bleeding persisted, though not at an alarming rate, blood was drawn for cross-match and clot observation. The test revealed a poor, friable clot which formed in 10 minutes. The blood pressure now had fallen from 120/90 to 96/60. Bleeding per vaginam persisted. At 12:30 A.M. blood replacement therapy was begun. From this time until 3:05 A.M. the patient received 500 c.c. of dextran, 21500 c.c. of bank blood, 500 c.c. of fresh blood, 1,000 c.c. of 5 per cent glucose in dis· tilled water, 500 c.c. of normal saline, and 8 Gm. of fibrinogen. The blood pressure now was 88/64 having risen from a low of 40/0 during the period of blood and fluid replacen1ent. The bleeding from the vagina was now much less and clots were present. The patient's course had been followed by numerous clot observation tests. Blood drawn at 12:50 A.M. showed no evidence of clotting even after standing for eight hours, whereas blood drawn following the described therapy formed a stable clot in four minutes. At about 4 A.M. uterine atony was observed, evidenced by a soft fundus and resumption of vaginal bleeding. The vaginal blood :flow contained clots at this time. The uterine atony was successfully treated with an additional 1,000 c.c. of bank blood and intravenous Pitocin drip. By 6:05 A.M. the blood pressure was 118/70 and the vaginal bleeding had ceased. An oxalated blood sample, drawn at 12:50 A.M., at the same time as the clot observation sample which did not clot in eight hours, was reported by the laboratory to contain less than 50 mg. per cent of fibrinogen. The blood fibrinogen level ten hours after delivery was 600 mg. per cent. The eight-day postpartum hospital stay of the patient was uneventful except for fever on the first postpartum day. It was of further interest that there was a complete absence of lactation during the puerperium, despite the fact that following both previous pregnancies marked breast engorgement and discomfort had been encountered. When seen in the postpartum clinic ten weeks following delivery, the patient had not yet resumed menstruation. In the ensuing nine months the patient complained of amenorrhea (except for slight staining for two days at three months post par tum), absence of sweating, a dry, coarse skin, a weight gain of 15 pounds, general asthenia, partial loss of scalp, axillary, and pubic hair, dizziness, faint spells, and decreased libido. The patient's blood pressure varied between 70/40 and 90/70. Certain laboratory data obtained during the patient's visits to the Endocrine Clinic are worthy of mention: 24 hour urinary estrin, negative and 1 to 2 rat units; endometrial biopsy showed simple proliferative hyperplasia; basal metabolism rate was minus 28 per cent; 17-hydroxycorticoids (including 11-oxysteroids) were 1.5 and 0.9 mg. per 24 hour urine specimen; the serum potassium was 6.26 milliequivalents per liter. The 17 -ketosteroids, as well as the serum sodium and the glucose tolerance test, were within normal limits. X-rays of the sella turcica, likewise, were normal. The clinical symptoms and pertinent abnormal laboratory findings are indicative of pituitary hypofunction invoiving the iactogenic, gonado-, thyro-, and adrenotrophic factors.
Comment
We have presented a case of tumultuous labor with resultant afibrinogenemia (possibly due to amniotic fluid infusion), leading in turn to hemorrhage, shock, and partial pituitary necrosis.
GOLD AND LIBRACH
192
Am.]. Obst. & Gynec. July, 1957
The postulated intravascular clotting following the amniotic fluid infusion may have partly occluded the pituitary sinusoids, the circulation in which must have been further markedly embarrassed by the progressive fall in blood pressure 1vhich follo1ved delivery. The danger of a subsequent pregnancy in a patient with Sheehan's syndrome was emphasized by Israel and Conston. 6 These authors reported a case of their own, and four others from the literature, in which pregnancy followed the development of Sheehan's syndrome. Symptomatic improvement was evident during the subsequent pregnancy in each case, yet all five patients died at delivery or in the puerperium in acute collapse. These sudden deaths were attributed to an inability of the previously impaired pituitary to supply the ''acutely demanded corticotrophin, or the failure of the adrenals to secrete the essential corticoids, " 6 in rpsponse to a stress situation.
Summary 1. A case of Sheehan's syndrome following an episode of proved and successfully treated afibrinogenemia is reported. 2. The danger of rare subsequent pregnancy in such a case is mentioned.
References 1. 2. 3. 4. 5.
