Complicated pericardial cyst: atypical anatomy and clinical course.

Pericardial cysts are usually detected by chance are are clinically silent in most cases. Nevertheless, symptoms and serious complications may occur. We describe a case of pericardial cyst diagnosed in an 8-year-old boy who was admitted with chest pain. Echocardiography revealed a mild to moderate pericardial effusion and a 7.5 x 5.5 cm intrapericardial echo-free lesion consistent with a pericardial cyst. Surgery was carried out 3 days afterward because of the patient's worsening condition, the progressive increase of pericardial effusion, and the onset of initial signs of cardiac tamponade. The cyst showed a long and easily movable vascular pedicle and inflammatory areas involving the pericardial surface. Like the pericardial effusion, the contents of the mass appeared as serosanguineous fluid on aspiration. Histologic examination confirmed the diagnosis of pericardial cyst and showed findings according to ischemia-related lesions of the cyst. The coexistence of pericardial cyst and cardiac tamponade is very unusual. The atypical anatomy and clinical course suggest a distinct and so far undescribed pathogenetic mechanism for this association: the torsion of a vascular pedicle and the subsequent development of ischemia-related lesions of the cyst.

Aortopulmonary septal defects. A review of the literature and report of ten cases.

From 1981 to 1992, 10 infants with aortopulmonary septal defect (APSD) underwent surgical repair. The mean age at operation was 5.6 +/- 5.5 months, and the mean weight 4.6 +/- 2 kg. Intracardiac associated anomalies were as follows: ventricular septal defects (7 cases), tetralogy of Fallot (2 cases), aortic valve stenosis (2 cases), atrial septal defect (3 cases), patent ductus arteriosus (3 cases), pulmonary valve stenosis (1 case). Cardiac catheterization was performed in 8 out of 10 patients. Eight patients had type I (proximal) defect, 1 had type II (distal) defect and 1 had type III (absent aortopulmonary septation) defect. A variety of surgical procedure was employed. APSD closure with hemoclip was feasible in 3 cases with small window. In 6 patients, during a period of cardiopulmonary by-pass, a side biting clamp was positioned on the ascending aorta close the defect; the border of the window was divided leaving a flap of pulmonary wall on the left side to close the aortic defect; the pulmonary artery was repaired by an autologous pericardial patch. In the patient with type III APSD, aortopulmonary septation was carried out through a transwindow approach. Associated anomalies were repaired in all infants except one. Hospital mortality was 10% (1 case). No late deaths occurred. At a mean follow-up of 47 +/- 35 months 8 patients are asymptomatic and 1 is awaiting for repair of associated anomalies. Conclusions. APSD is a rare but nonetheless well identifiable anomaly. Surgery is indicated as soon as the diagnosis is established, regardless of the patient's age.(ABSTRACT TRUNCATED AT 250 WORDS)

Goretex patch aortoplasty for coarctation in children: nuclear magnetic resonance assessment at 7 years.

From 1979 to 1990, 56 children ranging between 4 days and 16 years of age (mean 73 +/- 51 months) underwent Goretex patch aortoplasty for coarctation of the aorta. The mean weight at operation was 20.2 +/- 3.5 kg (range 3.3-42 kg). Forty-two patients had primary repair, and the remaining 14 had reoperation for recoarctation. The aorta was opened through a standard left thoracotomy, the posterior fibrous ridge was partially excised when it was prominent, and a large patch from a Goretex tube was sutured into place. The postoperative complications were as follows: paradoxical hypertension in 14 cases, massive haemorrhage due to aortic wall rupture in a diabetic child, and intestinal bleeding in 1 case. There were no early deaths and only 1 case of late death, which was not related to coarctation repair (mortality rate 1.8%). The average follow-up was 48 +/- 26 months. Continuous wave-Doppler examination at rest showed no arm-leg systolic gradient in 52 cases and a gradient of about 15 mmHg in 4 cases. Graded exercise testing showed only 1 case with an arm-leg gradient higher than 35 mmHg. Nuclear magnetic resonance (NMR) imaging, performed on 26 patients at a mean of 7 years from operation, showed excellent morphology and size of the aortoplasty. No cases of recoarctation or late aneurysm formation were found. We conclude that Goretex patch aortoplasty can be performed effectively and safely in children. Nuclear magnetic resonance provides high resolution imaging of the coarctation repair site.

Large plaque-like glomuvenous malformation (glomangioma) simulating venous malformation.

Glomuvenous malformations and venous malformations are vascular lesions that can be distinguished on the basis of clinical and pathological features. A vascular lesion of the skin and superficial and deep soft tissues of a lower limb in a 5-year-old child is described. The clinical and radiological features, including skeletal muscle involvement, were typical of venous malformation, whereas the histopathological features were those of a glomuvenous malformation. The clinical and histopathological features are briefly discussed.

Life-threatening hemorrhage from a vulvar hemangioma.

Capillary hemangioma, the most common tumor of infancy, usually does not require any treatment, but in a minority of cases it may cause morphologic, functional, or life-threatening complications. The authors report a case of a vulvar emangioma complicated by life-threatening hemorrhage not responsive to corticosteroids therapy, which showed no signs of spontaneous involution. The therapeutic approach consisted of 3 steps of selective arterial embolizations followed 5 months later by surgical excision and reconstruction of the vulva. Selective embolization rarely is used in the treatment of hemangiomas but may be required in cases of intractable bleeding, severe heart failure, large and poorly involuting hemangiomas, and as preparation for surgery. The results of the combined approach in this case are reported, and the indications discussed.

