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J Ped Surg Case Reports 2 (2014) 302e304

Journal of Pediatric Surgery CASE REPORTS

journal homepage: www.jpscasereports.com

Successful treatment of visceral infantile hemangioma of theomentum and mesentery with propranololq

Jill Carol Rubinstein a, Emily Rachel Christison-Lagay a,b,*

aYale University School of Medicine, Department of General Surgery, P.O. Box 208062, New Haven, CT 06520-8062, USAb Yale University School of Medicine, Department of Pediatric Surgery, P.O. Box 208062, New Haven, CT 06520-8062, USA

a r t i c l e i n f o

Article history:Received 18 April 2014Received in revised form2 June 2014Accepted 4 June 2014

Key words:Infantile hemangiomaVisceralPropranolol

q This is an open access article under the CCcreativecommons.org/licenses/by-nc-nd/3.0/).* Corresponding author. Yale University School o

General Surgery, P.O. Box 208062, New Haven, CT 06522701; fax: þ1203 785 3820.

E-mail addresses: emily.christison-lagay@yale.edu,(E.R. Christison-Lagay).

2213-5766/$ e see front matter � 2014 The Authors.http://dx.doi.org/10.1016/j.epsc.2014.06.004

a b s t r a c t

Infantile hemangiomas (IH) are the most common tumors of infancy. In the typical cutaneous presen-tation, they follow a predictable and benign clinical course requiring medical intervention only forgrowth that interferes with vision or respiration, or, rarely, in cases of intractable bleeding. Hemangi-omas arising from within visceral structures or surfaces are far less common, but can be associated withincreased morbidity and mortality secondary to gastrointestinal hemorrhage, abdominal compartmentsyndrome, high output cardiac failure, and hypothyroidism. Here we present the case of a three-week-old boy with acute abdomen caused by hemorrhage of a hemangioma of the omentum and mesentery.He underwent operative exploration with debulking of the lesion and was subsequently treated withpropranolol. Serial imaging demonstrated gradual involution and he remained free of further life-threatening events.

� 2014 The Authors. Published by Elsevier Inc. All rights reserved.

Infantile hemangioma (IH) is themost common tumor of infancywith an incidence between 1 and 9%, varying by race [1]. The mo-lecular mechanisms underlying pathogenesis remain incompletelyunderstood, but the clinical course follows a stereotyped pattern: aphase of early vascular proliferation over the first year of life fol-lowed by a gradual phase (1e7 years in duration) of spontaneousinvolution and replacement of vascular channels by fibro-fattytissue. Most cutaneous lesions follow a benign course. In theabsence of bleeding, ulceration, or impairment of vision or respi-ration, treatment consists of observation and reassurance [2]. It hasbeen suggested that the presence of five or more cutaneous hem-angiomas should arouse suspicion for the possibility of viscerallesions [3], which are associated with higher rates of morbidity.

Visceral hemangiomas, most common in the liver, may beassociated with high output cardiac failure, coagulopathy, abdom-inal compartment syndrome, or respiratory distress [4,5]. Cases ofsmall bowel perforation, intussusception, and gastrointestinalhemorrhage from hemangiomatosis have been reported [6e8].

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f Medicine, Department of0-8062, USA. Tel.: þ1203785

echristisonlagay@gmail.com

Published by Elsevier Inc. All right

Here we describe a unique presentation of an omental andmesenteric IH in a newborn boy, which hemorrhaged and caused aninflammatory obstruction of the small bowel. After operativeexploration, lysis of adhesions, and partial debulking of the lesion,post-operative treatment with propranolol was initiated and thepatient was followed with serial imaging. One year after initiationof treatment, the hemangioma had largely regressed and heremained symptom free.

1. Case report

A three-week-old boy born via emergent caesarian section (36days, 6 weeks gestation) due to maternal HELLP syndrome and pre-eclampsia, was noted shortly after birth to have multiple heman-giomas on his lower lip, chin, and bilateral pre-auricular regions inaddition to one sublingual lesion. He was discharged homewithoutincident, but on day of life nineteen had decreased oral intake, ir-ritability, and non-bilious emesis. He became febrile and lethargicwith a distended, firm abdomen and was admitted to the intensivecare with presumed sepsis. An abdominal x-ray demonstrateddilated loops of small bowel and he had multiple heme-positivesmall bowel movements. His infectious work-up remained negativewhile his abdomen became increasingly firm and bowel move-ments ceased. Laboratory values were significant only for a nor-mocytic anemia, with a hematocrit of 30.1%. Ultrasounddemonstrated a 4.3 � 1.9 cm complex collection in the upper

s reserved.

Fig. 1. Abdominal ultrasound demonstrating 4.3 � 1.9 cm complex fluid collectionwithmultiple, thick, internal septations and adjacent thickened loops of bowel.

Fig. 2. Immunohistochemistry showing GLUT-1 positivity (20�).

J.C. Rubinstein, E.R. Christison-Lagay / J Ped Surg Case Reports 2 (2014) 302e304 303

abdomenwith multiple internal septations, adjacent thickened andhyperemic loops of bowel, and a large amount of free fluid withechogenic debris (Fig. 1). No solid organ visceral hemangiomaswere appreciated.

