Adv Pediatr Surg.  2018 Dec;24(2):60-67. 10.13029/aps.2018.24.2.60.

Abdominal Lymphatic Malformation in Children

• Lee S
• Park J

• KMID: 2429802
• DOI: http://doi.org/10.13029/aps.2018.24.2.60

Abstract

PURPOSE
Lymphatic malformations are benign congenital malformations of the lymphatic system that occur predominantly in children. Most lymphatic malformations occur in the head and neck region, with those in abdominal locations, such as the mesentery, omentum and retroperitoneum, being less common, accounting for fewer than 5% of lymphatic malformations in children. This study analyzed the clinicopathologic characteristics and treatment outcomes of abdominal lymphatic malformations in children.
METHODS
The medical records of 12 pediatric patients treated for abdominal lymphatic malformations at our institution between April 1999 and September 2017 were retrospectively reviewed. Demographic and clinical characteristics, including gender, age, symptoms and signs, diagnostic modalities, and treatment results, were analyzed.
RESULTS
The 12 patients included 11 boys and 1 girl, ranging in age from 3 months to 17 years (median 36.5 months) at presentation. The primary signs and symptoms included abdominal pain, abdominal mass and abdominal distention. Other symptoms and signs included fever, vomiting, scrotal pain and mass, and right inguinal mass. All patients were diagnosed by abdominal ultrasonography and computed tomography, and all underwent surgical excision with or without bowel resection. The lymphatic malformations occurred in the retroperitoneum (n=4), omentum (n=4), jejunal mesentery (n=2), and retroperitoneum and mesentery (n=2). Seven patients underwent complete mass excision, including 3 who underwent laparoscopic excisions, and 3 who underwent mass excision with segmental resection of the adjoining bowel. Two patients underwent incomplete excision because the lesion was extensive and invaded the superior mesenteric vessels. There was no major perioperative morbidity in any patient. At a median follow-up of 50 months (range, 8-183 months), only 1 patient experienced recurrence.
CONCLUSION
Although abdominal lymphatic malformations are benign, most children present with acute abdominal symptoms, necessitating early surgical treatment.

Keyword

Mesentery; Abdomen; Lymphangioma; Children

MeSH Terms

Abdomen
Abdominal Pain
Child*
Female
Fever
Follow-Up Studies
Head
Humans
Lymphangioma
Lymphatic System
Medical Records
Mesentery
Neck
Omentum
Recurrence
Retrospective Studies
Ultrasonography
Vomiting

Figure

• 

  Fig. 1 Abdominal ultrasonography of patient No. 7, showing a hypoechoic cystic mass with an ovoid wall, separated by intracystic septation in the lower abdomen.

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  Fig. 2 Abdominal computed tomography scan with intravenous contrast enhancement of patient No. 7, showing a multiloculated cystic mass with a thin wall occupying the lower abdomen.

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  Fig. 3 Photograph of the 9 cm sized round cystic mass removed from patient No. 7. The mass originated from the jejunal mesentery and stretched the adjoining jejunum.

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  Fig. 4 Photograph of the huge retroperitoneal multicystic mass removed from patient No. 9. The mass was attached to the ascending colon and cecum and extended from the retrocecal region medially to the root of the small bowel mesentery, superiorly to the paraduodenal space level and inferiorly to the level of the pelvic inlet.

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