Anorectal Manometry in Children: The Update on the Indications and the Protocol of the Procedure.

Anorectal disorders are common in children. They are related to structural and/or functional abnormalities of the anorectum or pelvic floor with a variety of symptoms. Therefore, diagnostic tests to evaluate anorectal function can help to better understand the underlying pathophysiology and aetiology as well as facilitate patient management.

Use of Simulation to Visualize Healthcare Worker Exposure to Aerosol in the Operating Room.

Simulation resources offer an opportunity to highlight aerosol dispersion within the operating room environment. We demonstrate our methodology with a supporting video that can offer operating room teams support in their practical understanding of aerosol exposure and the importance of personal protective equipment.

Gastroesophageal Reflux Disease and Foregut Dysmotility in Children with Intestinal Failure.

Gastrointestinal dysmotility is a common problem in a subgroup of children with intestinal failure (IF), including short bowel syndrome (SBS) and pediatric intestinal pseudo-obstruction (PIPO). It contributes significantly to the increased morbidity and decreased quality of life in this patient population. Impaired gastrointestinal (GI) motility in IF arises from either loss of GI function due to the primary disorder (e.

Atherosclerotic Risk Factors in Children with Celiac Disease.

Results: We found significantly lower concentrations of total cholesterol, lipoprotein LDL-C, apolipoproteins A1 and B, as well as hCRP in all children with CD. We showed decreased level (<5 ng/mL) of folic acid among 46% of children treated for >5 years. Moreover, we showed significant decrease of folic acid level already after 1 year of a GFD (12 .

Seeing Through the Lies: Surgical Innovation and the Need for Transparency.

The Robert E Gross Lecture was delivered by Professor Martin Elliott of London, at Boston Children's Hospital June 1st, 2016. Professor Elliott makes a plea for revision to the system of medical publication and academic reward, especially in the field of surgery.

The Society of Thoracic Surgeons Congenital Heart Surgery Database: 2017 Update on Outcomes and Quality.

The Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital and pediatric cardiac surgical clinical data registry in the world. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This report summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency.

The Society of Thoracic Surgeons Congenital Heart Surgery Database: 2016 Update on Outcomes and Quality.

The Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital and pediatric cardiac surgical clinical data registry in the world. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This article summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency.

Aorto-Tracheopexy in the Treatment of Hunter Syndrome.

Hunter syndrome is a multisystem genetic disease in which a significant proportion of morbidity and mortality arise from respiratory dysfunction. Notably, tracheal abnormalities in Hunter syndrome can compromise clinical stability, and management is primarily supportive. We describe here the first successful implementation of aorto-tracheopexy in a 19-year-old patient as a surgical strategy to resolve progressive respiratory deterioration.

Frontofacial advancement by distraction osteogenesis: a long-term review.

Background: Frontofacial advancement by distraction osteogenesis is known to produce significant functional and aesthetic gains in the short and medium term. Little is known about the long-term effects of this technique. The authors present a long-term review of frontofacial advancement by distraction in patients with syndromic craniosynostosis.

The use of de-epithelialized skin flap in the surgical repair of terminal myelocystoceles.

Background: Terminal myelocystocele is a severe form of spinal dysraphism characterized by cystic expansion of the terminal spinal cord that herniates through a deficiency of the posterior sacral spinal elements to fuse with the subcutaneous fat. Postnatal enlargement of the subcutaneous fluid-filled sac may result in progressive neurological deficit and threaten the viability of the overlying skin. Surgical repair entails spinal cord untethering, resection of nonfunctional neural elements and watertight reconstruction of the terminal thecal sac.

Telephone cognitive-behavioral therapy for adolescents with obsessive-compulsive disorder: a randomized controlled non-inferiority trial.

Objective: Many adolescents with obsessive-compulsive disorder (OCD) do not have access to evidence-based treatment. A randomized controlled non-inferiority trial was conducted in a specialist OCD clinic to evaluate the effectiveness of telephone cognitive-behavioral therapy (TCBT) for adolescents with OCD compared to standard clinic-based, face-to-face CBT.

Method: Seventy-two adolescents, aged 11 through 18 years with primary OCD, and their parents were randomized to receive specialist TCBT or CBT.

Defining success in infant penetrating keratoplasty for developmental corneal opacities.

Background And Purpose: Neonatal corneal opacities (NCO) are one of the common causes of visual impairment in infants. We present the two infants with penetrating keratoplasty (PKP) for unilateral NCO with different visual and graft outcomes, and illustrate the importance of timing of surgery, importance of visual rehabilitation, and the lack of necessity of a clear graft to achieve reasonable visual function in infants with NCO.

