DOI: 10.1148/radiol.2432040385
(Radiology 2007;243:594-597.)
© RSNA, 2007
Case 112: Pituitary Stalk Transection Syndrome with Ectopic Posterior Pituitary Gland1
A. S. A. van der Linden, MD and
Hendrik W. van Es, MD, PhD
1 From the Department of Radiology, St Antonius Hospital, Koekoekslaan 1, 3435 CM Nieuwegein, the Netherlands. Received February 26, 2004; revision requested May 6; revision received August 26; accepted October 4; final version accepted November 3.
Correspondence: Address correspondence to A.S.A.v.d.L. (e-mail: a.van.der.linden{at}antonius.net).
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HISTORY
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A girl delivered at term with normal birth length and weight developed severe neonatal hypoglycemia and convulsions on her 2nd day of life. The pregnancy and delivery were uncomplicated. During this patient's 1st year of life, her growth rate slowed and her stature was short because of a growth hormone deficiency. At the age of 6 years, growth hormone supplementation was started. During the next 5 years, her growth rate was parallel to the P3 (third percentile, 97% of the female population in the Netherlands has reached a greater length at the same age) growth curve, with a growth rate of 4.6 cm during the 5th year of growth hormone supplementation. (This growth rate is normal for a girl of this age growing along this growth curve.) Magnetic resonance (MR) imaging was performed 11 years after birth to determine the cause of growth hormone deficiency.
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IMAGING FINDINGS
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Midsagittal T1-weighted MR imaging revealed a small anterior pituitary gland and an absent pituitary stalk. The ectopic posterior pituitary gland appeared as an area of high signal intensity in the midline at the median eminence (Fig 1). Coronal unenhanced and contrast material–enhanced T1-weighted MR imaging also depicted the ectopic posterior pituitary gland; however, the pituitary stalk was still not visible, even after administration of gadopentetate dimeglumine (Magnevist; Schering, Berlin, Germany) (Figs 2, 3).
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Figure 1: Unenhanced midline sagittal T1-weighted MR image (repetition time msec/echo time msec, 450/15) shows the small anterior pituitary gland (short arrow) and ectopic posterior pituitary gland (long arrow), which is seen as an area of high signal intensity in the midline at the median eminence. P = posterior.
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Figure 3a: Gadolinium-enhanced coronal T1-weighted MR images (450/15). (a) The pituitary stalk (arrow) is not visibile. (b) The ectopic posterior pituitary gland (arrow) is visible. L = left.
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Figure 3b: Gadolinium-enhanced coronal T1-weighted MR images (450/15). (a) The pituitary stalk (arrow) is not visibile. (b) The ectopic posterior pituitary gland (arrow) is visible. L = left.
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DISCUSSION
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Growth hormone deficiency is a common endocrinologic cause of short stature. This hormone deficiency may be idiopathic or associated with organic causes, such as tumors or surgery (1). Idiopathic growth hormone deficiency occurs sporadically and may be isolated or associated with multiple pituitary hormone deficiencies. Findings associated with hypopituitarism include breech presentation, neonatal or early onset hypoglycemia, prolonged or severe neonatal jaundice, micropenis, and a single central incisor.
Clinical isolated growth hormone deficiency may progress to multiple pituitary hormone deficiency (2). Such progression cannot be predicted reliably on the basis of clinical and laboratory findings. However, MR imaging can be used to study the anatomic details of the pituitary-hypothalamic region. It has been shown that idiopathic growth hormone deficiency can result in an abnormal appearance of the hypothalamus or pituitary gland on MR images (3). Such anatomic details can be useful when predicting whether clinical isolated growth hormone deficiency has the potential to progress to multiple pituitary hormone deficiency (4).
