Lenticulostriate vasculopathy in twin-to-twin transfusion syndrome.

Intracranial pathology is a common and important complication in extremely low birth weight babies. Lenticulostriate vasculopathy (LSV) is an abnormal finding on cranial ultrasounds of sick babies and has been associated with congenital infection, chromosomal aberration and twin-to-twin transfusion. We describe a previously unreported situation of LSV being detected in both donor and recipient twin. This pair of monochorionic, diamniotic twins was admitted to the Neonatal Intensive Care Unit at 28 weeks of gestation. The mother underwent an emergency caesarean section because ultrasound and Doppler studies showed stage III twin-to-twin transfusion syndrome. The first twin weighed 998 g and second twin weighed 600 g. The first twin had an uneventful stay, whereas the second twin needed prolonged continuous positive airway pressure and indomethacin for patent ductus arteriosus. Both of them developed LSV. The clinical significance of this condition on the neuro-developmental outcome of a neonate has not yet been fully determined.     

Evaluation of the behaviour of a thermal diffusion sensor in a high field strength magnetic resonance system: an experimental study.

Patients with acute brain pathology requiring ferromagnetic bio-medical implants for on-going invasive monitoring are largely excluded from the benefits of MRI scanning. We evaluated the behaviour of a thermal diffusion cortical blood flow (TD-CBF) sensor both in vitro (phantom gelatin model) and in vivo environments in a high field strength MRI system.Two baboons underwent cranial subdural implantation of 2 TD-CBF sensors/hemisphere and a single left parietal sensor was implanted subcortically to determine any deleterious effects. Using standard MRI sequences, artefact size, thermal effects, current generation, movement and reliability of recordings were assessed during scanning.The deflection forces were negligible, no observable thermal effects were demonstrated, while wide fluctuations in cerebral blood flow recordings were recorded. Mean image artefact size for implanted sensors was 6 times larger than in vitro. Patients with an implanted TD-CBF sensor may be safely imaged provided the device is disconnected. The MRI images obtained are of an acceptable quality.     

Impact of Retrograde Cerebral Perfusion on Ascending Aortic and Arch Aneurysm Repair

Purpose. The effect of retrograde cerebral perfusion on the incidence of stroke and death among patients undergoing repair of aneurysms of the ascending aorta and transverse arch was determined.

Material and Methods. Between January 1991 and March 1995, 161 patients were operated on for aneurysms of the ascending aorta and transverse arch. Thirty-three of the patients (20%) had an aneurysm of the ascending aorta only and 128 (80%) had aneurysms of both the ascending aorta and the transverse arch. All the patients underwent cardiopulmonary bypass, profound hypothermia, and circulatory arrest, and 120 (74%) also underwent retrograde cerebral perfusion. Median pump time was 143 minutes (range, 21 to 461 minutes). Median circulatory arrest time was 42 minutes (range, 8 to 111 minutes), and median myocardial ischemic time was 71 minutes (range, 14 to 306 minutes).

Results. The overall 30-day mortality rate was 6% (9 patients) and the incidence of stroke was 4% (7 patients). The use of retrograde cerebral perfusion demonstrated a protective effect against stroke (3 of 120 patients, or 3%) compared with no retrograde cerebral perfusion (4 of 41 patients, or 9%; odds ratio, 0.24; confidence interval, 0.06 to 0.99; p < 0.049). This was most significant in patients more than 70 years of age; none of the 36 elderly patients who received retrograde cerebral perfusion had a stroke, compared with 3 of the 13 (23%) who did not (p < 0.003). Only pump time was associated with an increased risk of stroke (odds ratio, 1.01; 95% confidence interval, 1.00 to 1.02; p < 0.005). Pump time also was associated with increased mortality (odds ratio, 1.01; 95% confidence interval, 1.00 to 1.02; p < 0.008).

Conclusion. Retrograde cerebral perfusion decreased the incidence of stroke in patients undergoing repair of aneurysms of the ascending aorta and transverse arch.     

Retrohepatic cavoatrial bypass for coarctation of inferior vena cava with a polytetrafluoroethylene graft

Eight patients with chronic Budd-Chiari syndrome resulting from coarctation of the inferior vena cava underwent operation. Transatrial dilatation was of no avail in the first case. The obstructed segment was directly visualized in the subsequent seven cases by a transthoracic, transdiaphragmatic, retroperitoneal approach. In these latter seven cases, severe hourglass constriction of the inferior vena cava was observed just above the right hepatic vein. There was no evidence of inflammation, extrinsic compression, or thrombosis. Retrohepatic cavoatrial bypass with an antibiotic-sterilized aortic homograft was unsuccessful in three patients. Five patients including one with homograft failure underwent successful retrohepatic cavoatrial bypass with a polytetrafluoroethylene graft (20 mm plain in four cases and 16 mm ringed graft in one case). These patients have been followed up for 21 months to 6 years with no recurrence of symptoms. The term coarctation of the inferior vena cava appears more appropriate than membranous obstruction of the inferior vena cava because of the operative findings in the present series.     

Segmental jejunal lipomatosis--a rare cause of intestinal obstruction

A rare case of a segmental small intestinal (jejunal) lipomatosis is described. A 33-year-old male was admitted with a clinical diagnosis of an acute intestinal obstruction. A plain erect abdominal x-ray showed multiple air-fluid levels. On an exploratory laparotomy, a jejunojejunal intussusception was found secondary to a segmental submucosal lipomatosis. This was treated by a segmental resection and anastomosis, which resulted in a complete cure. Here we present this case with a review of the relevant literature.     

Bilateral sternalis with unusual left-sided presentation: a clinical perspective

An unusual variation creates interest among anatomists, but is a cause of concern among clinicians when it mimics a pathology. The sternalis muscle is one such variant of the anterior chest wall located subcutaneously over the pectoralis major, ranging from a few short fibers to a well-formed muscle. We observed a bilateral case, which was accompanied by an atypical presentation on the left side where a huge, bulky sternalis muscle was associated with the absence of the sternal fibers of the pectoralis major. The fibers arose as a lateral strip from the upper two-thirds of the body of the sternum and costal cartilages 2 through 6 with the intervening fascia and aponeurosis of the external oblique. The right sternalis was strap-like and was placed vertically over the sternal fibers of the pectoralis major, arising from the underlying fascia and aponeurosis of the external oblique. The sternalis muscles, on each side, converged into an aponeurosis over the manubrium that was continuous with the sternal heads of the right and left sternocleidomastoid muscle, respectively. This rare anomaly has puzzled radiologists and surgeons in confirming diagnosis, missing it all together or mistaking it for a tumor on mammography or CT scan. These findings prompted us to review its topography, development, and application in relation to the anterior chest wall.     

Use of VA ECMO and percutaneous palliation of ductal dependent coarctation in a neonate with trisomy 21 and COVID-19 pneumonia

We present a case of a neonate with trisomy 21, ductal-dependent aortic coarctation, and severe respiratory failure secondary to coronavirus disease 2019 (COVID-19) pneumonia. The neonate was managed with venoarterial extracorporeal membrane oxygenation (VA ECMO), palliative stenting of the coarctation, and a vascular plug occlusion of a large patent ductus arteriosus. The patient was successfully weaned off extracorporeal membrane oxygenation (ECMO). The patient is currently awaiting a definitive surgical repair in the near future.