Abdominal Cystic Lymphangioma in Children

Transcription

1 Annals of Pediatric Surgery Vol 5, No 2, April 2009, PP Original Article Abdominal Cystic Lymphangioma in Children Hisham Fayad Aly Department of Pediatric Surgery, Faculty of Medicine, Tanta University, Tanta, Egypt Background/ Purpose: Abdominal cystic lymphangiomas are rare entities in children. They are known to be the result of mal development of lymphatic vessels. This study was undertaken to report the various pattern of presentation, the management and outcome of children with intraabdominal cystic lymphangioma. Materials & Methods: The medical records and imaging studies of 13 patients with intraabdominal cystic lymphangioma treated at the department of surgery Tanta University from 2001 to 2009 were reviewed. The data included the age, sex, clinical presentation, imaging studies, operative findings, histological features, and outcome. Results: This study included 13 children (8 boys and 5 girls Their ages ranged between 1 week and 9 years with a mean age 3 years. The main presenting symptoms were abdominal pain (n=7) with or without palpable abdominal mass. Four patients were diagnosed antenatally by ultrasonography as having intraabdominal cystic lesions. Preoperative ultrasounds were diagnostic for a cystic mass in all other patients. Four patients required emergency surgery and eight underwent elective intervention. The cysts were located in the mesentery of small bowel in 6 cases, in the base of the mesentery with retroperitoneal extension in 5 cases, and the transverse mesocolon in 2 cases. Complete excision of the cyst with or without limited resection of the involved bowel was feasible in 10 of 13 patients; while marsupialization was done in 3 cases one of them developed recurrence 2 years after surgery. Conclusion: Accurate preoperative diagnosis is possible with maintaining a high index of suspicion and liberal use of ultrasonic imaging and computed tomography (CT). Retroperitoneal cysts should be considered a different entity from mesenteric cysts even though they present clinically in a similar fashion. Complete cyst resection is the procedure of choice and results in an excellent outcome. Index Word: Cystic lymphangioma, children, recurrence, mesenteric cyst. INTRODUCTION ntra abdominal cystic lymphangiomas (CL) in I children are rare. They are considered to be congenital malformation due to an abnormality in lymphatic development. These lesions are generally reported in children and young adults. 1,2 The incidence of CL with intraperitoneal and retroperitoneal localization is reported to be 1 to 4 per ,3 The clinical findings are variable and usually not helpful in establishing the diagnosis, which may present a great preoperative diagnostic difficulty. 1,4 Cases of intraabdominal cystic lymphangioma are often misdiagnosed as having appendicitis, Meckel's diverticulitis, or duplication cyst. 5 Handelsman and Ravitch reported a cystic mass that was observed on the mesentery of intestine during the exploration of 4 cases for intestinal obstruction. 6 Complications of CL as infection, hemorrhage, torsion or perforation may be the presenting features. 7 Correspondence to: Hesham Fayad M.D, Department of Pediatric Surgery, Faculty of Medicine, Tanta University, Tanta, Egypt.

2 Fayad H The cysts are thought to arise from lymphatic spaces associated with the embryonic retroperitoneal lymph sac, analogous to cystic lymphangioma of the neck Development of the lymphatic system is not understood clearly, but it appears to be a failure of communication between lymphatic vessels during intrauterine life. 8 Godart reported that fetal lymphatic sacs that have defective connection with the venous system develop irregularly and induce malformation of the lymphatic system. 9 This lymphatic venous network grows abnormally and regional dysplasia develops. Sencan et al explained occurrence of CL due to either centrifugal (proliferation from the venous system) or centripetal theory (coalescence of the mesenchymal spaces). 8 Histologically, cystic lymphangiomas are usually multilocular masses with thin or thick wall and lined by flat endothelium containing lymphatic spaces and collections of lymphoid tissue, foam cells and smooth muscle cells in the cyst wall. 9 This study was undertaken to report the various pattern of presentation, the management and outcome of children with intraabdominal cystic lymphangioma. PATIENTS AND METHODS In this retrospective study, the clinical records and the imaging studies of 13 patients with intraabdominal lymphangiomas treated at the department of surgery, Tanta University from 2001 to 2009 were reviewed. The clinical data included the age, sex, clinical presentation, and duration of symptoms prior to the correct diagnosis, diagnostic studies, surgical intervention, histological results and follow up. Abdominal ultrasound was done to all patients and a computed tomography (CT) scan of the abdomen was done to 8 patients. The diagnosis was proven in all cases at laparotomy as well as by histological examination of each surgical specimen RESULTS This study included a total number of 13 patients (8 males and 5 females) with intraabdominal cystic lymphangioma. Their ages ranged between 1 week and 9 years with a mean age of 3 years. The main presenting symptoms were abdominal pain (n=7) with or without palpable abdominal mass. Two children presented with marked abdominal distension due to huge mesenteric cyst, which were misdiagnosed initially as ascites. Other symptoms included constipation (n=3) and anemia (n=2). Four patients were diagnosed antenatally by ultrasonography as having intraabdominal cystic lesions. The cysts were located in the mesentery of small bowel in 6 cases, in the base of the mesentery with retroperitoneal extension in 5 cases, and the transverse mesocolon in 2 cases. Four patients required emergency surgery for complicated mesenteric cysts (intestinal obstruction, intracystic hemorrhage and infection). The remaining eight patients underwent elective intervention. Investigation: Ultrasonography was diagnostic for cystic masses in 9 patients. The accurate preoperative diagnosis of cystic lymphangioma was possible in 7 of these 9 patients. The other 2 patients were diagnosed as ovarian cyst or duplication cysts. Eight patients had CT studies that showed large single (n=3) or multiseptated cystic (n=5) lesion containing fluid of water density. Seven of these 8 patients were correctly diagnosed as intraabdominal cystic lymphangioma. Definitive preoperative diagnosis of cystic lymphangioma was possible in 9 of the 13 patients. The diagnosis of mesenteric lymphangioma was only made intraoperatively in 4 patients. Three of them presented with manifestations of intestinal obstruction. Two of these 4 patients had only plain x ray prior to the emergency surgery for the bowel obstruction and were misdiagnosed as ovarian cyst (n=1) or duplication cyst (n=1) Management: The four patients with complicated cysts required emergency surgery. The bowel obstruction was found at the descending colon due to mechanical compression of the colon by inflamed cyst in 2 patients. Another patient had a large mass adherent to the small bowel mesentery at the proximal jejunum and the last one had mass adherent to the ileocecal region. Complete resection of the cyst only was done in one patient. Two patients had bowel resection including the cyst and one patient had marsupialization of the cyst due to severe adhesion to the colon and non feasibility of complete excision 133 Vol 5, No 2, April 2009