Isolated growth hormone deficiency and multiple pituitary hormone deficiency can be part of the pituitary stalk transection syndrome (5). Imaging characteristics of this syndrome are lack of pituitary stalk visibility and hypoplasia of the anterior hypophysis. Pituitary stalk visibility is assessed after injection of a gadolinium-enhanced contrast agent by using techniques intended to optimize visualization of a thin stalk. Furthermore, there may be a hyperintense ectopic posterior pituitary gland (6–8).
As already mentioned, this syndrome is associated with either isolated growth hormone deficiency or multiple pituitary hormone deficiency. A truncated or thin pituitary stalk is highly indicative of isolated growth hormone deficiency. In 21 patients with isolated growth hormone deficiency evaluated with MR imaging, 19 had a thin or truncated stalk (3). Isolated growth hormone deficiency is also more common in patients with an ectopic posterior pituitary gland associated with a visible pituitary stalk. Of 25 patients with growth hormone deficiency and an ectopic posterior pituitary gland, 12 had isolated growth hormone deficiency and all 25 had a pituitary stalk that was visible on MR images (5). Absence of the pituitary stalk is associated almost exclusively with multiple pituitary hormone deficiency. In 23 patients with multiple pituitary hormone deficiency, the stalk was not visible in 22 and truncated in one. In 22 patients with isolated growth hormone deficiency, the stalk was absent in only one (3). These are reliable and easily recognizable signs that can be helpful in the care of patients with growth hormone deficiency.
Patients with imaging characteristics indicative of multiple pituitary hormone deficiency need to undergo repeated assessment of pituitary function. Progression to complete anterior pituitary hormone deficiency may occur, even during the 2nd or the 3rd decade of life.
In this patient, the pituitary stalk was not visible, even after injection of a gadolinium-containing contrast agent, and the ectopic posterior pituitary gland had high signal intensity on MR images. Also, the history of the patient was indicative of multiple hormone deficiency (severe neonatal hypoglycemia combined with growth hormone deficiency). The patient's history, in combination with MR findings, was diagnostic for pituitary stalk transection syndrome with an ectopic posterior pituitary gland.
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FOOTNOTES
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Authors stated no financial relationship to disclose.
| Part one of this case appeared 4 months previously and may contain larger images.
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References
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- Hamilton J, Blaser S, Daneman D. MR imaging in idiopathic growth hormone deficiency. AJNR Am J Neuroradiol 1998;19:1609–1615.[Abstract]
- Meszaros F, Vergesslich K, Riedl S. Posterior pituitary ectopy in children with idiopathic growth hormone deficiency. J Pediatr Endocrinol Metab 2000;13:629–635.[Medline]
