A case of postpartum necrosis of the anterior lobe of the pituitary, known as Sheehan's syndrome, is presented. The patient has done well on a combined replacement therapy with cortisone and thyroid. It is hoped that increased awareness will be given to this clinical entity.</p

Kibata, Masayoshi

Miyoshi, Isao

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Sheehan's syndrome (postpartum hypopituitarism), report of a case

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Acta Medica OkayamaVolume 16, Issue 1 1962 Article 6FEBRUARY 1962Sheehan’s syndrome (postpartumhypopituitarism), report of a caseIsao Miyoshi∗ Masayoshi Kibata†∗Okayama University,†Okayama University,Copyright c©1999 OKAYAMA UNIVERSITY MEDICAL SCHOOL. All rights reserved.Sheehan’s syndrome (postpartumhypopituitarism), report of a case∗Isao Miyoshi and Masayoshi KibataAbstractA case of postpartum necrosis of the anterior lobe of the pituitary, known as Sheehan’s syn-drome, is presented. The patient has done well on a combined replacement therapy with cortisoneand thyroid. It is hoped that increased awareness will be given to this clinical entity.∗Copyright c©OKAYAMA UNIVERSITY MEDICAL SCHOOLActa Med. Okayama 16, 37-43 (1962)SHEEHAN'S SYNDROME (POSTPARTUM HYPOPITUI-TARISM), REPORT OF A CASEIsao MIYOSHI and Masayoshi KIBATADepartment of Internal Medicine, Okayama University Medical School. Okayama(Director: Prof. K. Hiraki)Received for publication. December 19. 1961Simmonds' diseasel follows the destruction of the anterior lobe of the pitui-tary gland. The underlying pathological lesions may be caused by postpartumhemorrhage2, tumors3, head injuries4, vascular disturbances, or surgical hypo-physectomy. An obstetric difficulty complicated by excessive hemorrhage andcollapse often results in Simmonds' disease. In 1937 SHEEHAN, in a series ofbrilliant papersbB, clarified the causative relationship between the ischemicnecrosis of the anterior pituitary and postpartum hemorrhage. As a tribute tohis work, this type of Simmonds' disease has been called Sheehan's syndrome.The patient described below appears to be a typical case of the Sheehan's syn-drome. The present paper is being presented, because of the rarity of reportedcases of this syndrome in Japan.CASE REPORTA 35-year-old housewife was admitted to the hospital on August 31, 1961,because of pallor and easy fatigability. In April 1959 she had her fourth deliverythat was complicated by severe hemorrhage due to delayed separation of theplacenta and went into profound shock. For the next half a year she remainedbedfast because of generalized weakness, headache and tinnitus. Since she be-came able to be up and around, she slowly exhibited signs and symptoms ofmyxedema and anemia. She became intolerant to cold, and physically andmentally inactive. She had falling out of the scalp hair and gradually lost thepubic hair. Following delivery she failed to lactate and had amenorrhea, loss oflibido and atrophy of the breasts. At one time she developed swelling of the leftknee and 'a diagnosis of rheumatoid arthritis was made. Before she came to ourhospital, she was seen by the practicing physicians who treated her with vita-min and iron tablets to no avail.Family history was unremarkable except for her twin, second and thirdchildren.Past history revealed that her menarche was at the age of 14, and she371Miyoshi and Kibata: Sheehan's syndrome (postpartum hypopituitarism), report of a caseProduced by The Berkeley Electronic Press, 196238 1. MIYOSHI and M. KIBATAmarried at the age of 24. Seven years previously she had a vague history ofnephrolithiasis on the left. In the past she had undergone artificial abortionssix times.Physical examination on admission revealed a slow and soft speaking womanin no distress (Fig. 1). The temperature was 36.1 cc., the pulse 88, and theFig. 