3 Fig 1: Multiple cysts located at the mesentery of ileum in a 2 week old neonate presented with abdominal mass which was diagnosed prenatally. Fig 2: Four mesenteric cysts were excised completely without bowel resection Fig 3: Retroperitoneal cysts located at the mesentery of cecum and ascending colon in a 3 year old female presented with abdominal mass Fig 4: Four mesenteric cysts were excised completely without bowel resection Fig 5: multiple cysts located at the mesentery of jejunum containing chylous fluid in a 4-year-old boy presented with abdominal pain Fig 6 Limited bowel resection was mandatory to ensure complete excision of the cysts Annals of Pediatric Surgery 134

4 Fayad H The other 9 patients had elective surgery. Complete excision of the cyst was possible in 7 of those patients. Limited resection of the involved bowel was required in 2 of them. Two patients had marsupialization of the cyst. The histopathological studies confirmed the presence of a layer of a flat endothelium containing lymphatic spaces in all patients. Cysts contain serous fluid in 7 patients, serosangenous fluid in 4 and infected serous fluid in 2 patients. Outcome: Follow up ranged from 3 months to 8 years. There was no mortality in the present series. Recurrence occurred in one patient who had incomplete excision that required another marsupialization of the multiple retroperitoneal cysts. DISCUSSION Cystic lymphangiomas are uncommon benign tumors of congenital origin. Symptomatic mesenteric cyst accounts for only 1 in in acute adult and 1 in in acute pediatric admission. 11 Symptoms usually present due to compression caused by the mass. Abdominal lymphangioma may develop in the mesentery of the intestine, omentum or retroperitonium. 12 Nearly one third of cases involve children less than 15 years of age. 13 Presentation of such cysts depends on the size and location. Abdominal pain with or without palpable mass was the most common presenting symptom in our cases (53.8 %), followed by presence of abdominal pain and mass (29.5%). Palpable masses are reported in 25% to 28% of cases. 14,15 On the other hand, other authors reported palpable masses in 77% of cases. 12 Abdominal pain may be secondary to bleeding, infection, torsion or compression. 16 Kosir et al reported a 38% bowel obstruction associated with cystic lymphangioma in their cases. 12 In the current series we have (30.7%) cases of bowel obstruction due to cystic lymphangioma. Plain film may show displacement of bowel loops or opacification. Ultrasound is usually able to define the cystic mass, its septation and wall thickness. Computed tomography is more specific in defining anatomic location, size and adjacent organ involvement. 17. Medial displacement of the bowel and colon with visible septation is indicative of an abdominal lymphangioma and not ascitis. 18 The optimum treatment of cystic lymphangioma is complete resection. Surgical intervention is recommended as soon as possible following diagnosis because of the risk of torsion, infection, hemorrhage and obstruction. For large multiple lesions that are intimately adherent to the bowel and mesenteric vessels, bowel resection is often necessary. A 10% incidence of recurrence had been reported with incomplete resection. 1,12 Complete excision of the cyst with or without limited resection of the involved bowel was feasible in 10 cases of 13 patients (77%). Complete Excision was not feasible in 3 patients with severe inflammation and or extensive retroperitoneal involvement. Excision of a number of cysts and marsupialization of the remaining cysts were done. Recurrence occurred in one of these 3 patients. Bowel resection was required in 4 patients to enable complete resection of the cysts. Kosair et al reported 6.1 % bowel resection with the cyst. 12 whereas Kwetz et al reported 30% bowel resection. 19 There were no mortalities in this study. A 3% mortality rate has been reported in a review of the literature. 19 None of our cases were operated laparoscopically. although this approach will be considered in the future due to the reported minimal morbidity, short hospital stay and faster recovery. 20 CONCLUSION Intraabdominal cystic lymphangioma is a benign lesion that does not regress. It causes symptoms because of the mass effect. Although rare, cystic lymphangioma should be considered when children have associated vague abdominal complaint. Liberal use of ultrasonography in evaluation of children with vague and recurrent abdominal pain is highly advocated. Computed tomography scan findings of septation and medial displacement of bowel contents are classic for cystic lymphangioma. Complete excision of the cyst is the optimal treatment. Bowel resection might be necessary to ensure complete excision of the cyst. Once the cyst is excised, the prognosis is excellent 135 Vol 5, No 2, April 2009

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