Intrapericardial teratoma in a twin with severe failure to thrive.

We report on one affected twin who presented with cough, dyspnoea and severe failure to thrive. He was found to have gross cardiomegaly on chest X-ray. This proved to be due to an intrapericardial teratoma with an associated pericardial effusion. The operation on this rare tumour was successful.

Low molecular weight heparin in the successful treatment of a spontaneous aortic thrombosis in a neonate.

A successfully treated case of a spontaneous aortic thrombosis in a neonate is described as an illustration of the advantages of using low molecular weight heparin (LMWH) over unfractionated heparin (UFH) for anticoagulation therapy. A 5-day-old neonate presented with an abdominal aortic thrombosis detected by echocardiography. Intravenous UFH was commenced following thrombectomy. Poor venous access made monitoring the anticoagulation therapy problematic. Subcutaneous LMWH was substituted for UFH. It requires substantially less monitoring and no intravenous access, has fewer side effects, and allows for much earlier discharge from the hospital. Extensive investigation for a hypercoagulable state revealed no definite cause for the thrombus. The findings of homozygosity for a methylene tetrahydrofolate reductase (MTHFR) mutation and a mildly elevated homocysteine level are interesting but unlikely to account for the thrombotic event in this case. Anticoagulation with LMWH proved effective and more convenient than using UFH.

Sterile vegetations in children with severe tetralogy of Fallot.

The finding of bland, sterile vegetations in children with severe tetralogy of Fallot is unexpected, and to our knowledge, has not been reported previously. Eight patients diagnosed with tetralogy between January 1993 and July 1997 had sterile vegetations proven by histological and microbiological evaluation, in their right ventricular outflow tracts. Four of these patients were experiencing severe hypercyanotic spells, and four had severely reduced effort tolerance at presentation. They all underwent cardiac catheterization and were submitted for surgical repair. At surgery, the vegetations were thought to be causing further narrowing of the already tight fibrotic infundibular stenosis. Two of these patients had evidence of damaged valves, without evidence of active endocarditis. Although initially sterile, these vegetations, may in some instances, become infected.

A review of Takayasu's arteritis in children in Gauteng, South Africa.

We have reviewed 31 patients with Takayasu's arteritis followed at two pediatric nephrology units in Gauteng, South Africa over a 15-year period. There were 25 black patients, 4 white, and 2 of mixed race. The mean age at diagnosis was 8.42+/-3.59 (range 2.4-14.5, median 8) years. The most common presenting sign was hypertension, followed by cardiac failure, bruits, and absent pulses. The Mantoux test was strongly positive in 27 patients (90%, control population 5%). Markers of activity included a raised erythrocyte sedimentation rate (23 patients) or Gallium single photon emission tomography (positive in 12 of 16 patients). Angiography revealed type II (abdominal aorta) and III (arch plus abdominal aorta) lesions to be most common (11 in each group). All patients received antituberculous therapy and most low-dose aspirin for its antithromboxane effect. Corticosteroids and further immunosuppression were used to control disease activity. We added total lymphoid irradiation (TLI) or cyclophosphamide. Twenty-six patients in all received further immunosuppression, with 13 patients in each group. Results were similar in the two groups, with similar pre- and posttherapy systolic blood pressures and creatinine clearances. Two patients in each group relapsed, 3 died in the TLI group and 2 in the cyclophosphamide group. Surgical intervention, usually in the quiescent phase, consisted mainly of renal autotransplantation. Because of the problems with TLI and 2 patients with papillary carcinoma of the thyroid with long-term follow-up, we no longer use TLI. We have shown that with active medical and surgical intervention the aggressive course of this disease in children can be modified.

Atrioventricular septal defect and type A postaxial polydactyly without other major associated anomalies: a specific association.

Four children are described, (three black and one white, two boys and two girls) with type A postaxial polydactyly. All four of them, in addition, had either a partial or complete atrioventricular septal defect (AVSD). None of these children had associated major malformations. Minor anomalies were observed (e.g., two patients with hypersegmentation of the sternal segments, one patient with undescended testes, one patient with hypoplastic lumbar vertebra, and one patient with a degree of craniofacial abnormality). Chromosome analysis was carried out for three of the four patients, and was normal in all of them. It is suggested that there is a specific association between type A postaxial polydactyly and the AVSD found in each of these patients. This picture does not conform to, but bears some resemblance to, the Ellis-van Creveld syndrome.

Long-term follow-up of coarctation of the aorta repaired by patch angioplasty.

