Emergency pulmonary autograft mitral valve replacement in a child

Mitral valve replacement in small children imposes significant clinical difficulties because of the relatively small mechanical prosthetic valves required and the need for lifelong anticoagulation therapy. A child weighing 10.4 kg presented with thrombosis of her 19-mm mechanical mitral prosthesis 4 weeks after implantation despite appropriate oral anticoagulation therapy. An emergency mitral valve replacement with a pulmonary autograft was successfully performed with encouraging short-term results.     

Therapeutic strategies,including a high surgical ligation rate,for patent ductus arteriosus closure in extremely premature infants in a North American centre

OBJECTIVE:

To document the rate of surgical ligation of a patent ductus arteriosus (PDA) in extremely premature infants who had received more than one course of indomethacin. Outcomes were compared among three subgroups (ligation, further indomethacin and no further treatment) of infants who received at least one course of indomethacin, and between two subgroups (one course of indomethacin and more than one course) among infants who underwent ligation.

STUDY DESIGN:

A retrospective chart review of all 23 weeks+0 days to 26 weeks+6 days’ gestational age infants with a PDA born between 1994 and 2005 was performed. Secondary outcomes were compared among the subgroups.

RESULTS:

The final study population consisted of 196 extremely premature infants with a PDA. The rate of surgical ligation in the 88 infants who received more than one course of indomethacin was 64%. The ligation subgroup, in comparison with the no further treatment subgroup, spent a greater median time on mechanical ventilation (39 versus 29 days, P<0.001) and in hospital (115 versus 92 days P=0.002), while trending toward lower mortality (18% versus 40%, P=0.07). The PDA closed following the first course of indomethacin in only 20% of infants.

CONCLUSIONS:

A majority of extremely premature infants receiving more than one course of indomethacin underwent surgical ligation. Repeated indomethacin courses were generally well tolerated, but were mostly unsuccessful. Ligation appears to have potential risks and benefits. A randomized trial should be performed after studies define a hemodynamically significant PDA that will result in morbidity and/or mortality unless treated.     

R179H mutation in ACTA2 expanding the phenotype to include prune-belly sequence and skin manifestations

Mutations in ACTA2 (smooth muscle cell-specific isoform of α-actin) lead to a predisposition to thoracic aortic aneurysms and other vascular diseases. More recently, the ACTA2 R179H mutation has been described in individuals with global smooth muscle dysfunction. We report a patient heterozygous for the mutation in ACTA2 R179H who presented with megacystis at 13 weeks gestational age and, at birth, with prune-belly sequence. He also had deep skin dimples and creases on his palms and soles, a finding not previously described but possibly related to ACTA2. To our knowledge, this is the first report of the R179H mutation in ACTA2 in a child with prune-belly sequence. We think the R179H mutation in ACTA2 should be included in the differential diagnosis of individuals presenting with the sequence without an identified mechanical obstruction. Furthermore, as ACTA2 R179H has been reported in patients with severe vasculomyopathy and premature death, we recommend that molecular testing for this mutation be considered in fetuses presenting with fetal megacystis with a normal karyotype, particularly if the bladder diameter is 15?mm or more, to allow expectant parents to make an informed decision.     

Descriptive retrospective analysis of the diagnostic yield and morbidity of four vessel catheter-directed cerebral angiography and multidetector computed tomographic angiography (MDCTA) performed at Inkosi Albert Luthuli Central Hospital (IALCH)

Imaging studies are indispensable in order to determine the source, location and pattern of intra and extra-axial brain haemorrhages. In our study of 277 patients carried out over an 18 month period, the most common reason of referral was subarachnoid haemorrhage followed by trauma.Aneurysms were the most common diagnosis (36%) with anterior and posterior communicating arteries being the most common locations. Fifty percent (50%) of patients investigated had a normal study.Our findings show that the yield from MDCTA and conventional angiography was relatively comparable, however, conventional angiography was superior in detection of aneurysms; hence, in cases were the MDCTA result was found to be normal despite a high index of suspicion for a subarachnoid haemorrhage, a follow-up MDCTA study or conventional angiography is useful. Moreover, conventional angiography was superior in detection of multiple aneurysms.     

Lemierre syndrome caused by Arcanobacterium haemolyticum

A54-year-old male smoker was admitted to our hospital after undergoing the drainage of a left peritonsillar abscess.Ten days prior to admission (day 1),he complained of a "scratchy" throat with a fever of 38.5°C.He had an enlarged left tonsil with prominent exudate;white blood cell count (WCC) 8.8×109/L;platelet count (Pit) 103 × 109/L;chest X-ray was normal in a medical clinic.Then the patient received an intravenous injection of 4 million units of penicillin G every 6 hours and 500 mg of oral azithromycin once daily.But his condition worsened with a temperature of 41°C and laryngoscopy confirmed a marked left-sided peritonsillar abscess which was drained.On day 10,the patient was transferred to our hospital after developing persistent fever,rigors,left-sided neck pain,pain on swallowing and purulent bloody sputum.On examination,his temperature was 39.6°C,pulse 95 beats/min;respiratory rate 25 breaths/min and blood pressure 110/80 mmHg (1 mmHg=0.133 kPa).The left side of his neck was swollen and tender,anterior to the sternocleidomastoid muscle.