Clinical features of mesenteric lymphatic malformation in children.
BACKGROUND
Mesenteric lymphatic malformations (MLs) are a heterogeneous group of benign diseases of the lymphatic system that present with cystic dilated lymphatics of the mesentery. MLs are rare and represent less than 5% of all lymphatic malformations. The aims of this study were to analyze the characteristics of MLs in children and to suggest a modified classification.


METHODS
We investigated 25 patients who underwent ML surgery. The clinical data and pathological findings were reviewed retrospectively. We divided the patients into 4 groups according to the operative findings. Group 1 included patients with MLs involving the intestinal walls. Group 2 included patients with pedicle-type MLs with no relationship to the mesenteric vessels. Group 3 patients presented with MLs located in the mesenteric boundaries near the mesenteric vessels. Group 4 patients had multicentric and diffusely infiltrated MLs.


RESULTS
The male-to-female ratio was 11:14, and the median age at diagnosis was 5years of age. The most common symptom was abdominal pain. The jejunal mesentery was the most frequently involved site in this study. Five patients showed the macrocystic type and 20 patients showed the mixed cystic type. With the exception of one patient with a large mixed cystic-type ML who underwent incomplete mass excision, 24 patients underwent complete mass excision. The group 1 patients (n=14) underwent mass excision performed with segmental resection of the bowel. The group 2 patients (n=3) only underwent mass excision surgery. The patients in group 3 (n=7) underwent mass excision with segmental resection of the intestine because ML excision altered the blood supply of the adjacent intestines. The group 4 patients (n=1) presented with MLs involving the entire mesentery and underwent incomplete excision.


CONCLUSIONS
The relationships between MLs and the neighboring organs determine the surgical strategy, and the size and location of MLs affect the operative methods. The modified classification based on these findings can facilitate effective treatment planning.
Department of Pediatric Surgery, Pusan National University Yangsan Hospital, Yangsan, Korea.
Soo-Hong
SH
Kim
Department of Pediatric Surgery, Seoul National University Children's Hospital, Seoul, Korea. Electronic address: spkhy02@snu.ac.kr.
Hyun-Young
HY
Kim
Department of Pathology, Seoul National University Hospital, Seoul, Korea.
Cheol
C
Lee
Department of Pathology, Seoul National University Hospital, Seoul, Korea.
Hye Sook
HS
Min
Department of Pediatric Surgery, Seoul National University Children's Hospital, Seoul, Korea.
Sung-Eun
SE
Jung
D015746:Abdominal Pain / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D018191:Lymphangioma, Cystic / Q000145:classification / Q000473:pathology* / Q000601:surgery; D044148:Lymphatic Abnormalities / Q000473:pathology; D042601:Lymphatic Vessels / Q000473:pathology; D008297:Male; D008639:Mesenteric Cyst / Q000145:classification / Q000473:pathology* / Q000601:surgery; D008643:Mesentery / Q000473:pathology; D010534:Peritoneal Neoplasms / Q000473:pathology* / Q000601:surgery; D012189:Retrospective Studies; D016896:Treatment Outcome; D014839:Vomiting / Q000209:etiology
Children; Classification; Lymphangioma; Lymphatic malformation; Mesenteric; Treatment