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Successful treatment of pulmonary arteriovenous malformation and infantile hepatic hemangioendothelioma with alpha-interferon/Infantil hepatik hemanjiyoendotelyoma ve pulmoner arteriyovenoz malformasyonun alfa-interferon ile basarili bicimde tedavisi.

Introduction

Pulmonary arteriovenous malformation (PAVM) is a rare cause of cyanosis in early infancy. PAVMs are direct communications between the smaller pulmonary arteries and veins (1, 2). Pulmonary arteriovenous malformation in its acquired form usually occurs in juvenile cirrhosis, but has also been reported in patients with trauma, pulmonary schistosomiasis, mitral stenosis, actinomycosis, Fanconi syndrome, and metastatic thyroid carcinoma (3). However, as far as we know infantile hepatic hemangioendothelioma (IHHE) which consists of clinical triad including hepatomegaly, congestive heart failure, and cutaneous hemangiomas with coexistent PAVM has not been reported.

Here, we describe this unusual presentation.

Case Report

A 10-week old boy was admitted to our hospital from a community hospital for abdominal distension, hepatomegaly and respiratory distress. He developed neonatal jaundice requiring phototherapy on the 3rd day of life. Neonatal screening for congenital hypothyroidism demonstrated elevated thyrotropin (TSH) level. He was treated with L-thyroxine.

At the time of referral, the patient was tachypneic, tachycardic and cyanotic with prominent hepatomegaly. The patient was hypoxemic while breathing room air (percent arterial oxygen saturation 70); with oxygen enriched air the percent arterial oxygen saturation raised to 80%. On physical examination he had a heart murmur and congestive heart failure was obtained on echocardiogram. He was started on furosemide and digoxin with rapid stabilization of his cardiac status.

On laboratory evaluation, alpha-fetoprotein (AFP) level was markedly elevated to 2901 ng/ml. The results of blood counts, coagulation studies and liver function tests were all normal. Excretions of catecholamines or metabolites in the urine were within the normal range. Thyroid function tests showed an elevated TSH level of 27.7 mclU/ml. An abdominal sonogram of the patient showed hepatomegaly caused by multiple hypoechoic-isoechoic nodules in both lobes of the liver. Abdominal magnetic resonance imaging (MRI) studies of the hepatic nodules showed decreased signal intensity on T1 images and high signal intensity on T2. There were lesions, up to 3.0 cm in diameter (Fig. 1).

A diagnosis of IHHE was based on the signs of congestive heart failure and hypothyroidism, and the ultrasonography (US) and MRI findings. Then, we started oral prednisolone (2 mg/kg/day) therapy. The patient's hypoxemic and cyanotic status did not change in spite of the therapy. Contrast echocardiography study suggested the presence of intrapulmonary arteriovenous malformation. A contrast echocardiogram showed rapid return of contrast into the left atrium less than 5 seconds after it is seen in the right atrium (Fig. 2) and a diagnosis of diffuse type PAVM was made. We started alpha-interferon 3 million units/m2 per day over 18 days followed by 3 million units/m2 3 times per week on the fifth day of the prednisolone therapy. On the 7th day of this combined prednisolone and alpha-interferon therapy his hypoxemic status became normal and the passage of contrast into the left atrium disappeared 1 month after alpha-interferon therapy (Fig. 3). In addition, regression of the hepatic nodules was shown by ultrasound examination approximately 3 months later.

Discussion

The diagnosis of PAVM should be considered in infants with severe cyanosis without a structural cardiac lesion or pulmonary hypertension, after excluding other causes of cyanosis, such as parenchymal lung disease and the rare methemoglobinemia (1, 4). The gold standard in diagnosis is a pulmonary angiogram showing abnormal peripheral vascular formations that may be either localized or diffuse. Multi-slice computed tomography and contrast echocardiography are also useful. Due to the rapid transit of blood flow through the pulmonary veins into the capillaries, the left atrium is seen to be filled early with contrast echoes (within 2-5 seconds) following contrast passage through the right atrium, as we found in our case (5-7).

