Secondary Adrenal Insufficiency Caused by Adult Development of Pituitary Stalk Transection

Gotyo, Naoki; Doi, Masaru; Izumiyama, Hajime; Hirata, Yukio

doi:10.2169/internalmedicine.46.6404

Cited by 9 publications

(8 citation statements)

References 6 publications

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“…The first one of our cases had low free T4 and normal TSH levels along with response to TRH test. There is one case report similar to ours in the literature (11). The patient had low free T4 and normal TSH levels along with the response to TRH test.…”

Section: Discussionsupporting

confidence: 78%

“…The fixed head of the fetus and breech presentation due to small maternal pelvis induce traction and transection of the pituitary stalk, but rarely brain trauma which may be caused by major events such as traffic accident (9). Some of the patients present with growth retardation due to hypotalamic and pituitary deficiency, others are diagnosed with acute adrenal deficiency (10,11). This syndrome is the most commonly diagnosed in childhood, whereas some of the patients are diagnosed in adulthood (12).…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Two Adult Patients with Ectopic Neurohypophysis and Panhypopituitarism Due to Absent Pituitary Stalk

Acibucu¹,

Kilicli²,

Dökmetaş³

2014

tjem

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Hipofiz sapı yokluğuna bağlı total hipofizer yetmezliği ve ektopik nörohipofizi olan 27 ve 19 yaşlarında iki olguyu sunuyoruz. Bu hastalar endokrinoloji kliniğine boy kısalığı, gelişme geriliği ve primer amenore şikayetleri ile başvurdular. Bazal hormonların değerlendirilmesi ile sekonder hipotiroidi, adrenal yetersizlik, hipogonadizm ve büyüme hormonu eksikliği tespit edildi. Kısa synacten testinde pik kortizol cevabı normalken insülin tolerans testinde yetersizdi. Diğer dinamik hipofiz fonksiyon testlerinde total anterior hipofiz hormon yetersizliği vardı. Hipofiz bezinin manyetik rezonans görüntülemesinde hipofiz sapının olmadığı ve posterior hipofiz dokusunun ektopik yerleştiği görüldü. Hormon replasman tedavisine başlandı. Bu rahatsızlık genellikle çocuk yaş grubunda tespit edilmesine rağmen biz ektopik posterior hipofiz dokusu, hipofiz sap yokluğu ve total anterior hipofiz hormon yetersizliği olan iki erişkin hastayı sunuyoruz. Turk Jem 2014; 18: 143-146 Anah tar ke li me ler: Ektopik nörohipofiz, total hipofizer yetmezlik, hipofiz sapı yokluğu We report two cases of 27-year-old and 19-year-old female patients with ectopic neurohypophysis and panhypopituitarism due to absent pituitary stalk. They were admitted to the endocrinology clinic with short stature, growth retardation and primary amenorrhea. Basal hormones revealed secondary hypothyroidism, adrenal insufficiency, hypogonadism and growth hormone insufficiency. Peak cortisol response to the short synacthen test (SST) was normal but was inadequate to insulin tolerance test. The other dynamic pituitary function tests showed panhypopituitarism. Magnetic resonance imaging of the pituitary gland revealed an ectopic posterior pituitary tissue and absent pituitary stalk. We administered hormone replacement therapy. As this disorder is usually encountered in the pediatric age group, we report here two adult patients with ectopic posterior pituitary tissue, absent pituitary stalk and panhypopituitarism.

