Effectiveness of Combined Direct and Indirect Revascularization for Moyamoya Disease with Concurrent Congenital Rubella Syndrome.

BACKGROUND: For several variants of quasi-moyamoya disease, cerebral revascularization treatment is as effective as it is for the more typical cases of moyamoya disease. Here, we examined a case of moyamoya disease with concurrent congenital rubella syndrome (CRS). On the basis of concurrent underlying disease, the patient was considered to have quasi-moyamoya disease and was treated with cerebral revascularization. CASE DESCRIPTION: A 36-year-old female presented with a large cerebral infarction. She was diagnosed with quasi-moyamoya disease on the basis of clinical and imaging features. The ischemic symptoms and cognitive dysfunction improved after combined direct and indirect revascularization. CONCLUSIONS: To our knowledge, this is the first known report of moyamoya disease with concurrent CRS. We treated this patient with revascularization as typical for other quasi-moyamoya conditions including Down syndrome. This case emphasizes the effectiveness of revascularization treatment for moyamoya disease with concurrent CRS for the prevention of ischemic stroke and improvement of cognitive function, despite existing cerebral infarction.

Characteristics of Patent Ductus Arteriosus in Congenital Rubella Syndrome.

This study investigated the characteristics of congenital rubella syndrome (CRS)-associated cardiac complications, particularly patent ductus arteriosus (PDA). We reviewed the medical records of patients with CRS who were admitted to the Children's Hospital 1 in Vietnam between December 2010 and December 2012, and patients with CRS who underwent PDA transcatheter occlusion therapy at the cardiology department between December 2009 and December 2015. We compared the characteristics of PDA treated with transcatheter closure between children with CRS (CRS-PDA) and those without CRS (non-CRS-PDA) who underwent PDA transcatheter closure between July 2014 and December 2015. One-hundred-and-eight children with CRS were enrolled. Cardiac defects (99%), cataracts (72%), and hearing impairment (7%) were detected. Fifty CRS-PDA and 290 non-CRS-PDA patients were examined. CRS-PDA patients had smaller median birthweight (p < 0.001), more frequent pulmonary (p < 0.001) and aortic stenosis (p < 0.001), higher main pulmonary artery pressure, and higher aortic pressure in systole/diastole (p < 0.001 for each) than did non-CRS-PDA patients. The proportion of tubular-type PDA was higher in CRS-PDA patients (16%) than in non-CRS-PDA patients (3%) (p = 0.020). Tubular-type PDA was frequently seen in patients with CRS and accompanied by pulmonary/systemic hypertension and pulmonary/aortic stenosis; in these patients, more cautious device selection is needed for transcatheter PDA closure.

Pre-anesthetic echocardiographic findings in children undergoing non-cardiac surgery at the University of Benin Teaching Hospital, Nigeria.

BACKGROUND: A pre-anaesthestic echocardiogram (echo) is requested for most non-cardiac surgeries to identify possible cardiac structural anomalies. OBJECTIVE: To describe the prevalence and spectrum of structural cardiac abnormalities seen in various non-cardiac conditions. METHODS: We carried out a retrospective review of pre-anaesthetic echos performed over five years on children scheduled for non-cardiac surgery. The requests were categorised according to referring specialities, and the biodata and echo findings were noted. RESULTS: A total of 181 children and 181 echocardiograms were studied, and 100 (55.2%) of the patients were male. Most of the children (87, 48.1%) with oro-facial clefts were referred from dentistry. Of the 181 children, 39 (21.5%) had cardiac abnormalities, most (34, 87.2%) of whom had congenital heart disease (CHD). Ophthalmic requests with suspected congenital rubella syndrome (CRS) had the highest prevalence of 8/12 (66.7%) while the lowest was oro-facial clefts at 15/87 (17.2%). Atrial septal defect was the commonest abnormality, found in 14 patients (35.9%). CONCLUSION: Pre-anaesthetic echo should be performed, especially for children with suspected CRS and other congenital anomalies, requiring non-cardiac surgery.

Transient leopard spot corneal endothelial staining with trypan blue during cataract surgery in a child with congenital rubella syndrome.

We report the complication of corneal endothelial staining with trypan blue that limited the surgical view during cataract extraction in a 10-month-old boy. The boy had presented with a pigmentary retinopathy, microphthalmia, and a dense, white, unilateral congenital cataract. He was suspected of having, and was later diagnosed with, congenital rubella syndrome. We hypothesize that the corneal staining may have resulted from virally induced corneal endothelial damage. To our knowledge, this is the first reported case of trypan blue adversely affecting congenital cataract surgery.