Simmonds, W.: Virchows Arch. Path. Anat. 217: 226, 1914. Sheehan, H. L.: J. Path. & Bact. 45: 189, 1937. Sheehan, H. L., and Murdock, R.: J. Obst. & Gynaec. Brit. Emp. 45: 456, 1938. Sheehan, H. L.: Quart. J. Med. 8: 277, 1939. Farquharson, R. F.: Simmonds' Disease: Extreme Insufficiency of the Adenohypophysis, Springfield, Ill., 1950, Charles C Thomas, Publisher. 6. Israel, S. L., and Conston, A. S.: J. A.M. A. 148: 189, 1952. 47 PLAZA STREET 17
BROOKLYN
FOLLOWING PROVED AND TREATED AFIBRINOGENEMIA* A Case Report EDWIN
M.
GOLD,
M.D.,
AND SAMUEL LIBRACH,
M.D.,
N. Y.
BROOKLYN,
(From the Department of Obstetrics and Gynecology, Jeu'ish Hospital of Brooklyn and the State University of New York, College of Medicine at New York City)
P
OSTPARTUM hemorrhage and shock have been described preceding the development of postpartum panhypopituitarism. Simmonds/ in 1914, re-
T\o~torll"tr J:-'VU!VV'-A..I-,J
~n,,,nrl
.LV\A.L.&.'-4..
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;,..,
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p\..t..VL_tJV.I.l.AI.L
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Sheehan • • in a series of papers beginning in 1937 greatly clarified this subject. From observations made in a large maternity hospital in Glasgow, he showed that areas of pituitary necrosis of varying size are commonly found in women who die during the puerperium after a delivery associated with postpartum hemorrhage. He was satisfied that infarction was due to marked ischemia of the pituitary with resultant necrosis. A recent monograph by Farquharson5 gives an excellent discussion of the clinical aspects of insufficiency of the adenohypophysis following complications of pregnancy and other conditions. Review of the literature, to the best of our knowledge, has failed to disclose any report of the development of Sheehan's syndrome following an episode of proved and successfully treated afibrinogenemia. The following case report, we feel, exemplifies such a chain of events. 2
8
Case Report The patient, a 31-year-old white para ii-o-o-ii, made five visits to the prenatal clinic of the Jewish Hospital of Brooklyn, after the twentieth week of gestation. Her antepartum course and laboratory findings were within normal limits. At term the patient was admitted to the hospital at 10:30 P.M., on June 17, 1955, in early labor with mild contractions occurring every eight minutes. The cervix was effaced, 2 em. dilated, and the vertex presented at minus one station. Fifteen minutes after admission, while the enema was being expelled, the membranes ruptured spontaneously and strong uterine contractions ensued at two- to three-minute intervals; the patient was now given sedation with Demerol, 50 mg., scopolamine, 0.2 mg., and Benadryl, 50 mg., intramuscularly. By 11:00 P.M. (one-half hour after admission) the cervix was 7 em. dilated. At 11:10 P.M. a tetanic uterine contraction which lasted almost five minutes was noted, and the uterus seemed to be hypertonic until delivery at 11:20 P.M. During this ten-minute interval the patient was transferred to the delivery room, a pudendal block was performed with 50 c.c. of 1 per cent procaine, and, because of difficulty in obtaining the fetal heart tones, delivery was easily effected by low •Presented at a meeting of the Brooklyn Gynecological Society on May lG, 1956.
