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A newborn with cardiac failure secondary to a large vein of Galen malformation.

Abstract: We report the case of a term newborn with vein of Galen aneurysm. No antenatal diagnosis was done. The infant was admitted secondary to cardiac failure, with cardiomegaly and pulmonary hypertension. Physical examination was remarkable for acrocyanosis, microcephaly, cranial bruit, bilateral neck swelling, coarse breath sounds, and a hyperdynamic precordium with continuous murmur. Head ultrasound demonstrated a large, midline venous structure. Computed tomographic scan confirmed vein of Galen malformation.

Key Words: cardiac failure, newborn, vein of Galen aneurysm

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Case Report

A male infant was delivered vaginally to a 28-year-old primigravida at 38 weeks' gestation, after an uneventful pregnancy, at a community hospital. In the delivery room, the infant had respiratory distress, which did not improve with positive-pressure ventilation and required intubation. The infant's Apgar scores were 5, 6, and 6 at 1, 5, and 10 minutes, respectively, and his birthweight was 3,274 g.

Physical examination revealed a microcephalic infant with bilateral neck swelling. Coarse breath sounds and subcostal retractions were noted. Cardiovascular examination at the referring hospital was remarkable for a grade II/VI systolic murmur and a quiet precordium. Chest radiography revealed cardiomegaly. An echocardiogram obtained at 4 hours of life revealed a structurally normal heart, with evidence of persistent pulmonary hypertension of the newborn, tricuspid regurgitation, a large patent ductus arteriosus with a right-to-left shunt, and aortic isthmus narrowing, without coarctation. The infant was transferred to the regional neonatal center for further management.

At admission to the neonatal intensive care unit, physical examination revealed an intubated infant with acrocyanosis, with a temperature of 99.0[degrees]F; a heart rate of 154 beats/min; a respiratory rate of 60 breaths/min; and blood pressure of 74/46 mm Hg in the left arm, 61/40 mm Hg in the left leg, 66/37 mm Hg in the right arm, and 72/42 mm Hg in the right leg. Arterial blood gases (post-ductal) showed a pH of 7.33, a PC[O.sub.2] of 39.9 mm Hg, a Pa[O.sub.2] of 43.6 mm Hg, an HC[O.sub.3] of 20.8 mmol/L, a base deficit of 5.1 mmol/L, and an oxygen saturation of 91.7%. Examination of the head revealed molding and a cranial bruit heard through the anterior fontanel. A carotid bruit was auscultated over pulsatile neck vessels bilaterally. Breath sounds remained coarse over both lung fields, with intercostal retractions. Cardiovascular examination showed a hyperdynamic precordium with a grade II to III/VI harsh continuous murmur at the base. The liver was palpable 2 cm below the costal margin. Neurologic examination revealed intermittent tremors of upper and lower extremities.

Repeat echocardiography confirmed the aforementioned findings, with dilated atria and ventricles. A head ultrasound and CT scan were obtained to evaluate the cranial bruit. Despite mechanical ventilation with inhaled nitric oxide, the infant remained insignificant cardiopulmonary distress due to congestive heart failure. The infant's poor prognosis was discussed with the parents and supportive care was withdrawn. The infant died 2 days after birth. An autopsy was performed.

A head ultrasound scan demonstrated a large venous structure in the midline connecting to other spaces in the posterior portion of the cranium. No normal cerebral tissue was seen, and hydranencephaly was suspected (Fig. 1). CT scan of the brain revealed a large, hourglass-shaped density, which enhanced intensely after contrast administration (Fig. 2). This was consistent with a large vein of Galen malformation, due to fistulous communication between the supraclinoid carotid arteries and an enlarged draining venous structure, which took up most of the supratentorial compartment (Fig. 3). Calcifications were seen throughout the subependyma. TORCH titers were negative.

