Bilateral fibular hemimelia with recto-vestibular fistula associated with amniotic bands.

Fibular Hemimelia is a rare congenital disorder of deficiency to complete absence of fibula. It has been associated with post axial skeletal anomalies like shortening of femur, absence of lateral rays of foot and syndactyle, polydactyle etc. We report this case because of associated amniotic constriction bands over both the forearm, suggesting amniotic bands may be one of the causal factors of Fibular Hemimelia due to insult to the growing limb bud.

Ataxica telengiectasia (Louis-Bar syndrome).

A case of ataxia telengiectasia in a 7-year-old girl with symptoms for last 3 years has been reported in detail. The necessary literature has been reviewed with proper references.

Hypertension in the young in eastern India.

Three hundred and forty-one young hypertensives in the age group of 18-30 years were evaluated over a 7-year period. Essential hypertension constituted the single largest group (35.8%). Renal pathology was the most common cause of secondary hypertension (26.4%). Congenital coarctation of the aorta and endocrine causes accounted for 14.1 percent and 3.2 percent cases of secondary hypertension, respectively. A strikingly high incidence of nonspecific aortoarteritis (20.1%) was a distinguishing feature amongst secondary causes. Aortoarteritis was the commonest cause of renal artery stenosis. Renal angioplasty was performed in 11 patients with refractory hypertension. Forty percent of the patients achieved post-angioplasty control of blood pressure without drugs; in 25 percent, the blood pressure became easier to control. Restenosis was detected in 4 cases over 18-24 months of follow-up.

Pacing in children and adolescents.

Sixty patients aged 5-16 years underwent implantation of permanent pacemaker and were followed up for 3-11 years. All these patients presented with syncope or symptomatic bradyarrhythmias; two also had congestive cardiac failure in addition, which was ameliorated by pacemaker implantation. A majority of the patients (48) had congenital complete heart block. 8 patients presented with sick sinus syndrome; 4 patients presented with post operative chronic complete heart block. Transvenous endocardial electrode was used in 54 cases and epicardial pacing was done in 6 cases. A majority (55) of the patients received VVI pacemakers; AV sequential pacemakers were implanted in 5 cases. On follow up, the children returned to normal activity and their psychological well being was striking. Reoperation had to be done in 35 cases, a majority being due to over stretching of electrodes (15) and battery exhaustion (12). Two patients died, one due to bacterial endocarditis and the other succumbed to sudden death during vigorous physical activity. Major problems of pacing in children are that veins are often too thin and delicate for electrode insertion, and of the child's growth that stretches the lead system. The problems of pulse generator size and longevity have been partly overcome by introduction of newer models that are small and programmable.

Low dose amiodarone in refractory tachyarrhythmias.

Fifty patients with drug resistant tachyarrhythmias were treated with amiodarone for 6-22 months; 16 for recurrent ventricular tachycardia (VT), 2 for VT followed by ventricular fibrillation (VF), 14 for complex ventricular ectopics, and 18 for supraventricular tachyarrhythmias (SVT). Amiodarone was administered in a dose much lower than that used in western trials. The actual incidence of successful amiodarone therapy was 81.2% at 22 months for patients with VT. Among the patients with SVT, 88.6% patients were successfully treated for 22 months (range 3-22 months). Amiodarone toxicity appeared in 22 of 50 patients (44%) treated for more than 12 weeks. Withdrawal of therapy was required in 4 patients. Despite the lower dose, clinical efficacy and onset of action were comparable to the western experience.

Cardiac changes implicated in chronic heart block.

Fifteen cases of chronic heart block were studied. Eight of them could be designated as idiopathic or primary heart block; the others were associated with hypertension, diabetes and ischaemic heart disease, either singly or in various combinations. In six cases, the whole heart was available for histopathological study of the conduction system. In the other 9 cases, only a portion of the heart muscle was available for examination. A V nodal fibrosis extending upto the proximal bundle of His was seen in all the six whole heart autopsy materials. Fibrosis of the adjacent myocardium was seen in five cases. In three cases, conducting system fibrosis was associated with atherosclerotic (1 case) or diabetic changes (3 cases) of the intramural vessels. In the 9 partial autopsy studies, myocardial fibrosis was seen in two cases, diabetic microangiopathy in one and atherosclerotic changes in two including an old thrombus in one. Thus, diabetic microangiopathy was seen in total four cases. These changes may be responsible for the cardiomegaly and cardiac failure associated with conduction defects observed in diabetes. In the idiopathic group also, heart block could be considered as a significant facet of a primary myocardial degenerative process.

Aortic dissection in a non-marfanoid child.

A nine year old girl presented with chest pain and dyspnoea of sudden onset. She had no marfanoid features, with a normal slit lamp examination and a normal aortic valve on echocardiography. The diagnosis of dissecting aneurysm of the ascending aorta involving the aortic valve cusps, the right coronary artery and the pericardium was later established with the help of ECG, pericardiocentesis, echocardiography and aortography.

An unusual extension of pericardial effusion in a case of pulmonary adenocarcinoma.

A case of pericardial metastasis from adenocarcinoma of lung was having a separate paratracheal bulge at left border of aorta mimicking a tumour. This was a very uncommon extension of pericardial effusion. Interestingly this mediastinal bulge on left border of aorta disappeared completely immediately after pericardio-centesis. The rarity of chest roentgenographic findings prompted us to report this case.