Patients And Methods: Two infants with unilateral NCO (Peters anomaly) underwent PKP at age of 5.

Effect of Vacuum on Venous Drainage: an Experimental Evaluation on Pediatric Venous Cannulas and Tubing Systems.

Introduction: To observe how vacuum assisted venous drainage (VAVD) may influence the flow in a cardiopulmonary bypass circuit with different size of venous lines and cannulas.

Methods: The experimental circuit was assembled to represent the cardiopulmonary bypass circuit routinely used during cardiac surgery. Wall suction was applied directly, modulated and measured into the venous reservoir.

Recent advances in chronic granulomatous disease.

Chronic Granulomatous Disease (CGD) is a primary immunodeficiency caused by abnormities in the NADPH Oxidase that is involved in the respiratory burst responsible for initiating the killing of microbes ingested by phagocytic cells. The hallmark of CGD is recurrent infection but the inflammatory complications can prove difficult to treat. New insights into the mechanisms responsible for the inflammatory complications have led to new therapies.

D-transposition of the great arteries: the current era of the arterial switch operation.

This paper aims to update clinicians on "hot topics" in the management of patients with D-loop transposition of the great arteries (D-TGA) in the current surgical era. The arterial switch operation (ASO) has replaced atrial switch procedures for D-TGA, and 90% of patients now reach adulthood. The Adult Congenital and Pediatric Cardiology Council of the American College of Cardiology assembled a team of experts to summarize current knowledge on genetics, pre-natal diagnosis, surgical timing, balloon atrial septostomy, prostaglandin E1 therapy, intraoperative techniques, imaging, coronary obstruction, arrhythmias, sudden death, neoaortic regurgitation and dilation, neurodevelopmental (ND) issues, and lifelong care of D-TGA patients.

Recessive mutations in a distal PTF1A enhancer cause isolated pancreatic agenesis.

The contribution of cis-regulatory mutations to human disease remains poorly understood. Whole-genome sequencing can identify all noncoding variants, yet the discrimination of causal regulatory mutations represents a formidable challenge. We used epigenomic annotation in human embryonic stem cell (hESC)-derived pancreatic progenitor cells to guide the interpretation of whole-genome sequences from individuals with isolated pancreatic agenesis.

Do tracheas grow after slide tracheoplasty?

Background: Slide tracheoplasty has become the surgical technique of choice for repair of congenital tracheal stenosis. Despite the initial reluctance regarding the ability of this "reconstructed" trachea to grow, the reduced morbidity and mortality have allowed slide tracheoplasty to be widely adopted. The aim of this study was to evaluate tracheal growth after slide tracheoplasty.

A new morphologic classification of congenital tracheobronchial stenosis.

Background: Congenital tracheal stenoses are rare and life-threatening anomalies, associated with considerable variation in both morphology and prognosis. They have been classified previously according to the length of the stenosis or the severity of the symptoms, but not according to bronchial involvement.

Methods: Data from patients who underwent slide tracheoplasty for long-segment (>50%) congenital tracheal stenosis were collected.

Changing attitudes to the management of hypoplastic left heart syndrome: a European perspective.

Background And Aims: Several years ago, one of us described the difference in attitude to patients with hypoplastic left heart syndrome in the United States of America and Europe as similar to that between Mars and Venus. Uncertainty remains with regard to the long-term prognosis for patients with hypoplastic left heart syndrome. This prognosis may be considered in terms of survival, functional performance, including exercise capacity and neurodevelopment, as well as psychosocial effects on the patient, family, and siblings.

First experience with biodegradable airway stents in children.

Purpose: We here report our experience with biodegradable polydioxanone stents for tracheal narrowing in children.

Description: Eleven custom-made polydioxanone stents were implanted in 4 patients with airway narrowing due to external compression or intrinsic collapse. The median stent diameter was 9 mm (range, 6 to 14 mm) and median length was 15 mm (range, 13 to 70 mm).

Bronchomegaly as a complication of fetal endoscopic tracheal occlusion. A caution and a possible solution.

Fetal medicine is developing rapidly and aims to improve the outcome for fetuses with congenital anomalies. Fetal endoscopic tracheal occlusion (FETO) has been developed for fetuses with congenital diaphragmatic hernia to counterbalance the compression of the lung by the abdominal viscera, preserving the pulmonary maturation. Because the perinatal morbidity and mortality of patients treated with FETO have decreased, new complications are emerging in the older survivors.

Surgical outcome of slide tracheoplasty in patients with long congenital segment tracheal stenosis and single lung.

Objective: The aim of the study is to assess the surgical outcome of slide tracheoplasty in patients with congenital tracheal stenosis and single lung.

Methods: Pre, intra- and postoperative data were collected. Anatomy and associated anomalies were described.