The infant died as a result of congestive heart failure due to the vascular malformation. Autopsy confirmed a cerebral arteriovenous malformation associated with a vein of Galen aneurysm, measuring 2 cm in diameter, located beneath the splenium of the corpus callosum. The aneurysmal vessel wall was uniform in thickness. The brain weight (fixed) was 71 g (normal average brain weight at term, 362 g). Microcephaly, intracerebral (IC) hemorrhage, and hydrocephalus were also present, as a result of chronic hypoxic-ischemic cerebral necrosis.

Discussion

Vein of Galen malformation constitutes an IC mass lesion, and presents in the newborn in approximately 40 to 50% of the cases, (1) sometimes masquerading as cyanotic congenital heart disease. When it is severe enough to appear in the newborn, it constitutes a major therapeutic problem, in spite of the recent advances in radiologic diagnosis, neonatal intensive care, and neurosurgery.

The aneurysmal dilation of the vein of Galen generally has associated neuropathologic findings, consisting of a variety of ischemic lesions (cerebral infarction, basal ganglia infarction, periventricular leukomalacia, selective neuronal necrosis of cerebral cortex or brainstem), hemorrhagic lesions (subarachnoid hemorrhage, germinal matrix-intraventricular hemorrhage, IC hemorrhage, and venous thrombosis), and mass effects of the malformation (including hydrocephalus secondary to obstruction, and compression of other structures). (2, 3)

The clinical manifestations of the vein of Galen malformation in the neonatal period reflect the large size of the lesions and consist predominantly of congestive heart failure. (4) The low resistance of the vascular malformation leads to torrential flow into the malformation, leading to increased cardiac output and increased cardiac chamber volumes, resulting in the clinical picture of congestive heart failure on Day 1 of life. Clinical features accompanying congestive heart failure aid in the diagnosis, and can include a cranial bruit, a hyperdynamic precordium, and bounding carotid pulses. The cranial bruit is continuous in nature, and is frequently localized over the posterior rather than anterior cranium. Seizures and other neurologic signs are unusual. The subsequent course of newborns with the vein of Galen malformation who survive the neonatal period and whose congestive heart failure is stabilized medically is dominated by the development of neurologic disturbances, including delayed development and development of major neurologic deficits (presumably secondary to the IC ischemic and hemorrhagic phenomena), and progressive hydrocephalus.

[FIGURE 1 OMITTED]

[FIGURE 2 OMITTED]

[FIGURE 3 OMITTED]

This anomaly may be diagnosed prenatally by pulsedwave Doppler and color-velocity imaging. (5) The diagnosis of vein of Galen malformation should be suspected in any newborn with unexplained congestive heart failure, especially high-output heart failure, or with unexplained IC hemorrhage or hydrocephalus. Initial diagnostic evaluation should be cranial ultrasonography. The aneurysmally dilated vein presents as a large, echolucent structure in the region of the vein of Galen. Doppler study of flow velocity in the arterial feeders and in the vein could further define the hemodynamics and the anatomy of the lesion. (6) CT scanning is useful for assessment of parenchymal ischemia or IC hemorrhage. Magnetic resonance imaging (MRI) is effective in the evaluation of the aneurysmally dilated vein and also will demonstrate major arterial feeding vessels. Before endoarterial treatment, MRI will help to assess the conditions of the brain. Magnetic resonance angiography may help to delineate the vascular supply and drainage of the malformation, even in the prenatal period.

No difference in the outcome was found between treated and untreated patients that presented with symptoms in the neonatal period. (7) Neurologic outcomes in survivors are perceived to be uniformly poor, (8) although some successful cases have been reported. (9, 10)

Management is a major therapeutic challenge. Direct neurosurgical attack is made extraordinarily difficult by the large size and complicated nature of the lesions and by the vulnerability of the infant in congestive heart failure and with a compromised myocardium. The principal approaches to treatment in the newborn involve attempts to eliminate the high flow through the vascular lesion, either by arterial embolization, usually with a liquid adhesive agent (such as bucrylate or a related polymerizing compound) or microcoils, or by venous embolization, with placement of metal coils. Endovascular therapy with modern neuroanesthetic and neurointensive care can improve outcomes even in the highest risk neonates with vein of Galen malformation and cardiac failure. (11) A multidisciplinary approach involving neonatologists, pediatric cardiologists and neurologists, and interventional radiologists, with complementary noninvasive investigations such as transfontanellar ultrasonography, electroencephalography, MRI, and echocardiography, is necessary to optimize the management and limit the neurologic sequelae in children with this type of malformation. (12)

Accepted April 11, 2003.