Intracranial contrast-enhancing masses in infants with capillary haemangioma of the head and neck: intracranial capillary haemangioma?

Contrast-enhancing intracranial masses are rarely found in infants with extracranial capillary haemangiomas (CH). We aimed to assess their nature and progression in three patients undergoing CT and/or MRI. The changes in size of both extra- and intracranial lesions were recorded. In a fourth case, a single examination was obtained. All patients harboured one or two enhancing intracranial nodular, meningeal-based lesions. Diffuse leptomeningeal enhancement of the cerebellar surface was also seen in one, which disappeared at follow-up. In all but one of the cases, the intracranial lesions were on the same side as the extracranial CH. These lesions and the extracranial CH demonstrated parallel changes in size (suggesting that both represent CH) during follow-up of 1-2 years: the size of intracranial lesions and the extracranial CH decreased in two cases, whereas it was unchanged in the third. One patient had a persistent trigeminal artery, while another had cerebellar atrophy with high signal in the cortex on T2-weighted images. In some cases, extracranial CH are part of PHACE syndrome; the association with intracranial CH might represent a peculiar phenotype of this rare vascular phakomatosis. As extracranial CH are known to regress spontaneously in the majority of cases, a conservative approach is recommended also for presumed intracranial CH; surgery should be avoided unless follow-up studies demonstrate growth.

Posterior fossa and arterial abnormalities in patients with facial capillary haemangioma: presumed incomplete phenotypic expression of PHACES syndrome.

We report on the neuroradiological studies performed on three infants with capillary haemangioma (CH) of the head and neck with associated posterior fossa and arterial abnormalities. Posterior fossa malformations were represented by cerebellar hemispheric and vermian hypoplasia and cerebellar cortical dysgenesis, whereas arterial anomalies included bilateral agenesis, kinking, and looping of the internal carotid arteries. One patient had marked exophthalmos due to intraorbital CH. We suggest that these patients had an incomplete phenotypic expression of PHACES syndrome, a vascular phakomatosis characterised by the variable association of posterior fossa malformations, CH, arterial anomalies, coarctation of the aorta and cardiac defects, eye abnormalities, and sternal and medioventral defects. Evidence suggests that PHACES syndrome is not a random association but a true phakomatosis; further studies are awaited to shed light on a possible genetic background. The phenotypic spectrum is broad and still largely unexplored, and precise diagnostic criteria have not yet been identified. A causal teratogenic influence, possibly related to anomalous expression of vascular growth factors and their modulators, is suggested to occur between gestational weeks 3 and 5.5

Surgical management of complete atrioventricular canal associated with tetralogy of Fallot.

Between 1984 and 1993, 12 children with an atrioventricular canal and tetralogy of Fallot underwent surgical repair. The mean(s.d.) age at operation was 58(18) months, and the mean(s.d.) body weight 15(4) kg. Nine patients underwent 11 palliative procedures. The ventricular septal defect was closed through a combined (right atrial and right ventricular) approach in nine cases, and through a right atrial approach in three, using a prosthetic patch with a wide anterior extension, secured with a running suture. The 'ostium primum' defect was closed with a separate prosthetic patch in 11 cases (double-patch technique). Right ventricular outflow obstruction was relieved by a composite infundibular patch (seven cases) or a transanular patch (five). There were four hospital deaths (33%). These were caused by low cardiac output in three cases and infection in one (three deaths occurred in patients with a transanular patch). One patient has so far died during follow-up. Assessment at 50(36) months by echo-Doppler showed moderate-to-severe 'mitral' regurgitation in three cases, and moderate 'tricuspid' regurgitation with right ventricular dysfunction in one case. Two patients have required further surgery.

Bidirectional cavo-pulmonary anastomosis with an additional flow source to the lungs: clinical experience in 21 cases.

The Fontan operation is considered a palliative procedure for the treatment of complex congenital heart diseases with a morbidity and mortality that are not negligible, especially in high-risk patients. In these cases, a bidirectional cavopulmonary anastomosis with additional source of blood flow to the lungs can represent a valuable surgical option. Between November 1992 and December 1995, 21 patients with univentricular heart physiology were submitted to bidirectional cavopulmonary anastomosis with additional blood flow the additional flow consisted of an aortopulmonary shunt in 13 cases, pulmonary artery banding in seven cases, and pulmonary artery stenosis in one case. Mean age at operation was 35.5 months (range 4 months to 12 years) and mean body weight 10.9 (range 4-24.4) kg. Hospital mortality was zero. Morbidity (pleuropericardial effusions) was significant in one case. There were three late deaths (14%) caused by worsening atrioventricular valve regurgitation: two of these occurred in patients with right isomerism. Late haemodynamic evaluation documented a significant increase in cardiac output (P < 0.01) and oxygen saturation (P < 0.05) during pharmacological stress testing. Angiographic characterization of blood flow distribution to the lungs showed better results when the additional blood flow was represented by an anterograde source (pulmonary artery banding or native pulmonary artery stenosis). In conclusion, bidrectional cavopulmonary anastomosis with additional blood flow is a good alternative to classic or fenestrated Fontan technique in high-risk patients. Secondly, the diagnosis of isomerism could be a contraindication to this type of surgery, because the persistent volume overload could worsen atrioventricular valve regurgitation. Thirdly, the pharmacological stress testing shows a favourable behaviour of cardiac output and arterial oxygen saturation. Fourth, the type of additional blood flow seems to condition both pulsatility and distribution of pulmonary blood flow.