- Kornreich L, Horev G, Lazar L. MR findings in growth hormone deficiency: correlation with severity of hypopituitarism. AJNR Am J Neuroradiol 1998;19:1495–1499.[Abstract]
- Miyamoto J, Hasegawa Y, Ohnami N. Development of growth hormone and adrenocorticotropic hormone deficiencies in patients with prenatal or perinatal-onset hypothalamic hypopituitarism having invisible or thin pituitary stalk on magnetic resonance imaging. Endocr J 2001;48:355–362.[Medline]
- Chen S, Leger J, Garel C. Growth hormone deficiency with ectopic neurohypophysis: anatomical variations and relationship between the visibility of the pituitary stalk asserted by magnetic resonance imaging and anterior pituitary function. J Clin Endocrinol Metab 1999;84:2408–2413.[Abstract/Free Full Text]
- Fujisawa I, Kikuchi K, Nishimura K. Transection of the pituitary stalk: development of an ectopic posterior lobe assessed with MR imaging. Radiology 1987;165:487–489.[Abstract/Free Full Text]
- Kandemir N, Yordam N, Cila A, Besim A. Magnetic resonance imaging in growth hormone deficiency: relationship between endocrine function and morphological findings. J Pediatr Endocrinol Metab 2000;13:171–178.[Medline]
- Binder G, Nagel B, Ranke M. Isolated GH deficiency (IGHD) type II: imaging of the pituitary gland by magnetic resonance reveals characteristic differences in comparison with severe IGHD of unknown origin. Eur J Endocrinol 2002;147:755–760.[Abstract]
Congratulations to the 224 individuals and 10 resident groups that submitted the most likely diagnosis (pituitary stalk transection syndrome with ectopic posterior pituitary gland) for Diagnosis Please, Case 112. The names and locations of the individuals and resident groups, as submitted, are as follows:
Individual responses
- Patrik Aerts, MD, Roeselare, Belgium
- Gholamali Afshang, MD, Tinley Park, Ill
- Erhan Akpinar, Ankara, Turkey
- Canan Altay, MD, Izmir, Turkey
- Albert J. Alter, MD, Madison, Wis
- Nabil Farid Ammouri, MD, Zahle, Bekaa, Lebanon
- Jason Brandon Ashley, MD, London, Ontario, Canada
- Guis Saint-Martin Astacio, MD, Rio De Janeiro, Brazil
- Kenneth Francis Baliga, MD, Rockford, Ill
- Gregory J. Balmforth, MD, Tucson, Ariz
- Aldo Barbosa, Itapagipe, Brazil
- Paula Bartumeus Martinez, Valencia, Spain
- Fabrice Basseau, Tarbes, France
- J. Scott Bolton, MD, Dothan, Ala
- Eric Leigh Bressler, MD, Minnetonka, Minn
- Daniel F. Broderick, MD, Jacksonville, Fla
- Douglas Charles Brown, MD, Virginia Beach, Va
- Michael Patrick Buetow, MD, Okemos, Mich
- Marcelo Cabrini, Lomas De Zamora, Argentina
- Marcos Nogueira Chagas, MD, Brasilia, Brazil
- Govind Babusing Chavhan, MD, Toronto, Ontario, Canada
- Yun-Ju Chen, MD, Kaohsiung, Taiwan
- Natesan Chidambaranathan, MD, Chennai, India
- Michael Harold Childress, MD, Silver Spring, Md
- Honorio Chiminazzo, MD, Campinas, Brazil
- Haris Chrysikopoulos, Corfu, Greece
- Christopher Chu, MBBS, Sydney, Australia
- Alex T. Chuang, MD, Dallas, Tex