[FIGURE 1 OMITTED]

[FIGURE 2 OMITTED]

[FIGURE 3 OMITTED]

Treatment of localized PAVMs includes surgical lobectomy or trans-catheter embolization using coils, but there is no definitive treatment for the diffuse type. Interferon therapy has been used in patients PAVMs with juvenile cirrhosis (8-10). However, until the present time, its efficacy for the treatment of PAVM has not been clearly demonstrated. In our case, oral prednisolone was initially used because of the extensive and multiple lesions of IHHE. After the diagnosis of PAVM, we added interferon alpha to the prednisolone therapy based on the assumption that interferon alpha is beneficial for both of the illness.

Conclusion

Pulmonary arteriovenous malformation should be kept in mind in cyanotic infants with IHHE. Contrast echocardiography may be a useful non-invasive technique for detecting PAVM and the interferon alpha may be first treatment option for patients with IHHE and PAVM.

doi:10.5152/akd.2011.043

Available Online Date/Cevrimici Yayin Tarihi: 01.03.2011

References

(1.) Al-Qurashi M, Ghazal Y, Al Oufi S, Al Manea W. Diffuse pulmonary arteriovenous malformation: unusual cause of cyanosis in infancy. Ann Saudi Med 2000; 20:144-6.

(2.) Zain Z. Diffuse pulmonary arteriovenous malformation-a case report. Images Pediatr Cardiol 2004; 18:13-6.

(3.) Gianisella RB, Rossi Filho RI, Zielinsky R Diagnosis and therapeutics of pulmonary arteriovenous fistula in childhood. Case report and review of the literature. Arq Bras Cardiol 2001; 77: 274-81.

(4.) Shapiro JL, Stillwell PC, Levien MG, Latson LA, Ratliff NB. Diffuse pulmonary arteriovenous malformations (angiodysplasia) with unusual histologic features: case report and review of the literature. Pediatr Pulmonol 1996; 21:255-61.

(5.) Chang RK, Alejos JC, Atkinson D, Jensen R, Drant S, Galindo A, et al. Bubble contrast echocardiography in detecting pulmonary arteriovenous shunting in children with univentricular heart after cavopulmonary anastomosis. J Am Coll Cardiol 1999; 33: 2052-8.

(6.) Van Hare GF, Silverman NH. Contrast two-dimensional echocardiography in congenital heart disease: techniques, indications and clinical utility. J Am Coll Cardiol 1989; 13: 673-86.

(7.) Hind CR, Wong CM. Detection of pulmonary arteriovenous fistulae in patient with cirrhosis by contrast 2D echocardiography. Gut 1981; 22: 1042-4.

(8.) Bay A, Oner AF, Etlik 0, Koseoglu B, Kaya A. Unusual presentation of infantile hemangioendothelioma. Pediatr Blood Cancer 2005; 44: 267-9.

(9.) Woltering MC, Robben S, Egeler RM. Hepatic hemangioendothelioma of infancy: treatment with interferon alpha. J Pediatr Gastroenterol Nutr 1997; 24: 348-51.

(10.) Kasahara M, Kiuchi T, Haga H, Uemoto S, Uryuhara K, Fujimoto Y, et al. Monosegmental living-donor liver transplantation for infantile hepatic hemangioendothelioma. J Pediatr Surg 2003; 38: 1108-11.

Sonay Incesoy Uzdemir, Ceyhun Bozkurt, Utku Arman Orun *, Gurses Sahin,

Nazmiye Yuksek, Semra Cetinkaya **, Ulya Ertem

From Clinics of Pediatric Oncology, * Pediatric Cardiology, and ** Pediatri c Endocrinology, Dr. Sami

Ulus Children's Hospital, Ankara, Turkey

Address for Correspondence/Yazisma Adresi: Dr. Sonay Incesoy Ozdemir Clinic of Pediatric Oncology, Dr. Sami Ulus Children's Hospital, Ankara, Turkey Phone: +90 312 330 81 67 Fax: +90 312 317 03 53 E-mail: sincesoy@yahoo.co.uk
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Title Annotation:Case Reports/Olgu Sunumlari
Author:Uzdemir, Sonay Incesoy; Bozkurt, Ceyhun; Orun, Utku Arman; Sahin, Gurses; Yuksek, Nazmiye; Cetinkaya
Publication:The Anatolian Journal of Cardiology (Anadolu Kardiyoloji Dergisi)
Article Type:Disease/Disorder overview
Date:Mar 1, 2011
Words:1097
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