190
Volume 74 Number I
SHEEHAN'S SYNDROME FOLLOWING AFIBRINOGENEMIA
191
forceps under supplemental nitrous oxide-oxygen inhalation anesthesia. A moderately de· pressed 3,090 gram female infant, born with two tight loops of cord about the neck, responded to simple resuscitative measures within three minutes. The placenta was delivered spontaneously after a three-minute third stage and showed no abnormalities or evidences of premature separation. Methergine, 0.2 mg., was given intramuscularly at this time. Ten minutes after delivery, or one hour after admission, when the fundus was apparently well contracted, a gush of blood and small clots, estimated at 300 c.c., issued forth from the birth canal. Pitocin was administered intramuscularly and by intravenous drip. Examination of the birth canal showed that the blood was coming from the uterine cavity. By midnight, 40 minutes following delivery, because vaginal bleeding persisted, though not at an alarming rate, blood was drawn for cross-match and clot observation. The test revealed a poor, friable clot which formed in 10 minutes. The blood pressure now had fallen from 120/90 to 96/60. Bleeding per vaginam persisted. At 12:30 A.M. blood replacement therapy was begun. From this time until 3:05 A.M. the patient received 500 c.c. of dextran, 21500 c.c. of bank blood, 500 c.c. of fresh blood, 1,000 c.c. of 5 per cent glucose in dis· tilled water, 500 c.c. of normal saline, and 8 Gm. of fibrinogen. The blood pressure now was 88/64 having risen from a low of 40/0 during the period of blood and fluid replacen1ent. The bleeding from the vagina was now much less and clots were present. The patient's course had been followed by numerous clot observation tests. Blood drawn at 12:50 A.M. showed no evidence of clotting even after standing for eight hours, whereas blood drawn following the described therapy formed a stable clot in four minutes. At about 4 A.M. uterine atony was observed, evidenced by a soft fundus and resumption of vaginal bleeding. The vaginal blood :flow contained clots at this time. The uterine atony was successfully treated with an additional 1,000 c.c. of bank blood and intravenous Pitocin drip. By 6:05 A.M. the blood pressure was 118/70 and the vaginal bleeding had ceased. An oxalated blood sample, drawn at 12:50 A.M., at the same time as the clot observation sample which did not clot in eight hours, was reported by the laboratory to contain less than 50 mg. per cent of fibrinogen. The blood fibrinogen level ten hours after delivery was 600 mg. per cent. The eight-day postpartum hospital stay of the patient was uneventful except for fever on the first postpartum day. It was of further interest that there was a complete absence of lactation during the puerperium, despite the fact that following both previous pregnancies marked breast engorgement and discomfort had been encountered. When seen in the postpartum clinic ten weeks following delivery, the patient had not yet resumed menstruation. In the ensuing nine months the patient complained of amenorrhea (except for slight staining for two days at three months post par tum), absence of sweating, a dry, coarse skin, a weight gain of 15 pounds, general asthenia, partial loss of scalp, axillary, and pubic hair, dizziness, faint spells, and decreased libido. The patient's blood pressure varied between 70/40 and 90/70. Certain laboratory data obtained during the patient's visits to the Endocrine Clinic are worthy of mention: 24 hour urinary estrin, negative and 1 to 2 rat units; endometrial biopsy showed simple proliferative hyperplasia; basal metabolism rate was minus 28 per cent; 17-hydroxycorticoids (including 11-oxysteroids) were 1.5 and 0.9 mg. per 24 hour urine specimen; the serum potassium was 6.26 milliequivalents per liter. The 17 -ketosteroids, as well as the serum sodium and the glucose tolerance test, were within normal limits. X-rays of the sella turcica, likewise, were normal. The clinical symptoms and pertinent abnormal laboratory findings are indicative of pituitary hypofunction invoiving the iactogenic, gonado-, thyro-, and adrenotrophic factors.
Comment
We have presented a case of tumultuous labor with resultant afibrinogenemia (possibly due to amniotic fluid infusion), leading in turn to hemorrhage, shock, and partial pituitary necrosis.
GOLD AND LIBRACH
192
Am.]. Obst. & Gynec. July, 1957
The postulated intravascular clotting following the amniotic fluid infusion may have partly occluded the pituitary sinusoids, the circulation in which must have been further markedly embarrassed by the progressive fall in blood pressure 1vhich follo1ved delivery. The danger of a subsequent pregnancy in a patient with Sheehan's syndrome was emphasized by Israel and Conston. 6 These authors reported a case of their own, and four others from the literature, in which pregnancy followed the development of Sheehan's syndrome. Symptomatic improvement was evident during the subsequent pregnancy in each case, yet all five patients died at delivery or in the puerperium in acute collapse. These sudden deaths were attributed to an inability of the previously impaired pituitary to supply the ''acutely demanded corticotrophin, or the failure of the adrenals to secrete the essential corticoids, " 6 in rpsponse to a stress situation.
Summary 1. A case of Sheehan's syndrome following an episode of proved and successfully treated afibrinogenemia is reported. 2. The danger of rare subsequent pregnancy in such a case is mentioned.
References 1. 2. 3. 4. 5.
Simmonds, W.: Virchows Arch. Path. Anat. 217: 226, 1914. Sheehan, H. L.: J. Path. & Bact. 45: 189, 1937. Sheehan, H. L., and Murdock, R.: J. Obst. & Gynaec. Brit. Emp. 45: 456, 1938. Sheehan, H. L.: Quart. J. Med. 8: 277, 1939. Farquharson, R. F.: Simmonds' Disease: Extreme Insufficiency of the Adenohypophysis, Springfield, Ill., 1950, Charles C Thomas, Publisher. 6. Israel, S. L., and Conston, A. S.: J. A.M. A. 148: 189, 1952. 47 PLAZA STREET 17
BROOKLYN