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Section: Discussionsupporting

confidence: 78%

Section: Discussionmentioning

confidence: 99%

Two Adult Patients with Ectopic Neurohypophysis and Panhypopituitarism Due to Absent Pituitary Stalk

Acibucu¹,

Kilicli²,

Dökmetaş³

2014

tjem

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“…The disease initially manifests as growth-hormone-deficient short stature, followed by subsequent development of sequential insufficiency of several other pituitary hormones, and finally ACTH deficiency in adulthood. 4 Transection of the pituitary stalk occurs mainly during an abnormal delivery, such as breech delivery, but brain trauma rarely occurs. 5 The present case demonstrates sequential impairment of secretion of multiple pituitary hormones and transection of the pituitary stalk.…”

Section: Discussionmentioning

confidence: 99%

“…15 The mechanism responsible for pituitary stalk transection syndrome is still unknown. Although there have been many reports that have suggested that endocrinopathy and transection of the pituitary stalk may have an association with breech delivery, [3][4][5][6][7][8][9][10][11] Triulzi et al reported that only 32% of a study population of 101 patients had a birth history of breech delivery. They concluded that dysgenesis of the mediobasal prosencephalic structures or brain anomaly probably caused endocrinopathy, and that breech delivery may be the result of a midline brain anomaly, rather than the cause.…”

Section: Discussionmentioning

confidence: 99%

Case of pituitary stalk transection syndrome ascertained after breech delivery

Fukuta

Hidaka

Ono

et al. 2015

J of Obstet and Gynaecol

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Pituitary stalk transection syndrome (PSTS) is a rare complication that can accompany breech delivery. Early diagnosis of this syndrome is difficult, and it may cause a serious delay in the diagnosis. We present a case of PSTS ascertained after breech delivery. A 20-year-old woman presented with primary amenorrhea. The patient was born by breech delivery and had a history of treatment for pituitary dwarfism. Her laboratory findings showed pituitary hypothyroidism, and hormone replacement therapy was initiated. At 28 years old, she became pregnant and had a normal delivery at 38 weeks' gestation. One year after delivery, her thyroid hormone level changed. Laboratory test showed adrenocortical insufficiency, and magnetic resonance imaging of the pituitary gland showed transection of the pituitary stalk and development of an ectopic posterior lobe. These findings were compatible with PSTS. When a patient who has been born by breech delivery presents with symptoms of pituitary deficiency, PSTS should be considered in the differential diagnosis.

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“…Those patients are diagnosed with “pituitary stalk transection syndrome”. In most cases, GH insufficiency develops first, followed by insufficiencies of gonadotropins and thyroid stimulating hormone (TSH) during childhood, and finally adrenocorticotropic hormone (ACTH) insufficiency in later adulthood (2,3). They also have a potential risk of secondary adrenal insufficiency (2).…”

Section: Introductionmentioning

confidence: 99%

Successful Pregnancies and Deliveries in a Patient With Evolving Hypopituitarism due to Pituitary Stalk Transection Syndrome: Role of Growth Hormone Replacement

et al. 2017

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We herein report a 31-year-old Japanese woman with evolving hypopituitarism due to pituitary stalk transection syndrome. She had a history of short stature treated with growth hormone (GH) in childhood and had hypothyroidism and primary amenorrhea at 20 years old. Levothyroxine replacement and recombinant follicle stimulating hormone-human chorionic gonadotropin (FSH-hCG) therapy for ovulation induction were started. GH replacement therapy (GHRT) was resumed when she was 26 years old. She developed mild adrenocortical insufficiency at 31 years old. She succeeded in becoming pregnant and delivered twice. GHRT was partially continued during pregnancy and stopped at the end of the second trimester without any complications.

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Secondary Adrenal Insufficiency Caused by Adult Development of Pituitary Stalk Transection

Abstract: We report the case of a 38-year-old

Cited by 9 publications

References 6 publications

Two Adult Patients with Ectopic Neurohypophysis and Panhypopituitarism Due to Absent Pituitary Stalk

Two Adult Patients with Ectopic Neurohypophysis and Panhypopituitarism Due to Absent Pituitary Stalk

Case of pituitary stalk transection syndrome ascertained after breech delivery

Successful Pregnancies and Deliveries in a Patient With Evolving Hypopituitarism due to Pituitary Stalk Transection Syndrome: Role of Growth Hormone Replacement

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