Retroperitoneal arteriovenous malformation,a rare cause of heart failure in infancy: Consideration of therapeutic approaches

Summary We report an infant presenting with congestive heart failure secondary to a large retroperitoneal arteriovenous (AV) malformation. The heart failure was treated by catheter embolization of the feeding arterial vessels with Ivalon particles. Following resolution of the heart failure, a residual avascular mass remained. A biopsy revealed the diagnosis of an undifferentiated sarcoma that was successfully treated with chemotherapy and surgery.     

Surgical treatment of vascular malformations of the extremities in children and adolescents

The treatment of vascular malformations (VM) of the extremities is controversial. Six patients with large, localized, symptomatic VMs of the extremities underwent surgical excision. In five cases abnormal vessels were apparent under the skin; in two of these there was also red discoloration of the skin secondary to skin involvement. Magnetic resonance imaging was the most helpful imaging modality in evaluating these patients. Subfascial resection was performed in five cases. Muscle was involved in four patients and was removed in all four cases. At follow-up, there was improvement in symptoms and function with no clinically apparent recurrence an average of 30 months following the operation. There was improvement in pain intensity from an average of 4.3 on a scale of 1 to 10 before surgery to 1.3 after surgery. Surgical excision of localized VMs that are extensive and include fascia and muscle, as appropriate, can successfully improve pain and function without evidence of recurrence at 1 to 5 years of follow-up. Accepted: 18 January 2001     

59例儿童脑动静脉畸形诊治体会

目的报道59例儿童脑动静脉畸形诊治体会。方法回顾分析长征医院1990～2000年收治59例18岁以下脑AVM患儿诊治结果，其中单纯手术治疗10例；栓塞后再手术7例；栓塞后伽玛刀治疗18例；手术后伽马刀治疗3例；单纯栓塞治疗5例；直接伽玛刀治疗13例；未治3例。结果48例（48／59）患儿临床表现为出血，47例（47／56）治疗后没有产生任何新的神经功能缺损，6例（6／56）治疗后出现轻度神经功能缺损或神经功能缺损加重，2例（2／56）患儿遗留严重的神经后遗症，死亡1例。未治3例，死亡1例。结论儿童脑AVM具有出血率高及出血凶猛的特点，应争取积极治疗，合理正确使用显微手术、放疗、血管内栓塞等治疗手段．临床效果满意。     

Congenital renal arteriovenous malformation (aneurysmal type) in childhood

We report on a 9-year-old boy with a congenital renal arteriovenous fistula of the aneurysmal type, a form previously not observed in childhood. The clinical picture was unusual with severe arterial hypertension, excessive polyuria and decreased levels of serum sodium and chloride as main signs. Clinical and biochemical findings normalised after nephrectomy of the kidney involved.     

Cerebral arteriovenous malformation diagnosis by two-dimensional color-coded Doppler ultrasonography of the head

Summary Two-dimensional color-coded Doppler examination revealed a cranial arteriovenous (AV) malformation in a critically ill newborn with intractable congestive heart failure. This case emphasizes the value of color-coded Doppler in the diagnosis of this rare malformation.     

Conditions for treatment of cerebral arteriovenous malformation associated with ectasia of the vein of Galen in the newborn

The survival rate and subsequent quality of life has improved significantly for neonates with cerebral arteriovenous malformation associated with ectasia of the vein of Galen, with recent advances in interventional neuroradiology. Transarterial endovascular occlusive techniques have emerged as the treatment of choice. However, this treatment is technically very demanding and good results are seen only in institutions with large experience in treating this rare type of congenital malformation. The conditions for granting the neonate access to this sophisticated type of treatment include appropriate clinical and neuroradiological evaluation at the highest possible level, combined with the establishment of early communication with a trained interventional neuroradiological team capable of this treatment. Not all neonates are suited to this form of treatment, but only rapid, careful and competent treatment and evaluation of the neonate can assure appropriate pre-procedural evaluation in order to give each patient, wherever born, a similar and optimized chance of survival without unnecessary sequelae.     

New approaches to the diagnosis of tuberculosis in childhood with special reference to neurotuberculosis

Bacteriological diagnosis of tuberculosis in childhood is often unsuccessful owing to the difficulty in obtaining suitable specimens. Many attempts have been made to diagnose tuberculosis immunologically but with very limited success. Positive tuberculin reactions may be the result of nonspecific sensitization while negative reactions occur in undernourished children. Serodiagnostic tests suffer from problems of specificity, even when very specific antigens are used, and are often least helpful in diagnostically difficult cases. Detection of antigen has proved to be of more value, especially with clean specimens such as cerebrospinal and pleural fluids. Detection of specific components ofMycobacterium tuberculosis by linked gas chromatography and mass spectroscopy is very sensitive and specific but the equipment is very costly. Detection of specific DNA sequences ofM. tuberculosis in specimens by use of labelled ‘DNA probes’ is rather insensitive although the sensitivity may be increased greatly by use of the polymerase chain reaction to amplify small amounts of the specific DNA. Non specific indicators of tuberculosis are generally unhelpful although the bromide partition test and assay of the enzyme adenosine deaminase in cerebrospinal fluid appear to be of value in the diagnosis of tuberculous meningitis. More research is required to develop a simple, specific and automated test for tuberculosis in childhood.     

