Orbital lymphangioma with non-contiguous cerebral arteriovenous malformation, manifesting with thrombocytopenia (Kasabach-Merritt syndrome) and intracerebral hemorrhage

This study describes the first reported case in a preterm infant of an orbital lymphangioma with non-contiguous cerebral arteriovenous malformation, manifesting with thrombocytopenia (Kasabach-Merritt syndrome) and intracerebral hemorrhage. Conclusion: Neonates presenting with orbital lymphangiomas should undergo radiological investigations of the lesion and a detailed cerebral evaluation for associated arteriovenous developmental anomalies.     

Pulmonary arteriovenous fistula in the newborn: a case report of Rendu-Osler-Weber syndrome and a review of the literature.

In most instances, congenital arteriovenous fistula is only one manifestation of a more widespread abnormality; 60% of patients also have hereditary hemorrhagic telangiectasis (Rendu-Osler-Weber syndrome). Among those with congenital pulmonary arteriovenous fistula, the diagnosis is made during infancy in only 15% of patients. We present a case of pulmonary arteriovenous fistula in a newborn and review the literature. This rare condition of newborns can be treated with different surgical procedures. Only 17 cases of newborn pulmonary arteriovenous fistula/have been reported, and only two of those had associated Rendu-Osler-Weber syndrome. The results of surgical procedures were good in most of these cases. We treated our case with lobectomy successfully.     

An intracranial aneurysm and dural arteriovenous fistula in a newborn

The authors present the case of a newborn with an intracerebral aneurysm and a dural arteriovenous fistula. The patient initially presented with intraventricular hemorrhage and hydrocephalus, with evidence of remote subarachnoid hemorrhage, left hemispheric stroke, and sagittal sinus thrombosis. He was treated with a ventriculoperitoneal shunt and subsequent staged endovascular obliteration of both the aneurysm and fistula. Interestingly, the aneurysm did not appear on an artery feeding the abnormal fistula. Intracerebral aneurysms in the neonatal population are rare, and dural arteriovenous fistulae even more so; we present a case of a 2-month-old infant with both, as well as a review of the literature concerning these rare vascular abnormalities.     

Large fetal pulmonary arteriovenous fistula: impact on pulmonary development

A case report of a patient with a large pulmonary arteriovenous fistula and valvar pulmonary stenosis is presented. The fistula was diagnosed prenatally and its effect on in utero cardiovascular growth and development documented. Due to concerns about massive intrapulmonary shunting potentially causing profound cyanosis after delivery, an EXIT (EX-utero Intrapartum Treatment) procedure was used to transfer the infant from placental to extracorporeal membrane oxygenation (ECMO) support. Severe pulmonary microvascular disease resulted in prohibitive pulmonary hypertension despite surgical ligation of the fistula. Prenatal and postnatal hemodynamic assessments of the fistula are presented and are compared to the pathologic findings.     

Hemorrhagic stroke associated with pulmonary edema and catastrophic cardiac failure

Cerebral arteriovenous fistula (AVF) is a vascular malformation that is rare in the pediatric population. Older children with cerebral AVF tend to present with neurologic problems related to intracranial venous hypertension or intracranial hemorrhage. Cardiac and pulmonary complications following acute neurologic injury such as subarachnoid hemorrhage are common in adults, but are rarely reported in children. However, complications have been reported in cases of enterovirus 71 rhombencephalitis in infants and children and can cause high morbidity and mortality. Here, we report a 14-year-old boy who presented with cardiac failure associated with pulmonary edema following cerebral hemorrhagic stroke due to AVF. After aggressive investigation and management, we intervened before significant hypoxia and hypotension developed, potentially reducing the risk of long-term adverse neurologic consequences in this patient.     

Hereditary hemorrhagic telangiectasia. A case report with known cerebral involvement and cardiac catheterization data.

Numerous bleeding episodes, neurological signs and symptoms, and pulmonary arteriovenous malformations are well documented in reports of families with hereditary hemorrhagic telangiectasia. It is always assumed that these are due to the arteriovenous malformations. We are here reporting a case with positive cerebral angiographic findings of large arteriovenous communications, as well as catheterization data revealing pulmonary abnormalities secondary to the cerebral arteriovenous fistula, but no abnormalities within the pulmonary vasculature itself.     

