Delayed Growth and Puberty Revealing Pituitary Stalk Interruption Syndrome Seen at Befelatanana University Hospital in Antananarivo, Madagascar (Case Report)

Rija Eric Raherison; Thierry Razanamparany; Sitraka Angelo Raharinavalona; Miora Maeva Arielle Andrianiaina; Radonirina Lazasoa Andrianasolo; Andrianirina Dave Patrick Rakotomalala

doi:doi:10.11648/j.ijde.20220703.16

Delayed Growth and Puberty Revealing Pituitary Stalk Interruption Syndrome Seen at Befelatanana University Hospital in Antananarivo, Madagascar (Case Report)

Rija Eric Raherison, Thierry Razanamparany, Sitraka Angelo Raharinavalona, Miora Maeva Arielle Andrianiaina, Radonirina Lazasoa Andrianasolo, Andrianirina Dave Patrick Rakotomalala

Published in International Journal of Diabetes and Endocrinology (Volume 7, Issue 3)

Received: 4 August 2022 Accepted: 27 August 2022 Published: 5 September 2022

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Abstract

Pituitary stalk interruption syndrome (PSIS) is a rare pathology characterized by a triad associating a thin or absent pituitary stalk, small anterior pituitary gland and ectopic posterior pituitary location on pituitary MRI. It is responsible for global or selective impaired production of anterior pituitary hormones. This case concerns a PSIS discovered in 16 years old boy with delayed growth and puberty, bilateral cryptorchidism treated by orchiopexy and asthenia. His personal history was marked by a breech birth with retention of the after-coming head and neonatal asphyxia. The examination revealed a short stature and the lack of signs of puberty, without associated malformation. Biologically, the plasma IGF-1 level was low at 17.6 ng/ml, the plasma total testosterone level was less than 0.4 nmol/l, the plasma FSH level was low at 0.38 mIU/ml, the plasma LH level was less than 0.12 mIU/ml, plasma cortisol level at 8 am was low at 0.6 µg/dl and the plasma TSH and T4L levels were normal. These abnormalities are in favor of somatotropic, gonadotropic and corticotropic insufficiencies. Posterior pituitary’s function appears intact. Pituitary MRI shows a pituitary stalk interruption syndrome. Androgen and glucocorticoid supplementation was initiated. Recombinant growth hormone is not available. Thus, any delays in growth and/or puberty must be carefully explored and managed early, with a multidisciplinary approach to improve patient’s vital, statural and reproductive prognosis.

Published in	International Journal of Diabetes and Endocrinology (Volume 7, Issue 3)
DOI	10.11648/j.ijde.20220703.16
Page(s)	77-80
Creative Commons	This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.
Copyright	Copyright © The Author(s), 2022. Published by Science Publishing Group

Keywords

Anterior Pituitary Hormones, MRI, Growth, Puberty, Pituitary Stalk Interruption Syndrome (PSIS)

References

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APA Style

Rija Eric Raherison, Thierry Razanamparany, Sitraka Angelo Raharinavalona, Miora Maeva Arielle Andrianiaina, Radonirina Lazasoa Andrianasolo, et al. (2022). Delayed Growth and Puberty Revealing Pituitary Stalk Interruption Syndrome Seen at Befelatanana University Hospital in Antananarivo, Madagascar (Case Report). International Journal of Diabetes and Endocrinology, 7(3), 77-80. https://doi.org/10.11648/j.ijde.20220703.16

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ACS Style

Rija Eric Raherison; Thierry Razanamparany; Sitraka Angelo Raharinavalona; Miora Maeva Arielle Andrianiaina; Radonirina Lazasoa Andrianasolo, et al. Delayed Growth and Puberty Revealing Pituitary Stalk Interruption Syndrome Seen at Befelatanana University Hospital in Antananarivo, Madagascar (Case Report). Int. J. Diabetes Endocrinol. 2022, 7(3), 77-80. doi: 10.11648/j.ijde.20220703.16

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AMA Style

Rija Eric Raherison, Thierry Razanamparany, Sitraka Angelo Raharinavalona, Miora Maeva Arielle Andrianiaina, Radonirina Lazasoa Andrianasolo, et al. Delayed Growth and Puberty Revealing Pituitary Stalk Interruption Syndrome Seen at Befelatanana University Hospital in Antananarivo, Madagascar (Case Report). Int J Diabetes Endocrinol. 2022;7(3):77-80. doi: 10.11648/j.ijde.20220703.16

