Abdominal Distention Caused by Cystic Lymphangioma in a Neonate

Abdominal Distention Caused by Cystic Lymphangioma in a Neonate

J Exp Clin Med 2013;5(4):148–149 Contents lists available at SciVerse ScienceDirect Journal of Experimental and Clinical Medicine journal homepage: ...

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J Exp Clin Med 2013;5(4):148–149

Contents lists available at SciVerse ScienceDirect

Journal of Experimental and Clinical Medicine journal homepage: http://www.jecm-online.com

LETTER TO THE EDITOR

Abdominal Distention Caused by Cystic Lymphangioma in a Neonate Lymphangiomas constitute approximately 5% of all benign tumors in infants and children younger than 10 years; the diagnosis is made in approximately 80–90% of these children within the first few years of life and adult cases are rare.1 The age of patients with abdominal cystic lymphangioma in a report ranged from 8 days to 6 years.2 Although lymphangiomas are commonly found in the head, neck, and axillary region, the most common location of abdominal cystic lymphangioma in children appears to be the mesentery of the small bowel.3 We report an unusual case with

rapid growth of a retroperitoneal cystic lymphangioma within 3 days. A previously healthy 10-day-old girl presented with a 3-day history of milk vomitus, with subsequent development of abdominal distention for 1 day. Her medical history was unremarkable after birth. Her temperature was 95.8 F, blood pressure was 91/ 58 mmHg, heart rate was 110 beats/min, oxygen saturation was 99%, and Glasgow Coma Scale score was 12. The patient had signs of respiratory distress and the gag reflex was intact. She had no

Figure 1 (A) Initial abdominal distention. (B) Abdominal computed tomography shows a huge cystic lymphangioma measuring approximately 12.0 cm  9.0 cm. (C) Exploratory laparotomy revealed multicystic lymphangioma from retroperitoneal area (arrows). (D) Microscopic features of cystic lymphangioma (hematoxylin and eosin, 400). 1878-3317/$ – see front matter Copyright Ó 2013, Taipei Medical University. Published by Elsevier Taiwan LLC. All rights reserved. http://dx.doi.org/10.1016/j.jecm.2013.06.001

Letter to the Editor

fever or jaundice. She was admitted to the pediatric intensive care unit due to persistent nonbloody, nonbilious vomiting and lethargy. Her abdominal examination was significant for periumbilical tenderness with severe abdominal distention (Figure 1A). There was no visible rash, bruising, or bleeding. Her laboratory evaluation was notable for an elevated white blood cell count of 19.0  109/L with a lymphocytic predominance, a hematocrit of 34.3 g/dL, and an elevated C-reactive protein level of 178/mL. Her electrolyte and liver panels, amylase, lipase, coagulation panel, creatinine kinase, troponin-I, ammonia, extended serum, and urine toxicology screens were within normal limits. Subsequent bacterial cultures of blood, urine, and the throat were sterile. Stool samples sent for bacterial culture, ova, and parasites as well as Clostridium difficile toxins were negative. A plain film of the abdomen showed several dilated loops of sigmoid colon without evidence of an air-fluid level. Abdominal ultrasonography revealed a large, multilocular, cystic mass with an obscure margin. Using contrast medium, computed tomography of the abdomen showed a large homogeneous mass measuring 12  9  7 cm in size with multiple enhancement septa. This lesion was located at the anterior aspect of the left kidney and descending colon, causing compression and stretching of the sigmoid colon (Figure 1B). After consultation with the pediatric surgeon, laparotomy was performed. A yellowish cystic tumor with soft consistency was found in the mesentery of the retroperitoneum. The tumor was adhered to the wall of the sigmoid colon and perirectal area (Figure 1C). Ascites was not seen in the peritoneal cavity, nor was there any dilatation or inflammatory change of the small intestines or mesentery. With careful dissection of the mesenteric arteries, the tumor was excised completely without resection of the sigmoid colon. Microscopically, the sections showed numerous dilated lymphatic channels of varying sizes within loose fibroconnective tissue and a few disorganized bundles of smooth muscle present in the wall of the larger channels. Immunohistochemical stains were positive for Factor VIII and actin, as well as CD31 and CD34. The same cells did not react to cytokeratin. These findings were consistent with a cystic lymphangioma (Figure 1D). The patient had an uneventful postoperative course and no evidence of recurrence 18 months after the operation. The main treatment for abdominal cystic lymphangioma is surgical excision. However, cystic lymphangiomas may cause complications such as infiltration of the intestine or involvement of the main branch of mesenteric arteries or adjacent organs, requiring segmental resection of the intestine. Although some reports state that the resection could be performed using a

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laparoscopic technique without large abdominal incisions in children,4 sometimes complete resection might be technically impossible and local recurrence may occur, even during adulthood. Sclerosis techniques constitute an alternative and complementary treatment approach. Intracystic sclerotherapy using doxycycline is possible for symptomatic tumors associated with diffuse mesenteric lesions that are not resectable without extensive intestinal sacrifice in recent reports.5 Retroperitoneal cystic lymphangioma is rarely reported in infants, and pediatric practitioners should be aware of this condition, especially when evaluating newborns who present with progressive abdominal distention and nausea with vomitus after milk feeding. Surgical resection of the lesion is the main treatment modality, and long-term follow-up is advised in the case of incomplete resection of cystic lymphangioma. References 1. Tsukada H, Takaori K, Ishiguro S, Tsuda T, Ota S, Yamamoto T. Giant cystic lymphangioma of the small bowel mesentery: report of a case. Surg Today 2002;32:734–7. 2. Luo CC, Huang CS, Chao HC, Chu SM, Hsueh Cl. Intra-abdominal cystic lymphangiomas in infancy and childhood. Chang Gung Med J 2004;27:509–14. 3. Mendez-Gallart R, Bautista A, Estevez E, Rodríguez-Barca P. Abdominal cystic lymphangiomas in pediatrics: surgical approach and outcomes. Acta Chir Belg 2011;111:374–7. 4. Solari V, Mullassery D, Lansdale N, Jesudason EC. Laparoscopic excision of a retroperitoneal lymphatic malformation in a newborn. J Pediatr Surg 2011;46: e15–7. 5. Khattala K, Rami M, Elmadi A, Mahmoudi A, Bouabdallah Y. Giant cystic lymphangioma of the small bowel mesentery: case report. Pan Afr Med J 2011;9:46.

Sheng-Chieh Lin Department of Pediatrics, Shuang Ho Hospital, Taipei Medical University, New Taipei City, Taiwan Hon-Hua Lun Department of Surgery, Taipei Medical University Hospital, Taipei Medical University, Taipei, Taiwan Pi-Hua Chen* Department of Obstetrics and Gynecology, Shuang Ho Hospital, Taipei Medical University, New Taipei City, Taiwan * Corresponding author. Pi-Hua Chen, Number 291, Zhongzheng Road, Zhonghe District, New Taipei City, Taiwan. E-mail: P.-H. Chen <[email protected]> Mar 28, 2013