Endovascular treatment of pediatric intracranial arteriovenous shunt

Intracranial arteriovenous shunts (ICAVS) in young children are characterized by frequent high‐flow fistulas. In association with high‐flow fistulas and the physiological condition of the developing brain and heart, each ICAVS type tends to present at a certain age with unique symptoms. Vein of Galen aneurysmal malformation (VGAM) and dural sinus malformation with arteriovenous (AV) shunt tend to present in the neonate with high output cardiac failure. In infancy, VGAM, pial arteriovenous fistula (AVF) and infantile dural AVF (DAVF) tend to present with hydrodynamic disorder such as macrocephaly, ventriculomegaly, prominent facial veins, and developmental delay. Pial AVF, AV malformation, and infantile DAVF can present with focal neurological signs such as seizure or hemorrhage at older ages. Endovascular treatment is currently the first choice of treatment for most pediatric ICAVS. The treatment goal should be defined on a patient‐by‐patient basis, according to the unique physiological condition of the child.     

Endovascular treatment of a spinal arteriovenous malformation in a 21-month-old boy

Reports of spinal arteriovenous malformations in children are rare. This case report describes a 21-month-old boy whose first symptom was attacks of abdominal pain, followed gradually by neurological symptoms. The diagnosis was made using magnetic resonance imaging and spinal angiography, and the patient was successfully treated with embolization.     

Transumbilical embolization of hepatic arteriovenous malformation in a neonate with heart failure

A newborn infant presented with severe heart failure due to a large hepatic arteriovenous malformation (AVM). Umbilical artery (UA) access was safely used for immediate diagnosis and for embolization.     

Ultrasound features of a paratesticular arteriovenous malformation: a case report of an 11-year-old boy

A paratesticular arteriovenous malformation (AVM) in an 11-year-old boy with a solitary testicle led to a testicle-sparing excision. The radiological features of this rare lesion are reviewed.     

特殊类型静脉畸形发病机制研究进展

静脉畸形(venous malformation,VM)是最常见的血管畸形,根据其临床和组织学特征目前主要可分为普通静脉畸形和特殊类型静脉畸形.特殊类型静脉畸形主要包括家族性皮肤黏膜VM、蓝色橡皮乳头样痣(Bean)综合征、球形细胞VM等.除普通VM外,其余VM多伴有多个部位的病变.近年来随着基因测序技术的发展,关于这...     

Primitive megalencephaly in children: Natural history,medium term prognosis with special reference to external hydrocephalus

We studied 74 children with primitive megalencephaly retrospectively with attention directed to familial megalencephaly, birth history, enlarged pericerebral subarachnoid space (SAS) (idiopathic external hydrocephalus), head and statural growth dynamics, developmental and school prognosis, morphological findings and development of subdural haematoma. In the megalencephalic children, no significant differences were found between those with normal or those with enlarged pericerebral SAS. Out of 62, 31 children (50%) were already megalencephalic at birth. Of 74, 37 children (50%) showed variable degrees of developmental delay which in 18 was transient. Eight out of 74 were mentally retarded. Of 52 children at school age, 42 attend normal schools and 10, of whom 7 are mentally retarded, attend special schools. Three children showed subdural haematoma resulting from apparently minor trauma or occurring spontaneously. We suggest that idiopathic external hydrocephalus represents a variant of primitive megalencephaly with transient increase of intracranial pressure and that it could predispose to the development of idiopathic (spontaneous or non-traumatic) subdural haematoma.This work has been presented in part at the Société de Neurologie Infantile, Tlemcen, Algeria, November 1987     

Pleuropulmonary blastoma: an aggressive intrathoracic neoplasm of childhood

A case of a 4-year-old girl with pleuropulmonary blastoma is reported. Surgical resection of the tumor was performed and histologic examination revealed pleuropulmonary blastoma with rhabdomyosarcomatous differentiation. Postoperative chemotherapy was administered and 3 weeks after initiation of treatment protocol a second site of lesion in the retroperitoneum was revealed with extension to the mediastinum, which shared similar mesenchymal neoplastic characteristics to the previously diagnosed primary lesion. The girl died 4 1/2 months after initial evidence of disease because of brain metastasis, indicating a very aggressive neoplasm unresponsive to treatment.