congenital surgery

BACKGROUND: Congenital heart defects are common and occur in approximately 0.9% of births. In France, the registries cover approximately 20% of the population but not the entirety of France; therefore, we aimed to update the incidence data for congenital heart defects in France from 2013 to 2022 using the medico-administrative database PMSI-MCO (French Medical Information System Program in Medicine, Surgery, and Obstetrics). We aimed to compare the frequency of risk factors in a population with congenital heart defects and a reference population...

Diastolic wall strain (DWS), an echocardiographic index based on linear elasticity theory, has been identified as a predictor of heart failure (HF) in patients with sinus rhythm. However, its effectiveness in atrial fibrillation (AF) patients remains uncertain. This study aims to assess DWS as a predictor of HF in AF patients with preserved ejection fraction. We analysed a prospective database of AF patients undergoing transthoracic echocardiography. AF patients with reduced left ventricular ejection fraction (< 50%), posterior wall motion abnormality, hypertrophic cardiomyopathy, valvular heart disease, pericardial disease, congenital heart disease, or history of pacemaker/implantable cardioverter-defibrillator implantation or cardiac surgery were excluded...

Dysphagia lusoria is a rare pediatric condition caused by extrinsic compression of the esophagus by an abnormal subclavian artery. The most common congenital abnormality in aortic arch development is an aberrant right subclavian artery. The retroesophageal right subclavian artery is typically symptomatic in 10-33% of cases. The patient, an 8-month-old girl with a history of early dysphagia and stridor, was diagnosed with an abnormal right subclavian artery. She was admitted to the pneumology service multiple times due to stridor, vomiting, and failure to thrive...

Radial dysplasia (RD) is a congenital upper limb birth defect that presents with changes to the upper limb anatomy, including a shortened or absent radius, bowed ulna, thumb malformations, a radially deviated hand and a range of muscle and tendon malformations, including absent or abnormally shaped muscle bundles. Current treatments to address wrist instability caused by a shortened or absent radius frequently require an initial soft tissue distraction intervention followed by a wrist stabilisation procedure...

&lt;b&gt;&lt;br&gt;Introduction:&lt;/b&gt; Congenital inner ear malformations resulting from embryogenesis may be visualized in radiological scans. Many attempts have been made to describe and classify the defects of the inner ear based on anatomical and radiological findings.&lt;/br&gt; &lt;b&gt;&lt;br&gt;Aim:&lt;/b&gt; The aim was to propose and discuss computed tomography multi-planar and 3D image assessment protocols for detailed analysis of inner ear malformations in patients undergoing cochlear implantation counseling...

PURPOSE: To carry out a comprehensive critical review of the peer-reviewed literature on the refractive changes associated with oculoplastic surgeries. METHODS: This review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses Extension for Scoping Reviews (PRISMA-ScR) statement recommendations. Following specific inclusion and exclusion criteria, 20 articles were selected for the current scoping review. Each of them was analyzed carefully and their risk of bias was assessed with the Quality Assessment Tool for case Series Studies from the National Heart, Lung and Blood Institute...

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PURPOSE: This systematic review focused on reasons for conversions in neonates undergoing thoracoscopic congenital diaphragmatic hernia (CDH) repair. METHODS: Systematic search of Medline/Pubmed and Embase was performed for English, Spanish and Portuguese reports, according to PRISMA guidelines. RESULTS: Of the 153 articles identified (2003-2023), 28 met the inclusion criteria and offered 698 neonates for analysis. Mean birth weight and gestational age were 3109 g and 38...

Internal hernia is an uncommon cause of mechanical small bowel obstruction. This case report details a 66-year-old Chinese male with no prior abdominal surgeries who presented with colicky abdominal pain, abdominal distension, and vomiting. Initial investigations were unyielding, but escalating symptoms prompted a diagnostic laparoscopy. Laparotomy then revealed a closed-loop obstruction through a lateral type pericecal hernia, with a segment of ischemic jejunum. Adhesion bands in the right iliac fossa and a congenital hernia orifice in the mesentery were identified and addressed...

INTRODUCTION: The advancement of surgical techniques and perioperative management for congenital heart disease (CHD) has increased life expectancy. The surgical creation of the Fontan circulation maintains pulmonary blood flow without relying on an effective pump from the abnormal heart, relying on peripheral vascular resistance to maintain effective flow through the lungs. Unfortunately, this delicate mechanism is compromised when scoliosis restricts ventilation, leading to Fontan failure and a poor prognosis for life...

Neonates with congenital diaphragmatic hernia encounter a number of surgical and medical morbidities that persist into adulthood. As mortality improves for this population, these survivors warrant specialized follow-up for their unique disease-specific morbidities. Multidisciplinary congenital diaphragmatic hernia clinics are best positioned to address these complex long-term morbidities, provide long-term research outcomes, and help inform standardization of best practices in this cohort of patients. This review outlines long-term morbidities experienced by congenital diaphragmatic hernia survivors that can be addressed in a comprehensive follow-up clinic...

