A Case of Peutz-Zeghers Syndrome

ABSTRACT

A case of Peutz-Zeghers syndrome in a year and 8 month old girl was Presented. Her chief complaints were abdominal pain, vomiting and melanin pigmemtation on the lips and perioral area. On family history, her mother and elder sister suffered from same symptom. Physical examination showed melanin pigentation on the lips and perioral area, and markedly distended abdomen. Abdominal simple X-ray film showed fluid level, and so diagnosed as small bowel obstruction. Operation performed at 2nd admission day, and those findings were intusussusception of jejunojejunal type and multiple polyps. The diagnosis was confirmed by family history, characterisic pogmentation and multiple small intestinal polyps of jejunum. Brief review of related literatures were also presented.