[Manifestation of congenital rubella syndrome: clinical and epidemiologic aspects]. / Les manifestations ophtalmologiques de la rubéole congénitale: aspects cliniques et épidémiologiques.

INTRODUCTION: Congenital rubella syndrome is an ensemble of congenital malformations which results from a primary viral infection in non-immunised pregnant women. The main ophthalmologic manifestation is cataract. It involves at the same time visual and vital prognosis and can be responsible for multiple handicaps. METHODS: We did a retrospective study of 16 infants (32 eyes) having undergone congenital cataract surgery compatible with congenital rubella syndrome in the Casablanca paediatric ophthalmology department between January 2001 and December 2005. All the patients underwent a complete ophthalmologic examination, otorhinolaryngologic, cardiovascular and neurological examinations, and paraclinic explorations. RESULTS: The results were compared with those reported in the literature. In our series, 25 eyes (78.12%) had a cataract of which 56% were nuclear, 13 eyes (40.62%) had microphthalmia. One case each of corneal dystrophy and iris coloboma were described. Congenital glaucoma was found in only one case. Pigmentary retinopathy was found in 12 eyes (37.5%). Seven patients (43.75%) had associated cardiac anomalies, 6 (37.5%) deafness, 5 (31.25%) psychomotor delay and 2 (12.5%) facial dysmorphy. CONCLUSION: Considering the permanent disabilities caused by congenital rubella syndrome, care should be taken with the follow-up of the pregnancies and an immunization program should be implemented for good control of the circulation of the virus.

[Supravalvar and valvar aortic stenosis associated with valvar and subvalvar pulmonary stenosis. Coexistence of two clinical syndromes]. / Estenose supravalvar e valvar aórtica associada à estenose infundíbulo valvar pulmonar. Coexistência de duas síndromes clínicas.

A 4 year old patient with congenital rubella syndrome, confirmed serologically, presents with neurosensorial deafness and a rare association of cardiac anomalies: supravalvar and valvar aortic stenosis and subvalvar pulmonary stenosis. Bidimensional echocardiography and angiography confirmed the diagnosis and the surgical treatment was successful. Due to the presence of somatic characteristics of Williams's syndrome, mental retardation and supraortic stenosis, the authors postulate that there is a coexistence of clinical syndromes responsible for the malformations of this case. This fact is rare on clinical settings, requiring accurate diagnosis and treatment.

Estenose supravalvar e valvar aórtica associada à estenose infundíbulo valvar pulmonar. Coexistência de duas síndromes clínicas / Supravalvar and valvar aortic stenosis associated with valvar and subvalvar pulmonary stenosis. Coexistence of two clinical syndromes

A 4 year old patient with congenital rubella syndrome, confirmed serologically, presents with neurosensorial deafness and a rare association of cardiac anomalies: supravalvar and valvar aortic stenosis and subvalvar pulmonary stenosis. Bidimensional echocardiography and angiography confirmed the diagnosis and the surgical treatment was successful. Due to the presence of somatic characteristics of Williams's syndrome, mental retardation and supraortic stenosis, the authors postulate that there is a coexistence of clinical syndromes responsible for the malformations of this case. This fact is rare on clinical settings, requiring accurate diagnosis and treatment.

Paciente de 4 anos de idade, portador de rubéola congênita, confirmada sorologicamente, apresentou-se com surdez neurosensorial e rara associação de anomalias cardíacas: estenose supravalvar e valvar aórtica e estenose infundíbulo valvar pulmonar. O diagnóstico cardíaco foi firmado por ecocardiografia bidimensional e angiocardiografia e o tratamento cirúrgico obteve êxito. Devido à presença de características somáticas da síndrome de Williams, retardo mental e estenose supraórtica, os autores postulam que possa haver coexistência de síndromes clínicas responsáveis pelas malformações deste caso. Este é fato raro na prática, que requer do clínico diagnóstico e conduta acurados

The use of tissue expanders in the reconstruction of orofacial defects secondary to congenital rubella: case report.

A patient with a severe orofacial defect secondary to congenital rubella is described. The absence of the lower portion of the orbicularis oris muscle, the death of tissue in the infralabial region, as well as the presence of only a vestigial lower lip has hitherto not been reported in the literature. Tissue expanders were used to provide tissue for restoration of the deficient soft tissue of the submental region, a bipedicled flap from the upper lip was used to create a normal anatomic lower lip, and osseointegrated implants were used to stabilize a full denture prosthesis.