Arteriovenous malformations (AVMs) are rarely seen in neonates and most often present with congestive heart failure (CHF). We report a newborn with congestive heart failure due to AVM in the posterior fossa.

A term male baby was admitted to neonatology department with respiratory distress at 5 hour of life. On physical examination perioral cyanosis, dyspnea, tachypnea and a grade 3/6 systolic ejection murmur at left lower sternal border were noticed. These factors lead to the diagnosis of CHF. On echocardiography only right atrial, ventricular and superior vena cava dilatation was observed with no other cardiac anomaly. Cranial auscultation revealed a bruit over right frontoparietal area which necessitated further evaluation with cranial ultrasound and doppler. An AVM was detected and diagnostic angiography con-firmed the presence of an AVM supplied by many arteries and draining to the lateral mesencephalic and inferior petrosal sinus on the anterior surface of the right cerebellar hemisphere and pontomesencephalic corner. Because of its location over the brainstem and huge dimensions it was regarded inappropriate for endovascular surgery and the patient was managed conservatively. He was ventilated mechanically, received inotropic agents and diuretics for CHF but died on 19th day of life.

AVMs which can occur congenitally or can be acquired are non-neoplastic conglomerate vascular abnormality. They are formed by joining of the arteries and veins without lamina elastica by thin channels. AVMs are most commonly seen in cerebral hemispheres (65-85%). According to a published series, 10-18% of AVMs are located in the posterior fossa(1). Clinical manifestations of AVMs are CHF, hemorr-hage, convulsions, focal neurogical signs, hydrocephaly and macrocephaly(1). AVM’s are rare cause of CHF in the newborn(2-4). Doppler ultrasonography, computed tomo-graphy, MRI and MR angiography are useful tools in the diagnosis(1). However, angiography is the most important imaging technique that aids planning the appropriate management(1). Treatment of AVM may be surgical, endovascular therapy, radiosurgery or a combination of all. Although many cases treated by endovascular approach has been reported, this is not the standard therapy for AVMs(5).

Nalan Karabiyik,
Sultan Kavuncuoglu,
Bakirköy Social Security Maternity and
Children Education Hospital,
Neonatology Department, Istanbul, Turkey.
Correspondence to:
Nalan Karabiyik,
Incirli cd Tatlinar sok. Erdim apt.
4/5 Bakirköy/Istanbul.
E-mail: [email protected]

1. Younkin DP, Thorarensen O. Pediatric cerebrovascular diseases. In: Rudolph’s Pediatrics Eds. Rudolph CD, Rudolph AM, Hosteter MK, Lister G, Siegel NJ, 21st ed, USA, McGraw- Hill; 2002: pp.2239.

2. Rodesch G, Malherbe V, Alvarez H, Zerah M, Devictor D, Lasjaunia P. Nongalenic cerebral arteriorvenous malformations in neonates and infants. Review of 26 consecutive cases (1982-1992). Childs Nerv Syst 1995; 11: 231-241.

3. Nakayama H, Suzuki S, Hikino S, Tezuka J, Hara T. Multiple cerebral arteriovenous fistulas and malformations in the neonate. Pediatr Neurol 2001; 25: 236-238.

4. Melville C, Walsh K, Sreeram N. Cerebral arteriovenous malformations in the neonate: clinical presentation, diagnosis and outcome. Int J Cardiol 1991; 31: 175-179.

5. Martin NA, Khanna R, Doberstein C, Bentson J. Therapeutic embolization of arteriovenous malformations: the case for and against. Clin Neurosurg 2000; 46: 295-318.