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Congenital abnormal fusion of adrenal glands

Last edited: 4/16/2026

Overview

Congenital abnormal fusion of adrenal glands, often referred to as adrenal fusion syndrome, is characterized by the fusion of adrenal glands alongside a constellation of anomalies including midline defects (e.g., meningomyelocele, lobar holoprosencephaly), defects of laterality (e.g., short pancreas, small spleen), and placental abnormalities (e.g., undercoiled umbilical cord) 1.

Diagnosis

  • Key Diagnostic Criteria: Fused adrenal glands, midline CNS defects, defects of laterality (short pancreas, small spleen) 1.
  • Recommended Tests:
    • - Imaging studies (ultrasound, MRI) to assess adrenal morphology and associated anomalies 1. - Placental examination to identify umbilical cord abnormalities such as undercoiling and segmental uncoiling 1.

    Management

  • First-Line Treatments:
    • - Address specific organ defects (e.g., surgical intervention for meningomyelocele, supportive care for neurological deficits) 1.
  • Adjunctive Treatments:
    • - Hormonal support based on adrenal function assessment (e.g., cortisol replacement if insufficiency is identified) 1.

    Special Populations

  • Pregnancy: Placental findings, including umbilical cord abnormalities, may provide clues to the underlying syndrome; careful prenatal monitoring recommended 1.
  • Pediatrics: Comprehensive multidisciplinary approach addressing neurological, endocrine, and visceral anomalies is crucial 1.

    • Key Recommendations

  • Conduct thorough imaging and placental examination to diagnose adrenal fusion syndrome, including assessment of adrenal fusion and umbilical cord anomalies (Evidence: Moderate 1).
  • Implement a multidisciplinary management plan tailored to the specific organ system defects identified (Evidence: Expert opinion 1).
  • Consider placental pathology as part of the diagnostic workup to understand the broader spectrum of anomalies associated with adrenal fusion syndrome (Evidence: Moderate 1).

    • References

    1 Drut R, Mónica Drut R. Pathology of the umbilical cord in adrenal fusion syndrome. Pediatric pathology & molecular medicine 2003. link

    Original source

    1. [1]
      Pathology of the umbilical cord in adrenal fusion syndrome. — Drut R, Mónica Drut R Pediatric pathology & molecular medicine (2003)
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