Triple Vascular Compression in an Adolescent: A Report of a Rare Case

TL;DR

A 14-year-old adolescent with rare triple vascular compression syndromes experienced severe symptoms requiring hospitalization and surgery for long-term relief.

Contribution

This case report documents a rare co-occurrence of SMAS, NCS, and MTS in an adolescent, emphasizing diagnostic and management challenges.

Findings

Triple vascular compression syndromes (SMAS, NCS, MTS) were confirmed in a 14-year-old with severe symptoms.

Conservative treatments failed, necessitating surgical intervention (gastrojejunostomy) for symptom relief.

A multidisciplinary approach improved outcomes and quality of life after surgery.

Abstract

Superior mesenteric artery syndrome (SMAS), or Wilkie’s syndrome, is a rare cause of intestinal obstruction due to duodenal compression between the aorta and superior mesenteric artery (SMA). It is often linked to weight loss and anatomical variations that reduce the mesenteric fat pad, worsening the compression. Delayed diagnosis can lead to severe complications, including malnutrition. Similarly, nutcracker syndrome (NCS) and May-Thurner syndrome (MTS) involve vascular compressions that may result in serious complications if not promptly managed.

A 14-year-old previously healthy adolescent developed progressive postprandial abdominal pain and vomiting for six months, leading to severe weight loss. This led to multiple emergency visits for dehydration, requiring IV fluids and hospitalization. Symptoms worsened after a growth spurt, causing malnutrition. A CT scan confirmed SMAS, alongside NCS and MTS. Due to renal impairment, he required prolonged hospitalization with parenteral nutrition and psychological support. Symptoms recurred at 16 and 18 years old, leading to further hospitalizations. Given his poor quality of life and failed conservative treatment, a gastrojejunostomy was performed at 18 years old, resulting in a favorable outcome.

This case highlights the diagnostic challenges of SMAS and its frequent association with vascular compression syndromes. Early recognition of persistent vomiting, weight loss, and dehydration is crucial. A multidisciplinary approach, including nutritional, surgical, and psychological support, optimizes outcomes. Primary care physicians play a key role in early diagnosis, referral, and long-term management to prevent complications and improve quality of life.

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