1. Picture of patient with Sheehan's syndrome. Note the puffy face and almost completeabs'~nce of the axillary and pubic hair.blood pressure 110 systolic, 76 diastolic. She was moderately well nourish~dand there was no sign of cachexia. There was puffiness of the face. The skinwas dry, pale, coarse and cold. There was no abnormal skin pigmentation.The thyroid was not palpable. The heart and lungs were normal to percussionand auscultation. The abdomen was not remarkable. The palpebral conjunctivawas slightly anemic. The pupils were normal and reacted promptly to light andaccommodation. The tongue was normal. The head hair was brittle. There wasthinning of the lateral margins of the eyebrows. The axillary and pubic hairwas almost absent. The breasts were atrophic. The tendon reflexes of thelower extremities were bilaterally hypoactive, but there were no sensory or2Acta Medica Okayama, Vol. 16 [1962], Iss. 1, Art. 6http://escholarship.lib.okayama-u.ac.jp/amo/vol16/iss1/6Sheehan's Syndrome 39motor disturbances. Pelvic examination revealed atrophic vaginal mucosa anda small uterus.Urinalysis was normal. A stool specimen was negative for occult blood andparasitic ova. The erythrocyte sedimentation rate was 51 mm. per hour.Serology for syphilis was negative. Examination of the blood showed a hemo-globin of 80 % (Sahli), a red-cell count of 3,570,000, and a white-cell count of3,800, with 7 % band forms, 35 % segmented neutrophils, 52 % lymphocytes,5 % rnonocytes and 1% basophils. The platelet count was 275,000 and thereticulocyte count 1.1 96. The cholesterol was 278 rng., the fasting blood sugar87 mg., the non-protein nitrogen 27 mg. per 100 rnl. The sodium was 142.5milliequiv., the potassium 3.45 milliequiv., and the chloride 104.5 milliequiv.per liter. The serum iron was 63 microgm., and copper 141 microgm. per 100ml. Bromsulfalein retention was 13 % in 45 minutes. Other liver function testswere normal. The total serum protein was 7.0 g., with the albumin 45.7 % andthe globulin 54.3 %, per 100 ml. Gastric juice analysis revealed hypoacidity.Insulin sensitivity test showed a moderate hypoglycemic response. Urinary 17-ketosteroid excretion was 0.9 mg., and 17-hydroxysteroid 3.2 mg. per 24 hours.A Thorn test caused no decrease of the circulating eosinophils. A Robinson-Kepler-Power water test gave a positive result with the quotient 'A' equal to 5.7.The basal metabolic rate was -37 %. The protein-bound iodine was 4.5 microgm.per 100 ml. Urinary estrogen excretion was 8.2 microgm. per 24 hours. AnFig. 2. EKG showing low voltage and flattened T waves in all 12 leads.3Miyoshi and Kibata: Sheehan's syndrome (postpartum hypopituitarism), report of a caseProduced by The Berkeley Electronic Press, 196240 1. MIYOSHI and M. KIBATAelectrocardiogram demonstrated low voltage and flattened T waves in all 12leads (Fig. 2). An X-ray film of the chest was normal. X-ray films of the skullshowed no abnormalities of the sella turcica.On the basis of her clinical history that dates back to her last delivery, thecharacteristic physical findings and the abnormal endocrine laboratory tests, adiagnosis of Sheehan's syndrome was considered most likely. She was thenstarted on 2 mg. of dexamethasone initially and the drug was gradually decreasedto the maintenance dose of 0.5 mg. daily. One week later, 0.1 gm. of thyroidwas added to the steroid hormone. With this combined therapy, there wereincrease of her urinary output, elevation of her temperature and blood pressure,and improvement of her appetite. Prior to the therapy, her temperature andblood pressure tended to be subnormal. She appeared animated again and be-came active in speech and thought. One month later, there was a return of thepubic hair.Dexamethasone was replaced with cortisone in the maintenance dose of 25mg. daily because of the latter's superior carbohydrate-regulating effect. At onetime, 10 units of ACTH-Z was administered together with cortisone for about10 days. But because of urticaria she developed during the ACTH-Z therapy,the drug was discontinued. Gynecological work-up revealed severe gonadalinsufficiency that failed to respond to the administration of 1,000 units of humanchorionic gonadotropine for 6 days followed by 1,000 units of pregnant mereserum for 6 days. Despite atrophic endometrium, she once had a menstruation·like bleeding by the successive use of estrogen and progesterone. She wasdischarged on September 15, 1961 to be followed in the outpatient