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[The Peutz-Jeghers syndrome and intestinal invagination]
C Baeza Herrera1, L Velasco Soria, S T Domínguez Pérez
1Departamento de Cirugía, Hospital Pediátrico Moctezuma, México, D.F.
Insights
Peutz-Jeghers syndrome, a genetic disorder causing gastrointestinal polyps and skin pigmentation, frequently leads to intussusception. This report details two pediatric cases requiring surgical intervention for this common complication.
Area of Science:
- Genetics
- Gastroenterology
- Pediatric Surgery
Background:
- Peutz-Jeghers syndrome (PJS) is an autosomal dominant genetic disorder.
- PJS is characterized by hamartomatous polyposis throughout the gastrointestinal tract.
- Mucocutaneous melanin pigmentation is a hallmark clinical feature of PJS.
Observation:
- Intestinal obstruction, particularly intussusception, is a significant and frequent complication of PJS.
- This report presents two pediatric cases of Peutz-Jeghers syndrome.
- Both affected patients were female, aged 2 and 8 years.
Findings:
- The two pediatric patients diagnosed with Peutz-Jeghers syndrome presented with intussusception.
- Surgical intervention was required for both cases to address the intussusception.
Implications:
- Early recognition and management of intussusception are crucial in pediatric patients with PJS.
- Surgical treatment remains a primary approach for managing intussusception secondary to PJS.
- Understanding PJS complications informs pediatric care and surgical planning.
Abstract:
Peutz-Jeghers syndrome is a autosomal dominant disorder characterized by hamartomatous polyposis of the gastrointestinal tract and mucocutaneous melanin pigmentation. This condition can occur and cause symptoms at any age, but intestinal obstruction due to intussusception is a frequent complication. Two cases of this syndrome associated to intussusception are reported. Both patients were girls, 2 and 8 years old respectively and underwent surgical treatment.