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Clinico-radiological correlation of pituitary stalk interruption syndrome in children with growth hormone deficiency

Subbiah Sridhar1, Bhagadurshah Rameez Raja2, Raghavendran Priyanka2

  • 1Department of Endocrinology, Government Rajaji Hospital and Madurai Medical College, Madurai, Tamil Nadu, 625020, India. drsridharjipmer@gmail.com.

Pituitary
|September 11, 2023
PubMed

Insights

Pituitary stalk interruption syndrome (PSIS) in children with growth hormone deficiency (GHD) is associated with specific clinical features and often presents with multiple pituitary hormone deficiency. Early diagnosis and growth hormone treatment improve outcomes.

Area of Science:

  • Pediatric Endocrinology
  • Neuroendocrinology
  • Radiology

Background:

  • Pituitary stalk interruption syndrome (PSIS) is a rare congenital condition.
  • It is a significant cause of growth hormone deficiency (GHD) in children.
  • Understanding its characteristics is crucial for diagnosis and management.

Purpose of the Study:

  • To analyze the clinical, hormonal, and radiological features of PSIS in children with GHD.
  • To identify factors associated with PSIS.
  • To evaluate treatment outcomes in PSIS patients.

Main Methods:

  • Prospective cross-sectional study over three years.
  • Inclusion of 57 children with severe short stature and proven GHD.
  • Clinical evaluation, hormonal assays, and Magnetic Resonance Imaging (MRI).

Main Results:

  • 14% of children with GHD were diagnosed with PSIS.
  • PSIS patients often had multiple pituitary hormone deficiency (MPHD) compared to isolated GHD (IGHD).
  • Arginine vasopressin (AVP) deficiency was rare despite absent or ectopic posterior pituitary (EPP) on MRI; height gain with GH therapy was better in PSIS.

Conclusions:

  • Male gender, breech presentation, and congenital anomalies are more common in PSIS.
  • MPHD is frequent in PSIS, while IGHD is more common in non-PSIS.
  • Early diagnosis of PSIS in severe short stature is vital for timely GH treatment and improved outcomes.
Abstract

Keywords:
CPHDCombined pituitary hormone deficiencyGrowth hormone deficiencyIGF-1IndiaMPHDMultiple pituitary hormone deficiencyPituitary stalk interruption syndromeShort stature

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