clinic.DISCUSSIONSHEEHAN~, on the basis of many histologic observations, concluded thatobstetric hemorrhage and collapse drastically cut down the amount of blood thatcirculates into the anterior pituitary, leading to the stasis and thrombus forma'tion in the sinusoids of the anterior lobe of the pituitary. This sequence of eventsresults in the ischemic necrosis of the anterior pituitary cells, and the severity ofthe hemorrhage determines the degree of cellular involvement, and consequentlythe clinical course of the patient. Since traumatic bodily hemorrhage and shockresulting in the ischemic necrosis of the anterior pituitary in either sex must beextremely rare, some other factors such as vulnerability of the hypertrophiedpituitary to the decreased blood flow and increased coagulability of blood duringparturition may be operating in the development of the Sheehan's syndrome.European and American literatures are replete with case reports of theSheehan's syndrome1,6.6,8,9-12,16, but not many cases have been reported in theJapanese literature13-16• Indeed the lack of awareness of this condition might be4Acta Medica Okayama, Vol. 16 [1962], Iss. 1, Art. 6http://escholarship.lib.okayama-u.ac.jp/amo/vol16/iss1/6Sheehan's Syndrome 41in part responsible for a small figure of the reported cases in this country.As a matter of fact, Sheehan's syndrome is not so uncommon as has beengenerally believed. But in the early stage of the disease or in an atypical case,it is not always easy to make a correct diagnosis. Therefore, it is highly probablethat many patients having the Sheehan's syndrome are treated by physiciansas primary myxedema, Addison's disease, unexplained anemia, neurosis oranorexia nervosa.Anorexia and cachexia erroneously associated with Simmonds' disease areseen uncommonly in the Sheehan's syndrome7and may be more pronounced inanorexia nervosa. Reference may be made to Escamilla's extensive reviewconcerning differential diagnosis between Simmonds' disease and anorexianervosa16• In Addison's disease there is a striking pigmentation in contrast tothe Sheehan's syndrome.As a sequel to anterior pituitary insufficiency, sexual atrophy is usuallyearly and absolutell• While clinical evidence of thyroid and adrenocortical failuremanifests itself slowly and partially. Our case reported herein appears to be noexception to this rule.When endogenous adrenocorticotropic hormone becomes deficient, of threerepresentative adrenocortical hormones, 'N' hormone is the first to becomedeficient, followed by'S' hormone. It is generally believed that salt-regulatinghormone is not under the direct influence of the adrenocorticotropic hormone17.Since the pituitary lesion is irreversible, efforts should be made to restoreas much functions of the target endocrine organs. At present, there is nopituitary extract that can completely substitute the anterior pituitary. Tropichormones may be used effectively when the target orgalls are still in the stateof responsiveness to the tropic stimulation. However, by the time the patientwith hypopituitarism seeks medical help, the endocrine organs would haveundergone considerable atrophy to the point where the effects of tropic stimula-tion are unpredictable. WHITTAKER et ar9• reported advantageous use of ACTH,but the effect of ACTH does not usually last long after its withdrawal. In addition,a long-term use of ACTH may provoke allergic manifestations as in our case inwhich urticaria developed following its administration. It is for these reasonsthat replacement ,therapy is the treatment of choice in most of the patients.Cortisone is the ideal carbohydrate-regulating hormone for replacement and canbe given in small dosages of 25 mg. daily by tableeB• Our patient is doingwell on half a tablet of cortisone plus supplemental thyroid medication. It isdangerous to administer thyroid alone, because the drug would augumentmetabolic activity despite the associated adrenocortical insufficiency, precipitatingthe development of the Addisonian crisis3.2O.Many designations used in the earlier literatures such as 'pluriglandular in-5Miyoshi and Kibata: Sheehan's syndrome (postpartum hypopituitarism), report