- Ming-Tsung Chuang, MD, Kaohsiung, Taiwan
- Carla Conceição, Amadora, Portugal
- Neal Raymond Conti, MD, Seattle, Wash
- Yves-Sebastien Cordoliani, MD, Chatenay Malabry, France
- Gonzalo Lecumberri Cortes, MD, Bilbao, Vizcaya, Spain
- Sebastian Costantino, MD, Mar del Plata, Argentina
- Alberto Cunat, MD, Valencia, Spain
- Marco Antonio Cura, MD, San Antonio, Tex
- Jay Leo Curtin, MD, Acworth, Ga
- Marc G. De Baets, MD, Agra, Switzerland
- Peter De Baets, Damme, Belgium
- Johannes F. K. De Villiers, MBCHB, MMed, Gisborne, New Zealand
- Andrew Robert Deibler, MD, Winston-Salem, NC
- Mustafa Kemal Demir, MD, Istanbul, Turkey
- Thaworn Dendumrongsup, MD, Hat Yai, Songkla, Thailand
- Susana Dias, MD, Porto, Portugal
- Romeu Cortes Domingues, MD, Rio de Janeiro, Brazil
- Sathishkumar Dundamadappa, MBBS, Worcester, Mass
- Michael D. Edwards, MD, Knoxville, Tenn
- Seyed A. Emamian, MD, PhD, Rockville, Md
- Enrique Remartinez Escobar, MD, Melilla, Spain
- Aldo Fabbricatore, Jr, MD, Vigo, Spain
- Steven J. Fagan, DO, Mount Pleasant, SC
- Juliet Howard Fallah, MD, Clarendon Hills, Ill
- Susan Michelle Fanapour, DO, Lombard, Ill
- Renato Campos Soares de Faria, MD, Ribeirao Preto, Brazil
- Virginia Fattal Jaef, MD, Rosario, Argentina
- Laura Zindell Fenton, MD, Greenwood Village, Colo
- Brett D. Ferdinand, MD, Livingston, NJ
- James Fitzgerald, Milsons Point, Australia
- Akira Fujikawa, MD, Setagaya, Tokyo, Japan
- Ann S. Fulcher, MD, Midlothian, Va
- William H. Gallmann III, MD, Shreveport, La
- Rajneesh Galwa, Jaipur, India
- Ram Prakash Galwa, MD, Chandigarh, India
- Pedro Garcia, Gijon, Spain
- Denis J. Gardeur, MD, Paris, France
- Douglas Joseph Gardner, MD, Windsor, Ontario, Canada
- Robert William Gardner, MD, Seaside, Calif
- William George Gawman, MD, Waterloo, Ontario, Canada
- Gilles Genin, Annecy, France
- John William Gianini, MD, Gainesville, Fla
- Michael S. Gibson, MD, Rockville, Md
- Mark Gilbert Goldshein, MD, Andover, Mass
- Steven J. Goldstein, MD, Lexington, Ky
- Francisco Jose Gonzalez, Santander, Spain
- James W. Graham, MD, APO, United States
- Aleksandar Grgic, MD, Zweibruecken, Germany
- Donald J. Grunz, MD, Saint Louis, Mo
- Flavius F. Guglielmo, MD, Basking Ridge, NJ
- Pramod Kumar Gupta, MD, Plano, Tex
- Ferris M. Hall, MD, Brookline, Mass
- Michael J. Hall, MD, Milwaukee, Wis
- David Chaim Harrison, MD, Salem, Ore
- Clare M. Hartigan, MBBCH, London, England
- D. Cressler Heasley, Jr, MD, Dallas, Tex
- John R. Hebert II, MD, Shreveport, La
- Marc Heinrich, Erlangen, Germany
- Thomas Sacher Helling, Jr, MD, Richardson, Tex
- Yuusuke Hirokawa, MD, Kyoto, Japan
- Ronald J. Homer, MD, Weston, Conn
- Suzanne Yoon Homer, MD, Weston, Conn
- Alberto Carlucci Iaia, MD, Wilmington, Del
- Rajapandian Ilangovan, MD, FRCR, Harrow, England
- Teeranan Intharapat, MD, Hat-Yai, Songkhla, Thailand
- Kenji Kachi, MD, Setagaya, Tokyo, Japan
- Kiriakos Kalampoukas, MD, Halandri, Greece
- Boubagra Kamel, Grenoble, France
- Todd Mitchell Kaplan, MD, New Port Richey, Fla