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@article{10.11648/j.ijde.20220703.16,
  author = {Rija Eric Raherison and Thierry Razanamparany and Sitraka Angelo Raharinavalona and Miora Maeva Arielle Andrianiaina and Radonirina Lazasoa Andrianasolo and Andrianirina Dave Patrick Rakotomalala},
  title = {Delayed Growth and Puberty Revealing Pituitary Stalk Interruption Syndrome Seen at Befelatanana University Hospital in Antananarivo, Madagascar (Case Report)},
  journal = {International Journal of Diabetes and Endocrinology},
  volume = {7},
  number = {3},
  pages = {77-80},
  doi = {10.11648/j.ijde.20220703.16},
  url = {https://doi.org/10.11648/j.ijde.20220703.16},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijde.20220703.16},
  abstract = {Pituitary stalk interruption syndrome (PSIS) is a rare pathology characterized by a triad associating a thin or absent pituitary stalk, small anterior pituitary gland and ectopic posterior pituitary location on pituitary MRI. It is responsible for global or selective impaired production of anterior pituitary hormones. This case concerns a PSIS discovered in 16 years old boy with delayed growth and puberty, bilateral cryptorchidism treated by orchiopexy and asthenia. His personal history was marked by a breech birth with retention of the after-coming head and neonatal asphyxia. The examination revealed a short stature and the lack of signs of puberty, without associated malformation. Biologically, the plasma IGF-1 level was low at 17.6 ng/ml, the plasma total testosterone level was less than 0.4 nmol/l, the plasma FSH level was low at 0.38 mIU/ml, the plasma LH level was less than 0.12 mIU/ml, plasma cortisol level at 8 am was low at 0.6 µg/dl and the plasma TSH and T4L levels were normal. These abnormalities are in favor of somatotropic, gonadotropic and corticotropic insufficiencies. Posterior pituitary’s function appears intact. Pituitary MRI shows a pituitary stalk interruption syndrome. Androgen and glucocorticoid supplementation was initiated. Recombinant growth hormone is not available. Thus, any delays in growth and/or puberty must be carefully explored and managed early, with a multidisciplinary approach to improve patient’s vital, statural and reproductive prognosis.},
 year = {2022}
}

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TY - JOUR
T1 - Delayed Growth and Puberty Revealing Pituitary Stalk Interruption Syndrome Seen at Befelatanana University Hospital in Antananarivo, Madagascar (Case Report)
AU - Rija Eric Raherison
AU - Thierry Razanamparany
AU - Sitraka Angelo Raharinavalona
AU - Miora Maeva Arielle Andrianiaina
AU - Radonirina Lazasoa Andrianasolo
AU - Andrianirina Dave Patrick Rakotomalala
Y1 - 2022/09/05
PY - 2022
N1 - https://doi.org/10.11648/j.ijde.20220703.16
DO - 10.11648/j.ijde.20220703.16
T2 - International Journal of Diabetes and Endocrinology
JF - International Journal of Diabetes and Endocrinology
JO - International Journal of Diabetes and Endocrinology
SP - 77
EP - 80
PB - Science Publishing Group
SN - 2640-1371
UR - https://doi.org/10.11648/j.ijde.20220703.16
AB - Pituitary stalk interruption syndrome (PSIS) is a rare pathology characterized by a triad associating a thin or absent pituitary stalk, small anterior pituitary gland and ectopic posterior pituitary location on pituitary MRI. It is responsible for global or selective impaired production of anterior pituitary hormones. This case concerns a PSIS discovered in 16 years old boy with delayed growth and puberty, bilateral cryptorchidism treated by orchiopexy and asthenia. His personal history was marked by a breech birth with retention of the after-coming head and neonatal asphyxia. The examination revealed a short stature and the lack of signs of puberty, without associated malformation. Biologically, the plasma IGF-1 level was low at 17.6 ng/ml, the plasma total testosterone level was less than 0.4 nmol/l, the plasma FSH level was low at 0.38 mIU/ml, the plasma LH level was less than 0.12 mIU/ml, plasma cortisol level at 8 am was low at 0.6 µg/dl and the plasma TSH and T4L levels were normal. These abnormalities are in favor of somatotropic, gonadotropic and corticotropic insufficiencies. Posterior pituitary’s function appears intact. Pituitary MRI shows a pituitary stalk interruption syndrome. Androgen and glucocorticoid supplementation was initiated. Recombinant growth hormone is not available. Thus, any delays in growth and/or puberty must be carefully explored and managed early, with a multidisciplinary approach to improve patient’s vital, statural and reproductive prognosis.
VL - 7
IS - 3
ER -