of a caseProduced by The Berkeley Electronic Press, 196242 1. MIYOSHI and M. KIBATAsufficiency,211 'multiple ductless glandular sclerosis,22' 'hypophysare Magersucht,23and 'cachexia hypophyseopriva24' are in all likelihood synonymous to Simmonds'disease.SUMMARYA case of postpartum necrosis of the anterior lobe of the pituitary, knownas Sheehan's syndrome, is presented. The patient has done well on a combinedreplacement therapy with cortisone and thyroid. It is hoped that increasedawareness will be given to this clinical entity.ACKNOWLEDGEMENTWe are indebted to Prof. K. Hiraki of the Department of Medicine for the guidance givenin the preparation of this paper.REFERENCES1. SIMMONDS, M.: Uber Hypophysisschwund mit Wdlichen Ausgang. Dtsch. med. Wchnschr.40, 322, 19142. Textbook of Endocrinology. Edited by Williams, R. H. Saunders, Philadelphia; 1955. p.533. TUCKER, H. S. G. Jr., CHITWOOD, J. L. and PARKER, C. P. Jr.: Pituitary myxedema; reportuf three cases. Ann. Int. Med. 32, 52, 19504. Altman, R. and Pruzanski, W.: Post-traumatic hypopituitarism; anterior pituitary insuffici-ency following skull fracture. Ann. Int. Med. 55, 149, 19615. SHEEHAN,H.L.J.: Postpartum necrosis of the anterior pituitary. J. Path. Bact.45, 189,19376. SHEEHAN, H. L.: Simmonds' disease due to postpartum necrosis of the anterior pituitary.Quart. ]. Med. 8, 277, 19397. SHEEHAN, H. L. and SUMMERS, V. K.: The syndrome of hypopituitarism. Quart. ]. Med. 18,319, 19498. COOKE, R. T. and SHEEHAN, H. L.: Cases of hypopituitarism. Brit. Med. ]. 1, 928, 19509. COOKE, J. E., BEAN, W. B., FRANKLIN, M. and EMBICK, J. F.: Postpartum necrosis of theanterior lobe of the pituitary gland; Sheehan's disease. Arch. Int. Med. 87, 517, 195110. HEYDE, E. c.: Sheehan's syndrome (postpartum panhypopituitarism); Report of a case andcomment on clinical management. Arch. Int. Med. 92, 442, 195311. HUBBLE, D.: The course of anterior hypopituitarism. Lancet, 262, 1123, 195212. ISRAEL, S. L. and CONSTON, A. S.: Unrecognized pituitary necrosis (Sheehan's syndrome);a cause of sudden death. J.A.M.A. 148, 189, 195213. TANAKA, S., HOSONO, K. and YAZIMA, T.: Sheehan's syndrome (postpartum hypopituitarism);report of a case. Niigata Igakkai Zasshi. 71, 1037, 1957 (in Japanese)14. WATANABE, T. and TSUKAGOSHI, H.: Simmonds' disease (Sheehan's syndrome); report ofa case. Clin. Endocrinol. 4, 608, 1956 (in Japanese)15. KAWAKAMI, H. TANAKA, M. SAKURAl, K. and YONEYAMA, M.: Diagnostic usefulness ofThorn test using ACTE depot in Sheehan's disease. Obst. Gynecol. 24, 648, 1957 (inJapanese)16. ESCAMILLA, R. F. and LISSBR, H.: Simmonds' disease; Clinical study with review of theliterature; Differentiation from anorexia nervosa by statistical analysis of 595 cases, 101 of6Acta Medica Okayama, Vol. 16 [1962], Iss. 1, Art. 6http://escholarship.lib.okayama-u.ac.jp/amo/vol16/iss1/6Sheehan's Syndrome 43which were proved pathologically. ]. Clin. Endocrinol. 2, 65, 194217. VENNING, E. H., DYRENFURTH, F. and BECK,]. C.: Effect of corticotrophin and prednisoneon the excretion of aldosterone in man. ]. Clin. Endocrinol. 16, 1541, 195618. SHEEHAN, H. L. and SUMMERS, V. K.: Oral cortisone treatment of hypopituitarism. Brit.Afed. ]. 1, 723, 195419. WHITTAKER, S. R. F. and WHITEHEAD, T. P.: The diagnosis and treatment of hypopituitarism.Brit. Afed. ]. 2, 265, 195420. LERMAN,]. and STEBBINS, H. D.: The pituitary type of myxedema.]. A. M. A. 119, 391,194221. HIRSCH, S. and BERBERICH, J.: Beitrag zur Frage der multiplen BlutdrUsensklerose. Klin.fVchnschr. 3, 483, 192422. THuR, W.: 'Ober die Abgrenzung der Simmondsschen Kachexie van der multiplen Blut·drUsensklerose. Frankfurt. Ztschr. Path. 36, 661, 192823. LUCKE, H.: Hypohysare Magersucht und Insulin. Klin. Wchnschr. 11, 1988, 193224. ELSBERG, C. A. and KRUG, E. F. A.: A case of hyppituitarism with homonymous hemianopsiacompletely relieved by glandular treatment. Arch. Ophth. 46, 97, 19177Miyoshi and Kibata: Sheehan's syndrome (postpartum hypopituitarism), report of a caseProduced by The Berkeley Electronic Press, 1962

Sheehan's syndrome (postpartum hypopituitarism), report of a case

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