- Pinar S. Karakas, MD, Cleveland, Ohio
- Amna Abdulkarim Kashgari, MD, Riaydh, Saudi Arabia
- Masako Kataoka, MD, Cambridge, England
- Katsuhiko Kato, MD, Nagoya, Aichi, Japan
- Yasuhiro Kawahara, Sasebo, Japan
- Takuji Kiryu, MD, PhD, Gifu, Japan
- Sawako Kitahara, Otsu, Japan
- Steven A. Klein, MD, Shrewsbury, Mass
- Alexis Lacout, MD, Paris, France
- Mario A. Laguna, MD, Milwaukee, Wis
- Matias Landi, MD, Mar del Plata, Argentina
- Michael Laucella, MD, Bay Shore, NY
- Martin Lecompte, MD, Manotick, Ontario, Canada
- David Anthony Lisle, MBBS, Brisbane, Australia
- Patricia Ann Lowry, MD, Chattanooga, Tenn
- Natalia Madronal, MD, Valencia, Spain
- Naganathan B. S. Mani, MD, Nassau, Bahamas
- Paulo Mariz Filho, MD, Salvador, Brazil
- Michael Beckett Martin, MD, Austin, Tex
- Andrew C. Mason, MBBCH, Vancouver, British Columbia, Canada
- Waldir Heringer Maymone, MD, Rio de Janeiro, Brazil
- Frank J. McKowne, MD, Vancouver, Wash
- Sunil Labhshanker Mehta, MD, Mississauga, Ontario, Canada
- Koen Pieter Mermuys, MD, Heverlee, Belgium
- Nikolaos Michailidis, Thessaloniki, Greece
- Manabu Minami, MD, Yokohama, Kanagawa, Japan
- Sankar R. Mondal, MD, Nassau, Bahamas
- Jose Mondello, MD, Buenos Aires, Argentina
- Thomas Moser, MD, Strasbourg, France
- Utaroh Motosugi, MD, Yamanashi, Japan
- Sugoto Mukherjee, MBBS, Charlottesville, Va
- Tetsuo Nakayama, MD, Osaka, Japan
- Marcelo Ricardo Canuto Natal, MD, Brasilia, Brazil
- Tammam Naim Nehme, MD, East Wenatchee, Wash
- Karl Friedrich Rudolf Neufang, MD, Koein, Germany
- Soheil Niku, MD, San Diego, Calif
- Mizuki Nishino, MD, Boston, Mass
- Albert Nizzero, MD, Sudbury, Ontario, Canada
- Hiroshi Nobusawa, MD, PhD, Ota, Tokyo, Japan
- Patrick Augustine O'Keeffe, MBBCh, Clonmel, Ireland
- Anietie Edet Okon, MD, North Liberty, Iowa
- Laura Oleaga, Bilbao, Spain
- Sanford M. Ornstein, MD, Paradise Valley, Ariz
- Klaus Orth, Aachen, Germany
- Carlos Ovejero Vela, Barcelona, Spain
- Ann Burleson Owen, MD, Murfreesboro, Tenn
- Sean Chevalier Owens, MD, Louisville, Ky
- Narendrakumar P. Patel, MD, Newburgh, NY
- Prakash N. Patel, MD, New City, NY
- Fernando de Lamare Paula, Sr, MD, Tatui, Brazil
- Christopher R. Payne, MD, Greensboro, NC
- Yeliz Pekcevik, Izmir, Turkey
- Victor A. Perez-Candela, MD, Las Palmas, Spain
- Joseph Raymond Perno, MD, PhD, Hamilton, NJ
- Sudhakar N. Pipavath, MD, Seattle, Wash
- John M. Plotke, MD, Naperville, Ill
- Gary A. Podolny, MD, Tucson, Ariz
- Sanjay P. Prabhu, MBBS, Melbourne, Australia
- Henry Pribram, Orange, Calif
- Ilias Primetis, MD, Athens, Greece
- Karthikram Raghuram, Birmingham, Ala
- Keshav Prahalada Raichurkar, MD, Mysore, India
- Claudio Prata Ramos, MD, Juiz de Fora, Brazil
- Mauricio Corral Ramos, MD, El Paso, Tex
- Daniel C. Rappaport, MD, Toronto, Ontario, Canada
- Eytan Raz, BS, Rome, Italy
- Ryan Paul Rebello, MD, Dundas, Ontario, Canada
- Alan R. Reeves, MD, Lenexa, Kan
- Matthew C. Rheinboldt, MD, Nashville, Tenn
- Randall Earl Rhodes, MD, Belvidere, Ill
- Manoel De Souza Rocha, MD, São Paulo, Brazil