Dual left anterior descending artery (LAD) is a rare congenital coronary artery anomaly with a prevalence of approximately 1% in the general population. To date, 10 types of dual LAD artery anomalies have been reported. Among these, type 4 is one of the rarest. Knowledge and recognition of the dual LAD artery are important for correct diagnosis and planning of coronary bypass surgery and percutaneous coronary intervention. We report a case of a 59-year-old male with type 4 dual LAD artery who presented with dyspepsia and sweating for several months and had approximately 50%-70% stenosis in a major diagonal branch off the short LAD artery...

BACKGROUND: Pulmonary arteriovenous malformations (PAVMs) universally develop in patients with single ventricle congenital heart disease (CHD). Single ventricle PAVMs have been recognized for over 50 years, yet they are poorly understood, and we lack any medical therapies. To improve our understanding of single ventricle PAVM initiation and progression, we developed a surgical rat model of Glenn circulation and characterized PAVM physiology over multiple time points. METHODS: Using adult rats, we performed a left thoracotomy and end-to-end anastomosis of the left superior vena cava to the left pulmonary artery (unilateral Glenn), or sham surgical control...

BACKGROUND: Hysterectomy places a considerable physical and mental burden on young female patients with congenital cervical and complete vaginal atresia. Thus, it is necessary to develop a method to detach the obstruction and simultaneously preserve the vagina and uterus in these patients. This study sought to evaluate the efficacy and safety of laparoscopic vaginoplasty using peritoneal flaps and cervicoplasty in patients with congenital cervical and complete vaginal atresia. METHODS: Between April 2013 and June 2022, nine patients with congenital cervical and complete vaginal atresia at Henan Provincial People's Hospital were enrolled in this prospective study...

INTRODUCTION: Thyroid hemiagenesis is a rare congenital anomaly characterized by the absence of one thyroid lobe and the isthmus. This case report presents a 4-year-old girl with a history of prematurity. Incidentally, during a routine ultrasound evaluation of the neck, thyroid hemiagenesis was detected along with the presence of normal lymph nodes. The right thyroid lobe was absent, while the left thyroid lobe was preserved. No previous neck or thyroid surgeries were reported. DISCUSSION: This provides an overview of thyroid hemiagenesis, including its prevalence, predominant involvement of the left lobe, possible genetic and environmental factors, and associations with thyroid and extrathyroidal pathologies...

In this study, unipolar myomectomy was used to address limited neck movement and tight muscles in pediatric, adolescent, and adult patients. A retrospective chart review was performed for patients from January 2006 to February 2023, who were diagnosed with congenital muscular torticollis and underwent a unipolar myomectomy. Outcome evaluation, adapted from the Cheng and Tang system - cervicomandibular angle (CMA), facial asymmetry, cranial asymmetry, tilting limitation (TL), rotation limitation (RL), subjective assessment, and residual contracture - included various parameters scored from 0 to 3 points and categorized as poor, fair, good, or excellent...

INTRODUCTION AND IMPORTANCE: Intestinal duplication is an uncommon congenital malformation affecting the alimentary tract. This article presents a case of enteric duplication cyst (EDC) in an adult, accompanied by a review of the available literature. CASE PRESENTATION: A 34-year-old woman with polymyositis underwent a routine CT scan as part of her medical assessment revealing an 8 cm mass near the caecum and terminal ileum. Diagnostic procedures confirmed a cystic spherical mass...

Mayer-Rokitansky-Kuster-Hauser syndrome also known as mullerian agenesis is a rare congenital condition in which there is absence of uterus along with upper vagina. Patient usually presents with primary amenorrhea with or without cyclical lower abdominal pain but have normal secondary sexual characters. Modified McIndoe Vaginoplasty with amnion graft is the commonest surgery performed worldwide. A 23 year old girl with normal secondary sexual characters presented with primary amenorrhea with cyclical lower abdominal pain; on examination blind vagina was present...

INTRODUCTION: Popliteal pterygium syndrome is a rare congenital disorder characterized by orofacial, cutaneous, musculoskeletal, and genital anomalies. The popliteal pterygium (PP) affects ambulation due to severe knee flexion contracture and equinovarus deformities. Surgical treatment aims to correct these deformities while preserving limb sensibility. However, due to its rarity, surgical guidelines are lacking. MATERIALS AND METHODS: A meta-analysis according to PRISMA guidelines was conducted to summarize and compare surgical methods for PP...

Sinus venosus atrial septal defects (SVASDs), concurrent with partial anomalous pulmonary venous connections (PAPVCs), are a rare congenital heart disease in dogs. Surgical correction is essential when clinical signs or significant hemodynamic changes are present. We aimed to report on the successful surgical correction of an SVASD with PAPVCs, using a computed tomography (CT)-based customized 3D cardiac model. A 10-month-old male poodle was referred for corrective surgery for an ASD. Echocardiography confirmed a hemodynamically significant left-to-right shunting flow through an interatrial septal defect and severe right-sided heart volume overload...