Bleeding after circumcision in a newborn receiving sildenafil

This report describes a case of bleeding as a complication of circumcision in a 2-week-old infant being treated with sildenafil for persistent pulmonary hypertension of the newborn. The vasodilatory effects of sildenafil on the penile vasculature may have contributed to the postoperative bleeding in this patient.     

Arteriovenous fistula of the vertebral artery in a female infant with hypotonia and cephalocorporal disproportion

Background: Congenital arteriovenous fistulas are exceptional in childhood and imply a therapeutic challenge. Case report: A 9‐month‐old female infant was studied for cephalocorporal disproportion, hypotonia, progressive muscular atrophy and hyperreflexia. Computed tomography of the brain and electroencephalography were normal. Electromyographic patterns suggested proximal myopathic involvement. A continuous murmur with systolic reinforcement was audible in the neck. Angioresonance detected intracranial aneurysmal dilatations behind the bulbo‐medullary junction and cerebral panangiography evidenced a direct vertebrovertebral fistula with extra‐ and intra‐cranial varices and extreme medullary compression. Occlusion of the afferent vessel to the aneurismal sack was successfully achieved with a mixture of Histoacryl and 75% lipiodol via a microcatheter. Conclusion: Clinical signs of an arteriovenous fistula may be atypical. Considerable cephalocorporal disproportion and a bruit in the cervical or retromastoidal regions must suggest its existence. Complete obliteration with endovascular embolization permits somatic and neurological recovery.     

Pulmonary arteriovenous fistula

A case of pulmonary arteriovenous fistula in a 6 1/2 year-old girl is reported. The fistula which was first seen on X-ray films resulted in low arterial oxygen saturation, asthenia and dyspnea during exercise. It was confirmed by right catheterism and angiography. The child was cured after left inferior lobectomy. No other vascular malformations could be demonstrated in the child or in her family. Literature data are reviewed.     

Aneurysms of the vein of Galen.

Six neonates with aneurysms of the vein of Galen are described: five were associated with an intracerebral arteriovenous fistula and presented with heart failure in the neonatal period; one had hydrocephalus at birth. Two infants died from intractable cardiac failure within 48 hours of age and two from progressive hydrocephalus and cardiac failure at 8 months and 8 years respectively. Two of the infants had copper coil embolisation of the aneurysm--one has since died of an intracerebral haemorrhage. The diagnosis of an intracerebral arteriovenous fistula should be considered in any infant presenting with unexplained heart failure. Close attention to the volume of all arterial pulses, and the venous pulse in the neck as well as auscultation of the head for a cranial bruit may suggest the diagnosis, which can then be substantiated by cerebral ultrasound.     

Congenital Subclavian Artery to Subclavian Vein Fistula Presenting with Congestive Heart Failure in an Infant

Contrary to acquired arteriovenous fistulas, the congenital malformations are very rare. Here we report a case of congenital arteriovenous fistula forming a communication between the right subclavian artery to subclavian vein. To our knowledge, there is no similar case in which an infant has been successfully operated on for cardiac failure.     

Neonatal intraventricular hemorrhage due to an intracranial arteriovenous malformation: a case report.

A full-term newborn infant with intraventricular hemorrhage due to a small arteriovenous malformation draining into the vein of Galen is presented. Other reported series are reviewed, and differences between this case and the usual pattern of morbidity are discussed. The use of computerized tomography is demonstrated.     

Aortoesophageal fistula due to double aortic arch and prolonged nasogastric intubation: case report and review of the literature

Background The authors present a patient and describe other cases from the literature which demonstrate that prolonged use of nasogastric tubes can result in life-threatening aortoesophageal fistula formation in patients with a double aortic arch.Conclusion Aortoesophageal fistula (AEF) is an abnormal communication between the esophagus and the aorta. It can cause massive gastrointestinal hemorrhage. We present an infant with an operatively corrected double aortic arch, who developed this complication after prolonged nasogastric intubation.     

Pulmonary arteriovenous malformations

Pulmonary arteriovenous malformations rarely present in childhood. Two cases are presented in this report. Both the cases presented clinically with cyanosis and clubbing without a cardiac murmur. The second case had cerebral abscess in addition. Both the cases underwent a contrast-enhanced echocardiography which suggested the presence of pulmonary arteriovenous malformations. The first case also underwent^99mTc radionuclide scan and pulmonary angiography. The cases are being reported for their characteristic clinical features and for emphasizing the role of non-invasive modalities like contrast-enhanced echocardiography and radionuclide scan in reaching the diagnosis.     