- Jose Rueda-Vicente, MD, Almeria, Spain
- Tsutomu Sakamoto, MD, Tokyo, Japan
- Hatice Tuba Sanal, MD, Ankara, Turkey
- Roberto Queiroz dos Santos, MD, Rio de Janeiro, Brazil
- Andrei Savelov, Novosibirsk, Russian Federation
- Steven Schepers, Herent, Belgium
- Pierre Schmit, MD, Halifax, Nova Scotia, Canada
- Steven Mark Schultz, MD, Fort Worth, Tex
- Anthony J. Scuderi, MD, Johnstown, Pa
- Mustafa Secil, MD, Izmir, Turkey
- Rodney G. Shaffer, MD, Tulsa, Okla
- Matthew P. Shapiro, MD, Charlottesville, Va
- Hideki Shima, MD, Tokyo, Japan
- Taro Shimono, MD, Osaka, Sayama, Japan
- Grady V. Shue, Jr, MD, Hickory, NC
- Richard Jonas Silberstein, MD, Monte Sereno, Calif
- Ken Simmons, MD, Sydney, Australia
- Darrin S. Smith, MD, Visalia, Calif
- Annemie Snoeckx, MD, Zandhoven, Belgium
- David F. Sobel, MD, La Jolla, Calif
- Maria V. Spampinato, MD, Charleston, SC
- Anouk Stein, MD, Phoenix, Ariz
- Kouichi Sugiyama, Numazu, Japan
- Norio Takahashi, MD, Fukui, Japan
- Ken Tamai, Kyoto, Japan
- Ayako Tamura, MD, Tokyo, Japan
- Douglas L. Teich, MD, Brookline, Mass
- Rogério Teles de Melo, Belo Horizonte, Brazil
- Osman Temizoz, Edirne, Turkey
- Kazuma Terauchi, Fujieda City, Japan
- Fritz Josef Teufl, MD, Stollberg, Germany
- William C. Torreggiani, MB, Dublin, Ireland
- Ozgur Tosun, Ankara, Turkey
- Ricardo Hipolito Trueba, MD, Buenos Aires, Argentina
- Eugene Walter Tryciecky, DO, Farmington Hills, Mich
- Baris Ismail Turkbey, MD, Ankara, Turkey
- Meric Tuzun, Ankara, Turkey
- Hiroyuki Ueda, MD, Kobe, Japan
- Eleni Vafeiadou, Thessaloniki, Greece
- Pierre Vassallo, MD, PhD, L-Ibragg, Malta
- Nanda Venkatanarasimha, MBBS, MRCP, Plymouth, England
- Ricardo Luis Videla, Córdoba, Argentina
- Christopher P. Vittore, MD, Belvidere, Ill
- Ivan Vollmer, MD, Barcelona, Spain
- Guangyi Wang, MD, Flushing, NY
- Karen Weingarten, MD, New Rochelle, NY
- Steven Thomas Welch, MD, Parkville, Mo
- Jeffrey H. West, MD, Jacksonville, Fla
- Edward W. Williams, MBCHB, British Isles, United Kingdom
- Cecil G. Wood III, MD, Evanston, Ill
- David J. Wright, MD, Lake Oswego, Ore
- Dong Wu, Shanghai, China
- Stanko Yovichevich, MD, Sydney, Australia
- Joe Yut, Olathe, Kan
- Mohammed Zaki, Ube, Yamaguchi, Japan
- Jeffrey H. Zapolsky, MD, Oshkosh, Wis
Resident group responses
- Baylor University Medical Center Radiology Residents, Dallas, Tex
- Diwanchand Imaging and Research Centre Radiology Residents, New Delhi, India
- Hospital Italiano Cordoba Radiology Residents, Cordoba, Argentina
- Hospital Sírio Libanês Radiology Residents, São Paulo, Brazil
- Kaohsiung Medical University Radiology Residents, Kaohsiung, Taiwan
- Kyoto City Hospital Radiology Residents, Kyoto, Japan
- Prince of Songkla University Radiology Residents, Hat Yai, Songkla, Thailand
- Trakya University School of Medicine, Radiology Residents, Edirne, Turkey
- University of Pennsylvania Radiology Residents, Philadelphia, Pa
- Virginia Commonwealth University Radiology Residents, Richmond, Va
TOP
HISTORY
IMAGING FINDINGS
