A rare presentation of a mesenteric venolymphatic malformation with spontaneous hemorrhage in a newborn infant: A case report

Introduction: Abdominal venolymphatic malformations are benign rare congenital lymphatic malformations with various presentations. Reported presentations include acute abdomen, intestinal obstruction, torsion, and traumatic hemorrhage. Case Report: We present a case of a 36-week-gestational age female delivered via cesarean section secondary to developing intra-abdominal fluid on antenatal ultrasound. Subsequent imaging, exploratory laparotomy, and pathology were notable for a hemorrhagic left colonic mesenteric venolymphatic malformation with intra-abdominal fresh and coagulated blood. Conclusion: Although rare, previous cases of abdominal venolymphatic malformation have been described. This is the first reported case of spontaneous intrauterine hemorrhage requiring urgent cesarean section. (This page in not part of the published article.) International Journal of Case Reports and Images, Vol. 8 No. 4, April 2017. ISSN – [0976-3198] Int J Case Rep Images 2017;8(4):252–256. www.ijcasereportsandimages.com Mahmoud et al. 252 CASE REPORT PEER REVIEWED | OPEN ACCESS A rare presentation of a mesenteric venolymphatic malformation with spontaneous hemorrhage in a newborn infant: A case report Alaa Mahmoud, Shahla Bari, Dhanashree Rajderkar


INTRODUCTION
Venolymphatic malformations are benign rare lymphatic vessel anomalies that occur often in the neck and axilla [1]. Intra-abdominal cystic venolymphatic malformations are exceedingly rare accounting for less than 5% of cases and often arise from the mesentery, retroperitoneum, and visceral organs. Abdominal venolymphatic malformations have various clinical presentations including acute abdomen, intestinal obstruction, torsion, hemorrhage after trauma and asymptomatic presentations [2,3]. No cases of spontaneous hemorrhage have been reported to our knowledge.

CASE REPORT
A 36-week-gestational age female was delivered urgently via cesarean section due to developing intraabdominal fluid and bowel distention on ultrasound in utero. The mother was a 27-year-old G2P1001 Caucasian otherwise healthy female. Apgar scores post cesarean section was six and nine at one and five minutes respectively. On delivery, the patient required transfusion secondary to anemia (20% hematocrit with normal range being 55-68%). Paucity of gas in the left hemiabdomen was noted on plain films. Ultrasound demonstrated a heterogeneous mass in the left abdomen medial to the spleen and anterior to the left kidney measuring approximately 4.8x3.6x4.9 cm (Figures 1-3). This mass lacked flow on Doppler (Figure 4). Ultrasound also was notable for moderate complex ascites with debris consistent with blood products (Figure 2). Computed tomography scan confirmed the presence a large unilocular well circumscribed mass adjacent to the left colon with smooth margins and associated extensive complex ascites (Figures 5 and 6). Our differential diagnosis was a complicated mesenteric cyst versus hemorrhagic ruptured ovarian cyst.
Exploratory laparotomy was performed which revealed a left colonic mesenteric cystic mass with hemorrhage. Intraperitoneal clot and free peritoneal blood was noted. No bowel resection was required. The gross sample consisted of a 2.2x1.9x0.2 cm aggregate of tan-gray membranous friable tissue with clot. Pathology demonstrated benign cystic fibrovascular tissue septa with simple epithelial lining and rare irregular vascular channels filled with lymphocytes consistent with venolymphatic malformation (Figures 7-9). Postsurgically, the patient did well. The patient now is a year old without complications and is as expected developmentally for age.

DISCUSSION
Venolymphatic malformations account for about five percent of all benign tumors in infants and children. Fifty percent of cases involve the head and neck and only 5% are intra-abdominal [1][2][3]. Other rare anatomic sites have also been reported including the mediastinum, pleura, lungs, pericardium, and bone [4]. Sixty percent of    these tumors are noticed at birth. Almost ninety percent are detected by the mean age of two years. Of the intraabdominal venolymphatic malformations, the majority involve the mesentery of the small bowel. It is believed that these tumors result from an embryological failure of communication between the small bowel lymphatic tissue and the main lymphatic vessels resulting in blind cystic lymphatic spaces lined by endothelial layers.
Clinical presentation is variable and depends on mass size and location. Most abdominal cystic lesions (ACLs) present with a large, slow-growing and mobile mass along with abdominal distention [1]. Abdominal discomfort is common while acute peritoneal symptoms due to rupture, volvulus, hemorrhage or infection occur infrequently.
Ultrasound is the initial, quick and preferred modality to evaluate any suspected abdominal masses in a new     Computed tomography scan is usually performed prior to surgical planning to know the extent of the lesion and to evaluate for any other organ involvement. On CT, these lesions are well defined, uni/multilocular with internal fluid density [1]. However, there may be areas of hyperdensity related to hemorrhage (as in our patient).
Small lesions with no complications can be observed. They are known to regress on their own. Surgical resection remains the treatment of choice in patients who have large lesions. Bowel resection is sometimes necessary if bowel is involved. Regrowth in the residual lesion is the most common complication but recurrence remains low. The use of sclerosing agents is reserved for immediate decompression in the macrocystic variety [5,6].

CONCLUSION
Abdominal venolymphatic malformations are known to have various presentations including acute abdomen, intestinal obstruction, torsion, and traumatic hemorrhage. Cases typically present before the age of two. This is the first case to our knowledge of an abdominal mesenteric venolymphatic malformation with spontaneous hemorrhage in the perinatal age. *********

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