The long-term complications of patch plasty repair for coarctation of the aorta were assessed retrospectively in 119 patients who were operated upon from 4 days to 13 years of age. There were 7 late deaths and 17 patients were lost to follow-up. Thus 95 patients were followed up for a minimum period of 3 years (mean 6.3 years). In addition, graded exercise tests were performed on 15 patients and 11 normal controls, measuring systolic blood pressure response and arm-leg blood pressure gradients. In this series, 16 patients (17%) were hypertensive, while 25 (26%) had a resting systolic arm-leg gradient greater than 20 mm Hg. Re-coarctation occurred more frequently when surgery had been undertaken under 1 month of age. Review of chest X-rays revealed calcification in the patch in 4 patients, one of whom subsequently went on to aneurysm formation. In a second child aneurysm formation was detected at repeat operation. Exercise tests showed a significantly higher systolic arm pressure in patients (mean: 165.3 mm Hg) when compared to controls (mean: 139.2 mm Hg) (P = 0.017) and a significant increase in arm-leg systolic gradient viz. 36 and 5.9 mm Hg, respectively (P = 0.0016). A good correlation was found between the systolic arm pressure and the systolic arm-leg gradient after exercise (r = 0.822; P = 0.0001). We conclude that the most important long-term complication following an aortic patch plasty, is re-stenosis. The development of an aneurysm was observed only twice. We believe that this apparently rare occurrence, as assessed on chest radiographs; is related to the young age (53% under 1 year) at which the repairs were carried out. Another possible reason may be that the follow-up period is not yet long enough.

Surgical correction of ventricular septal defects in black and white children--an 11-year study.

The clinical and ECG features, anatomical subtypes and outcome in 309 children (169 black; 140 white; 58% female) who underwent surgical closure of ventricular septal defects (VSDs) are presented. Clinical presentation was more severe in the black children; with recurrent pulmonary infections in 65% blacks compared with 34% whites. Congestive cardiac failure was noted in 60% blacks and in 37% whites. At cardiac catheterisation a left to right shunt greater than 2.5/1 was found in 45.5% of the black and in 39.3% of the white children. Severe pulmonary hypertension (greater than 80% of systemic systolic pressure) was evenly distributed in both groups. Of the 140 white children, 74.3% underwent surgery under the age of 2 years compared with 68.6% of the 169 blacks. A perimembranous VSD was found in 65% of patients in each group. Infundibular (muscular outlet) defects were observed in 28.6% of white and 30.8% of black children. Left axis deviation (LAD) on ECG was found in 11.9% of white and 9.2% of black patients; and 93% of the total of 27 cases had a perimembranous defect. Early mortality was 3.6% in white and 7.1% among the black children. Of the 17 fatal cases in the total group, 16 had severe pulmonary hypertension. It is concluded that: (i) operative results compared favourably with those reported elsewhere; (ii) the anatomical subtypes occurred with equal frequency in both ethnic groups; and (iii) this was also the case for LAD on ECG, which was most commonly associated with a perimembranous VSD.

Posterior d-transposition of the great arteries with an unusual form of aortic obstruction.

An unusual case of posterior transposition of the great arteries, with previously undescribed aortic obstruction is described. The patient, who underwent an arterial switch operation, was found at surgery to have a mass of myxomatous tissue arising from the tricuspid valve. This finding was responsible for the hemodynamically significant aortic stenosis.

Origin of left coronary artery from right pulmonary artery co-existing with coarctation of the aorta.

We report 2 infants with the unusual combination of origin of the left coronary artery from the right pulmonary artery as well as coarctation of the aorta. In the first case congestive cardiac failure persisted despite satisfactory repair of the coarctation. Further investigation revealed the anomalous origin of the left coronary artery. Corrective surgery, 24 days later was unsuccessful. When an identical diagnosis was made in the second child, it was decided, at a single operation, to combine correction of the coarctation together with re-implantation of the anomalous left coronary artery into ascending aorta. This patient has survived, and to our knowledge is the first such case with this outcome. The haemodynamic reasons for this approach are discussed.

Aortic arch interruption in the neonate, with emphasis on early diagnosis and management.

Interruption of the aortic arch (IAA) is a rare but lethal congenital cardiovascular anomaly. Over a 3-month period we encountered 5 neonates with IAA, all of whom presented in the 1st week of life with congestive cardiac failure and diminished or absent pulses. All 5 underwent surgery, and 3 died from causes unrelated to the surgical repair. The diagnosis of this condition is difficult to make, although the absence of pulses may provide an important clinical clue (particularly if the left brachial and femoral pulses are involved). Using prostaglandins to re-establish ductal patency may be life-saving in these patients, since this restores blood flow to the lower trunk and kidneys. Administration of oral prostaglandin E2 to infants in whom this anomaly is suspected before they are referred to a tertiary care centre is therefore vital.

Surgical correction for one pulmonary artery arising from ascending aorta--report of five cases.

Over a 10-year period we encountered 5 infants with a pulmonary artery branch arising from ascending aorta. Surgical re-implantation of this vessel was carried out at ages 2.5, 5, 8, 9, and 19.5 months. Pre-operative cardiac catheterization demonstrated severely raised pulmonary artery pressures in all, equal to systemic in 3, and suprasystemic in 2. Four patients had origin of the right pulmonary artery from ascending aorta with a left aortic arch, and the remaining patient had an anomalous left pulmonary artery associated with a right-sided aortic arch. All patients had substantial reduction in pulmonary artery pressures immediately following surgery. One patient died 18 days post-operatively from extensive lung disease. In all 4 of the survivors, post-operative cardiac catheterization (11 to 85 months after surgery) has shown a drop in pulmonary artery pressures. One patient has been left with mildly elevated systolic values but normal diastolic levels. In 2 of the children, mild stenosis has been found at the site of reimplantation of the pulmonary artery. This anomaly should always be considered as a cause in the setting of a large left to right shunt with tricuspid incompetence and severe right ventricular hypertrophy. Prompt surgical repair, after confirmation of the diagnosis, should prevent death from heart failure or the development of irreversible pulmonary vascular disease.

