A 6-month-old girl with cystic fibrosis develops recurrent episodes of cramping abdominal pain with a 'target sign' and 'crescent sign' on ultrasound. Successful air enema reduction is performed. What is the most likely diagnosis and pathological lead point in this age group with CF?
- A Intussusception with inspissated meconium as the lead point
- B Intussusception; in CF, inspissated mucus / thickened lymphoid tissue from repeated infections acts as the lead point; Meckel's diverticulum is more common in children >2 years ✓
- C Hirschsprung's disease causing functional obstruction
- D Meconium ileus equivalent in infancy
Explanation
Intussusception in CF occurs with higher frequency than in the general population due to inspissated mucus in the bowel and thickened Peyer's patches/mesenteric lymph nodes acting as lead points. The target sign (concentric rings on transverse US) and crescent sign confirm intussusception. In children <2 years, ileo-colic intussusception is typically idiopathic (enlarged Peyer's patches post-viral). In CF, intestinal mucus impaction and recurrent infections predispose to recurring intussusception. Pathological lead points (Meckel's diverticulum, polyp, lymphoma) are more common in older children (>2–3 years). Air enema is both diagnostic and therapeutic; reduction rate is approximately 85–90%.
Reference: Bailey & Love's Short Practice of Surgery, 27th ed.
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Written and medically reviewed by the StethoPrep medical team.