Given a differential diagnosis that included visceral hemangioma,macrocystic lymphatic malformation, and bowel perforation withadjacent collection, the patient was taken for operative exploration.Dark red-brown, clear, non-purulent fluid was encountered uponentering the abdomen. There was no evident bowel ischemia, butthere was an inflammatory process with a resultant band causingobstruction of several loops of adjacent small bowel. Further dissec-tion revealed a complex, beefy red, bosselated soft tissuemass arisingfromtheomentumbetween the greater curvature of the stomachandtransverse colon, extending laterally along the splenic flexure andinferiorly along the descending colon. This tissuewas dissected off ofthe adjacent colonand resectedposteriorly to the retroperitoneumonthe anterior surface of the pancreas. It was felt that continuing thedissection to include stripping the peritonealized surface of thecolonicandsmallbowelmesenteryaswell as thepancreasandsplenichilum was too risky and the decision was made to leave a smallamount of tissue abutting the pancreas, splenic hilumandmesentericpedicles to the colon and small bowel. There was a small (sub-centimeter) hemangioma present on segment 2 of the liver, but noevidence of diffuse hepatic involvement.

Pathology revealed an aggregate of irregular, pink-red, diffuselyhemorrhagic and necrotic soft tissues with well-formed capillaryspaces grouped in a lobular fashion, consistent with hemangioma.The cells were subsequently shown to be positive for GLUT-1, butnot D2-40 further supporting the diagnosis of IH and making a

Fig. 3. a) T2 weighted, gadolinium enhanced MRI of the abdomen two months after initial psplenic vein, celiac artery, SMA, and IVC. b) T2 weighted, non-contrast enhanced MRI six mmesenteric hemangioma and a small decrease in the size of the retroperitoneal componen

combined vascular-lymphatic malformation less likely (Fig. 2). Thepatient was started on propranolol prior to discharge home. He wasreadmitted one week later with abdominal distension, increasedfussiness, and difficulty sleeping. Ultrasound showed a large,complex fluid collection in the left abdomen and InterventionalRadiology placed a drain with successful resolution of the cavity.Studies of the drain fluid were not consistent with chylous ascites.

The patient remained on propranolol with surveillance MRIperformed two months after initial presentation revealing persis-tent hemangioma along the root of the mesentery, encasing thesplenic vein, celiac artery, SMA, and IVC (Fig. 3a). Six months afterinitial presentation, repeat MRI showed substantial decrease in thesize of the mesenteric hemangioma with slight decrease in theextent of the retroperitoneal component (Fig. 3b). Clinically, hecontinued to do well, meeting all developmental milestones withappropriate weight gain. MRI one year after initial presentationrevealed near complete resolution of the lesion (Fig. 4).

2. Discussion

This is an unusual case of visceral IH presenting not withgastrointestinal bleeding, but rather as intra-abdominal

resentation demonstrating hemangioma along the root of the mesentery, encasing theonths after initial presentation demonstrating a significant decrease in the amount ofts.

Fig. 4. Repeat T2 weighted, contrast enhanced MRI 14 months after presentationdemonstrating near complete resolution of the hemangioma along all previouslyinvolved surfaces.

J.C. Rubinstein, E.R. Christison-Lagay / J Ped Surg Case Reports 2 (2014) 302e304304

hemorrhage. In 2008, Leaue-Labreze et al. reported a series of 11cases of infantile hemangioma treated with oral propranolol [9].The treatment effect was suspected after a child taking propranololfor cardiomyopathy demonstrated rapid involution of his nasalhemangioma. Multiple, small, retrospective case series followed,confirming the effect and making propranolol a first-line therapyfor IH, even in the absence of large, randomized, controlled trials[10e12]. In 2011, the first (albeit small) randomized, double-blin-ded study demonstrated the superior effect of propranolol overplacebo, with a significant reduction in volume, color, and elevationof IH in the treatment group and no significant side effects [13]. A2013 meta-analysis pooled 35 studies to demonstrate propranolol’ssuperior efficacy over steroids, vincristine, and laser treatmenthowever only 6 of the 324 cases were visceral in origin (all hepatic)[14]. Further literature search reveals reports of 15 hepatic lesionssuccessfully treated with propranolol [15e21].

Propranolol’s effect has been proposed to result from vasocon-striction due to decreased nitric oxide release, down-regulation ofvascular growth factors, blocking of GLUT-1 receptors, and inductionof apoptosis [22]. The drug is well tolerated, exists in liquid prepara-tion, and has a proven safety record with a long history of use in pe-diatric cardiology. In the absence of large-scale clinical studies todefine evidence-based guidelines for its use in IH, a 2011 Americanconsensus conference reviewed the existing data and produced a setof recommendations for propranolol dosing and monitoring [23].Although its efficacy has not been formally defined in large-scalerandomized controlled trials, a growing body of evidence, includingthis case of successful treatment of an omental andmesenteric lesion,supports the use of propranolol in visceral infantile hemangiomas.

Conflict of interest statementThe authors have no conflicts of interest.

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