Hypertension as the major problem of idiopathic arterial calcification of infancy.

We describe two infants with severe hypertension associated with idiopathic arterial calcification of infancy. In both children, blood pressure control was refractory to aggressive antihypertensive therapy. There was radiologic and laboratory evidence of renovascular disease requiring the use of specific renin antagonists and later nephrectomy, resulting in moderate improvement of hypertension. Although on occasion there is a familial incidence, in the vast majority of cases the diagnosis hinges on a high index of suspicion.

Patch graft aortoplasty for repair of coarctation of the aorta in infants under 1 year of age.

Patch graft angioplasty for symptomatic coarctation of the aorta was carried out in 34 infants aged from 6 days to 9,5 months (mean 49,4 days). Congestive cardiac failure was present in 32 and severe hypertension alone in 2 patients. Of the 34 children, 21 (61,8%) had significant associated cardiac lesions and 25 (73,5%) were less than 6 weeks of age. Systolic blood pressures were recorded by the Doppler technique before and after the operation. There were 6 hospital deaths (17,6%) and 3 late deaths, giving a total mortality of 26,5%. Pre-operative systolic pressure gradients between upper and lower limbs ranged from 20 to 92 mmHg (mean 54,5 mmHg). Of the 28 early survivors, 1 was lost to follow-up. Duration of follow-up ranged from 3,5 to 62 months (mean 24,5 months). Postoperative residual gradients of less than 20 mmHg were found in 19 of the 27 cases followed up (70,4%), while 8 patients had significant residual gradients (40 - 90 mmHg). Of these 8, 7 had undergone surgery when less than 6 weeks old, and 6 of them have subsequently undergone a second corrective operation. It is concluded that while patch graft angioplasty is a life-saving procedure for the correction of symptomatic coarctation of the aorta in early infancy, a significant proportion of the patients develop a recurrence of coarctation necessitating further surgery later.

Surgical correction of combined supravalvular and valvular aortic stenosis in homozygous familial hypercholesterolaemia.

Although premature coronary atherosclerosis is a well-recognized complication of homozygous familial hypercholesterolaemia, involvement of the aortic root and valve has not been recognized frequently during life. Two cases of supravalvular and valvular aortic stenosis due to familial hypercholesterolaemia are described. Both patients underwent successful surgical correction of these lesions. The importance of relieving left ventricular outflow tract obstruction by a technique of aortic root enhancement and oblique insertion of a prosthesis is stressed.

Arteriohepatic dysplasia: association of liver disease with pulmonary arterial stenosis as well as facial and skeletal abnormalities.

The clinical features and course of five children with the recently described syndrome of arteriohepatic dysplasia are presented. All had bilateral pulmonary arterial stenosis, proven at cardiac catheterization, as well as associated liver disease of varying severity. In one of the fatal cases, a hitherto undescribed anomaly was found--stenosis of the right coronary artery ostium. A viral etiology, eg, the congenital rubella syndrome, was considered most likely, but detailed investigations proved to be negative.

Rhabdomyoma of the heart in a newborn infant. Diagnosis by echocardiography.

An intracavitary right ventricular tumour was identified echocardiographically before operation in an infant with a rhabdomyoma of the heart. Necropsy showed associated tuberous sclerosis.

The use of a curved uterine vulsellum for removal of rectal foreign bodies: report of a case.

Removal of retained foreign bodies can be a tedious process, requireing considerable skill and ingenuity on the part of the surgeon. Employment of a curved, double-pronged, uterine vulsellum can be useful in simplifying removal of certain of these objects. A method which can be employed on an outpatient basis is described. When this method fails, suitable cooperation by the patient is lacking, or there is evidence of serious rectal injury or perforation, hospitalization for appropriate treatment under general or regional anesthesia is indicated.

Occurrence of primary hepatocellular cancer and peliosis hepatis after treatment with androgenic steroids.

Three patients are reported in whom treatment of Fanconi's anaemia with androgenic steroids was complicated by the development of either primary hepatocellular cancer (PHC) or peliosis hepatis. The first, a White woman aged 34 years, was found to have PHC after receiving first methyltestosterone and then oxymetholone for a total period of 7 years. She died 4 months after the diagnosis was made. The other 2 patients were White children who presented with peliosis hepatis after receiving methyltestosterone and oxymetholone for 8 years and oxymetholone for 5 years, respectively. Both died from their primary diseases shortly after oxymetholone treatment was discontinued. Possible pathogenic mechanisms involved in the development of these serious complications are discussed and the therapeutic dilemma raised by their occurrence is emphasised.