Optimizing evaluation in pediatric and young adult patients with Marfan syndrome: Novel longitudinal metrics to track growth of aortic structures.

Objective: Surveillance metrics in pediatric and young adult Marfan syndrome (pMFS) are challenging. We evaluated the utility of aortic root cross-sectional area/height index (CSA/Ht) on echocardiogram among pMFS patients as a risk stratification and surgical triage metric.

Methods: Genotype or phenotype positive pMFS patients aged 25 years or younger seen at our center from 2001 to 2020 were identified.

Unusual coronary artery arrangement in D-transposition of the great arteries with ventricular septal defect.

A term female with prenatally diagnosed D-Transposition of the great arteries, large membranous ventricular septal defect with inlet extension, moderate secundum atrial septal defect, and large patent ductus arteriosus (Fig 1) was born by scheduled caesarean section. Transthoracic echocardiogram confirmed the anatomy with both coronary arteries arising from a single sinus with separate ostia. The right coronary artery arose from right posterior facing sinus (Fig 2).

Streamlining Pediatric Emergency Medicine at a Tertiary-care Hospital of a Low- to Middle-income Country.

The factors of integral importance to run any pediatric emergency department efficiently are the ability to process a high volume of patients quickly and a sensitive triage system that identifies the sickest children. Achieving these aims in a low- to middle-income country setting is more complex as a result of scarce resources and data on which to base systems. In this article, we discuss existing models of streamlining pediatric emergency department services that are most applicable to resource-limited countries, and present suggestions for streamlining pediatric emergency care in such countries.

Imaging and intervention for coronary artery disease following irradiation of malignant thymoma.

Thymomas are rare malignant epithelial growths, constituting 20% of mediastinal tumours. Resection followed by irradiation may be employed in all thymomas except for stage 1 thymomas. Mediastinal irradiation is associated with coronary artery disease.

Benign granulomatous polyp obstructing the bronchus.

Inflammatory endobronchial polyps are rarely encountered. We report a case of a 14 years old girl with a benign granulomatous polyp originating in the bronchus. Computed tomography scan showed an intraluminal soft tissue opacity while fiberoptic bronchoscopy revealed a large soft tissue mass completely occluding the left lower lobe.

A radiographic analysis of ventricular trajectories.

Background: The prevalent method of ventriculostomy placement is via freehand insertion to cannulate the ventricle at a 90° angle to the skull to get ideal placement. Our goal was to test the validity of this practice in patients without midline shift and with normal ventricular size.

Methods: This study was a virtual radiographic analysis of 3-dimensional data of skull and ventricular anatomy.

The clinical, endoscopic and histological spectrum of the solitary rectal ulcer syndrome: a single-center experience of 116 cases.

Background: Solitary rectal ulcer syndrome (SRUS) is an uncommon although benign defecation disorder. The aim of this study was to evaluate the variable endoscopic manifestations of SRUS and its association with other diseases.

Methods: All the patients diagnosed with SRUS histologically from January 1990 to February 2011 at The Aga Khan University, Karachi were included in the study.

A US-based survey on ventriculostomy practices.

Background: The incidence of ICP monitoring has increased over the years and the indications for placement have expanded. Although ventriculostomy and ICP monitor placement are among the most commonly performed neurosurgical procedures, the current practice patterns have rarely been studied.

Methods: A 10-question survey was sent to 2006 neurosurgeons and 1060 neurosurgery residents in the US.