Iatrogenic Arteriovenous Fistula in Infancy

ABSTRACT. Iatrogenic arteriovenous fistulas (AVF) in infancy are rare and are usually located at the level of femoral and antecubital vessels. They are generally secondary to multiple diagnostic or therapeutic arterial or venous punctures. The diagnosis is usually easy to make on clinical grounds; however, invasive procedures such as digital subtraction angiography (DSA) can be used to locate the fistula. These iatrogenic AVF may present as direct vascular communications or pseudoaneurysms originating in the venous wall. Surgical treatment is the therapy of choice. The case of an infant with an iatrogenic AVF of the femoral vessels is presented.     

Iatrogenic arteriovenous fistula in infancy

Iatrogenic arteriovenous fistulas (AVF) in infancy are rare and are usually located at the level of femoral and antecubital vessels. They are generally secondary to multiple diagnostic or therapeutic arterial or venous punctures. The diagnosis is usually easy to make on clinical grounds; however, invasive procedures such as digital subtraction angiography (DSA) can be used to locate the fistula. These iatrogenic AVF may present as direct vascular communications or pseudoaneurysms originating in the venous wall. Surgical treatment is the therapy of choice. The case of an infant with an iatrogenic AVF of the femoral vessels is presented.     

Fulminant development of pulmonary arteriovenous fistulas in an infant after total cavopulmonary shunt

An infant with cyanotic congenital heart disease and polysplenia syndrome developed profound cyanosis within months of undergoing bilateral cavopulmonary anastomoses. Intrapulmonary shunting was diagnosed by contrast echocardiography with peripheral venous and selective pulmonary artery injection. Histopathology revealed abnormal, thin-walled vessels within the interstitium of the lung lobule. These vessels have not been reported previously and are likely to be the anatomic site of arteriovenous shunting. This case demonstrates that pulmonary arteriovenous fistulas (PAVFs) may develop rapidly after cavopulmonary anastomosis in young infants. It also illustrates the use of contrast echocardiography for following PAVF progression in these patients.     

Massive pulmonary hemorrhage before living donor liver transplantation in infants

A massive pulmonary hemorrhage in patients with liver cirrhosis is a life‐threatening complication that may result in a contraindication of a liver transplantation because of its high mortality rate. Herein, we present two infant biliary atresia cases that successfully underwent an LDLT that was followed by intensive respiratory care for the pretransplant massive pulmonary hemorrhage. Both cases exhibited severe respiratory failure (minimum PaO₂/FiO₂; 46 mmHg and 39 mmHg, respectively). To arrest the bleeding, we applied a very high positive pressure ventilation treatment (maximum PIP/PEEP; 38/14 cmH₂O and 55/15 cmH₂O, respectively), plasma exchange, several FFP transfusions, and recombinant factor VIIa via intrapulmonary administration. In addition, we used CHDF treatment, applied HFOV transiently, and treated the patient with inhalation of nitric oxide. Although we prepared ECMO for intra‐operative use, both cases were successfully managed with conventional mechanical ventilation without using ECMO, which may have worsened the pulmonary hemorrhage due to the use of an anticoagulant. Use of an excessive positive pressure management, although it poses a risk for barotrauma, could be acceptable to arrest the pulmonary bleeding in selected cases of liver failure patients who have no time remaining before LDLT.     

Carotid jugular arteriovenous fistula: An unusual complication of internal jugular vein catheterization in children

Central venous catheterization is essential in the management of patients requiring long‐term intravenous access. Various risks during central line insertion have been described in the medical literature, including the potentially life‐threatening complication of iatrogenic arteriovenous fistula (AVF). We describe a novel case of carotid jugular AVF following implanted port (BardPort® by C.R. Bard, Inc.) insertion in a pediatric oncology patient who had suffered cerebral infarct due to thromboembolism. Pediatr Blood Cancer 2012; 59: 1302–1304. © 2012 Wiley Periodicals, Inc.     

Successful Embolization of Coronary Arteriovenous Fistula Using an Interlocking Detachable Coil

Although the efficacy and feasibility of coil embolization of coronary arteriovenous fistulas have been reported, the procedure may be complicated by migration of the coil into peripheral vessels or pulmonary arteries. We report two cases of successful coil embolization of such lesions using an interlocking detachable coil. This system can provide safer and more effective coil embolization in patients with coronary arteriovenous fistula.