Copyright [c] 2004 by The Southern Medical Association

0038-4348/04/9705-0516

References

1. Hoffman HJ, Chuang S, Hendrick EB, et al. Aneurysms of the vein of Galen: Experience at The Hospital for Sick Children, Toronto. J Neurosurg 1982;57:316-322.

2. Meyers PM, Halbach VV, Phatouros CP, et al. Hemorrhagic complications in vein of Galen malformations. Ann Neurol 2000;47:748-755.

3. Ishimatsu J, Yoshimura O, Tetsuou M, et al. Evaluation of an aneurysm of the vein of Galen in utero by pulsed and color Doppler ultrasonography. Am J Obstet Gynecol 1991;164:743-744.

4. Chevret L, Durand P, Alvarez H, et al. Severe cardiac failure in newborns with VGAM: Prognosis significance of hemodynamic parameters in neonates presenting with severe heart failure owing to vein of Galen arteriovenous malformation. Intensive Care Med 2002;28:1126-1130.

5. Chisholm CA, Kuller JA, Katz VL, et al. Aneurysm of the vein of Galen: Prenatal diagnosis and perinatal management. Am J Perinatol 1996;13:503-506.

6. Stockberger S, Smith R, Don S. Color Doppler sonography as a primary diagnostic tool in the diagnosis of vein of Galen aneurysm in a critically ill neonate. Neuroradiology 1993;35:616-618.

7. du Plessis A, Volpe JJ. Prognosis for development in the newborn requiring neurosurgical intervention. Neurosurg Clin N Am 1998;9:187-197.

8. Mitchell PJ, Rosenfeld JV, Dargaville P, et al. Endovascular management of vein of Galen aneurysmal malformations presenting in the neonatal period. AJNR Am J Neuroradiol 2001;22:1403-1409.

9. Hamasaki T, Kai Y, Hamada J, et al. Successful treatment of a neonate with vein of Galen aneurysmal malformation. Pediatr Neurosurg 2000;32:200-204.

10. Fischer B, Palkovic S. Image in clinical medicine: Neonatal vein of Galen malformation. N Engl J Med 2002;346:1553.

11. Kleindienst A, Hildebrandt G, Klug N, et al. Management of vein of Galen malformations: A review based on five neurosurgically treated cases and literature reports. Zentralbl Neurochir 1999;60:172-182.

12. Bouvaist H, Rossignol AM, Rocca C, et al. Aneurysm of the vein of Galen and cardiac insufficiency: Current therapeutic approach [in French]. Arch Mal Coeur Vaiss 1998;91:637-643.

RELATED ARTICLE: Key Points

* The presence of a cranial bruit on physical examination is an abnormal sign that warrants further investigation.

* Vein of Galen malformation may present in the newborn in approximately 40 to 50% of cases.

* Vein of Galen malformation should be considered in the differential diagnosis of neonatal congestive heart failure.

Sergio G. Golombek, MD, Shamiza Ally, MD, and Paul K. Woolf, MD

From the Department of Pediatrics, New York Medical College, Westchester Medical Center, Valhalla, NY.

Reprint requests to Sergio G. Golombek, MD, FAAP, The Regional Neonatal Center, Westchester Medical Center, New York Medical College, Valhalla, NY 10595. Email: sergio_golombek@nymc.edu
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Title Annotation:Case Report
Author:Woolf, Paul K.
Publication:Southern Medical Journal
Date:May 1, 2004
Words:1807
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