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Author Information

Rija Eric Raherison

Faculty of Medicine, University of Antananarivo, Antananarivo, Madagascar
Thierry Razanamparany

Faculty of Medicine, University of Antananarivo, Antananarivo, Madagascar
Sitraka Angelo Raharinavalona

Faculty of Medicine, University of Antananarivo, Antananarivo, Madagascar
Miora Maeva Arielle Andrianiaina

Faculty of Medicine, University of Antananarivo, Antananarivo, Madagascar
Radonirina Lazasoa Andrianasolo

Faculty of Medicine, University of Antananarivo, Antananarivo, Madagascar
Andrianirina Dave Patrick Rakotomalala

Faculty of Medicine, University of Antananarivo, Antananarivo, Madagascar

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APA Style

Rija Eric Raherison, Thierry Razanamparany, Sitraka Angelo Raharinavalona, Miora Maeva Arielle Andrianiaina, Radonirina Lazasoa Andrianasolo, et al. (2022). Delayed Growth and Puberty Revealing Pituitary Stalk Interruption Syndrome Seen at Befelatanana University Hospital in Antananarivo, Madagascar (Case Report). International Journal of Diabetes and Endocrinology, 7(3), 77-80. https://doi.org/10.11648/j.ijde.20220703.16

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ACS Style

Rija Eric Raherison; Thierry Razanamparany; Sitraka Angelo Raharinavalona; Miora Maeva Arielle Andrianiaina; Radonirina Lazasoa Andrianasolo, et al. Delayed Growth and Puberty Revealing Pituitary Stalk Interruption Syndrome Seen at Befelatanana University Hospital in Antananarivo, Madagascar (Case Report). Int. J. Diabetes Endocrinol. 2022, 7(3), 77-80. doi: 10.11648/j.ijde.20220703.16

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AMA Style

Rija Eric Raherison, Thierry Razanamparany, Sitraka Angelo Raharinavalona, Miora Maeva Arielle Andrianiaina, Radonirina Lazasoa Andrianasolo, et al. Delayed Growth and Puberty Revealing Pituitary Stalk Interruption Syndrome Seen at Befelatanana University Hospital in Antananarivo, Madagascar (Case Report). Int J Diabetes Endocrinol. 2022;7(3):77-80. doi: 10.11648/j.ijde.20220703.16

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@article{10.11648/j.ijde.20220703.16,
  author = {Rija Eric Raherison and Thierry Razanamparany and Sitraka Angelo Raharinavalona and Miora Maeva Arielle Andrianiaina and Radonirina Lazasoa Andrianasolo and Andrianirina Dave Patrick Rakotomalala},
  title = {Delayed Growth and Puberty Revealing Pituitary Stalk Interruption Syndrome Seen at Befelatanana University Hospital in Antananarivo, Madagascar (Case Report)},
  journal = {International Journal of Diabetes and Endocrinology},
  volume = {7},
  number = {3},
  pages = {77-80},
  doi = {10.11648/j.ijde.20220703.16},
  url = {https://doi.org/10.11648/j.ijde.20220703.16},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijde.20220703.16},
  abstract = {Pituitary stalk interruption syndrome (PSIS) is a rare pathology characterized by a triad associating a thin or absent pituitary stalk, small anterior pituitary gland and ectopic posterior pituitary location on pituitary MRI. It is responsible for global or selective impaired production of anterior pituitary hormones. This case concerns a PSIS discovered in 16 years old boy with delayed growth and puberty, bilateral cryptorchidism treated by orchiopexy and asthenia. His personal history was marked by a breech birth with retention of the after-coming head and neonatal asphyxia. The examination revealed a short stature and the lack of signs of puberty, without associated malformation. Biologically, the plasma IGF-1 level was low at 17.6 ng/ml, the plasma total testosterone level was less than 0.4 nmol/l, the plasma FSH level was low at 0.38 mIU/ml, the plasma LH level was less than 0.12 mIU/ml, plasma cortisol level at 8 am was low at 0.6 µg/dl and the plasma TSH and T4L levels were normal. These abnormalities are in favor of somatotropic, gonadotropic and corticotropic insufficiencies. Posterior pituitary’s function appears intact. Pituitary MRI shows a pituitary stalk interruption syndrome. Androgen and glucocorticoid supplementation was initiated. Recombinant growth hormone is not available. Thus, any delays in growth and/or puberty must be carefully explored and managed early, with a multidisciplinary approach to improve patient’s vital, statural and reproductive prognosis.},
 year = {2022}
}

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