Hypoplast

Despite significant advancements in the care of patients with hypoplastic left heart syndrome (HLHS) morbidity and mortality remain high. Postnatal right ventricular dysfunction and tricuspid regurgitation (TR) are associated with worse outcomes in HLHS. We aim to determine if right ventricle functional parameters and TR on fetal echocardiogram are associated with postnatal outcomes in HLHS patients. Retrospective review was performed on all fetuses with HLHS from 2014 to 2022 at our institution. Initial and follow up fetal echocardiogram measurements of right ventricular myocardial performance index (MPI), fractional area change (FAC) and global longitudinal strain (GLS) were retrospectively measured...

UNLABELLED: We present three cases of hypoplastic left heart syndrome (HLHS) complicated by congenital esophageal atresia and trachea-esophageal fistula (EA/TEF). The standard treatment for HLHS involves a staged surgical approach, eventually reaching Fontan completion. There is no report of patients with both HLHS and EA/TEF, and no established treatment strategy exists for such cases. Given the significant risk of simultaneously operating on HLHS and EA/TEF, we elected to pursue staged repair for each condition separately...

BACKGROUND: The persistent primitive trigeminal artery (PPTA) is a persistent embryological carotid-basilar connection. Endovascular thrombectomy (EVT) for hypoplastic PPTA occlusion is a challenge. This case report aims to describe the successful recanalization of simultaneous occlusions in both the PPTA and basilar artery (BA) using the Solitaire FR (RECO SR)/Stent and Intermediate Catheter Assisting (SWIM) technique in a patient with acute cardiogenic cerebral embolism. To the best of our knowledge, this is the first report of such a case...

BACKGROUND: A Blauth IIIB hypoplastic thumb is a significant functional and cosmetic problem for the developing hand in children. The gold standard in treatment is amputation and index pollicization. Despite the good functional results, some parents do not consent to the operation, mainly for cosmetic reasons. OBJECTIVES: The aim is to present a detailed description and features of the technique used in our department for stabilization of a hypoplastic thumb type Blauth IIIB with a non-vascularized proximal interphalangeal joint from the toe...

INTRODUCTION: Thumb hypoplasia is a congenital birth defect in which a child is born with an underdeveloped or missing thumb. It is a rare condition affecting approximately 1 in 100,000 live births and occurs equally in both males and females. Pollicization is a surgical procedure used to treat severe thumb hypoplasia by transferring another finger to the thumb position. CASE PRESENTATION: Twin girls aged two years and eight months, born to a 42-year-old para III mother, presented with bilateral thumb hypoplasia...

Hypoplastic maxilla is a common skeletal anomaly that compromises function and esthetics. Beyond just a narrow-appearing smile, this abnormality presents significant restorative challenges in adult patients as it is often associated with crowding, compromised axial inclination of the teeth, lack of alveolar bone support, root proximity, and occlusal trauma.1 Recent research also confirms association of maxillary deficiency with nasal stenosis and a predisposition to compromised nasal airflow and pharyngeal collapse during sleep...

OBJECTIVES: The primary objectives were to examine utilization of the Hybrid vs. the Norwood procedure for patients with hypoplastic left heart syndrome (HLHS) or variants and the impact on hospital mortality. The Hybrid procedure was first used at our institution in 2004. METHODS: Review of all subjects undergoing the Norwood or Hybrid procedure between 1/1/1984 and 12/31/2022. The study period was divided into 8 eras: era 1, 1984 to 1988; era 2, 1989 to 1993; era 3, 1994 to 1998; era 4, 1999 to 2003; era 5, 2004 to 2008; era 6, 2009 to 2014; era 7, 2015 to 2018; and era 8, 2019 to 2022...

BACKGROUND: Utilization of Fontan fenestration varies considerably by center. OBJECTIVES: Using a multicenter Pediatric Heart Network dataset linking surgical and preoperative hemodynamic variables, the authors evaluated factors associated with use of Fontan fenestration and the impact of fenestration on post-Fontan length of stay (LOS). METHODS: Patients 2 to 6 years old at Fontan surgery from 2010 to 2020 with catheterization<1 year prior were included...

Johanson-Blizzard syndrome (JBS) is a rare hereditary autosomal recessive disorder caused by a mutation in the ubiquitin protein ligase E3 component n-recognin 1 (UBR1) gene. This syndrome is characterized by the following typical clinical features: hypoplasia or aplasia of the alae nasi, congenital scalp defects, sensorineural hearing loss, hypothyroidism, growth retardation, psychomotor retardation, imperforate anus, genitourinary anomalies, and atypical hair patterns. Here, we describe a case of a 12-year-old girl with JBS of consanguineous parents...

Nail-patella syndrome, also known as Fong disease, is an uncommon autosomal dominant disorder characterized by a distinctive set of features, including fingernail abnormalities, hypoplastic patellae, radial head dislocation, and iliac horns. This condition often leads to patellar subluxation or dislocation, resulting in knee instability and pain. While existing literature predominantly focuses on the clinical and radiological aspects of nail-patella syndrome-related knee manifestations, only a limited number of articles delve into a meticulous approach to the condition with a comprehensive strategy for diagnosis...

Encephaloceles and severe cardiac malformations are rare presentations in a newborn. The mechanism of these congenital abnormalities is relatively unknown, but it is hypothesized to be related to genetic, environmental, and maternal risk factors. This case report describes a newborn with an occipital encephalocele associated with severe right ventricular hypoplasia secondary to tricuspid and pulmonary atresia. The patient's maternal risk factors included obesity, type 2 diabetes mellitus, and everyday tobacco use during pregnancy...

Reversed flow in the basilar artery can be acquired or congenital. Acquired reversed flow in the basilar artery can result from acute thrombosis of the basilar artery or retrograde vertebral artery flow. Congenital continuous retrograde basilar artery flow has not been described. We report a 2-day-old male presenting with hypocalcemic seizures which led us to obtain a Duplex echoencephalogram. An echocardiogram was subsequently ordered. In the coronal plane through the anterior fontanelle, retrograde flow was seen in the basilar artery and the right vertebral artery...

In 2022, two novel classification systems for myelodysplastic syndromes/neoplasms (MDS) have been proposed: the International Consensus Classification (ICC) and the 2022 World Health Organization (WHO-2022) classification. These two contemporary systems exhibit numerous shared features but also diverge significantly in terminology and the definition of new entities. Thus, we retrospectively validated the ICC and WHO-2022 classification and found that both systems promoted efficient segregation of this heterogeneous disease...

PURPOSE: To evaluate the functional results after opponensplasty using an abductor pollicis brevis rerouting technique in type II and IIIA hypoplastic thumbs. METHODS: Eleven hypoplastic thumbs in nine children with type II and IIIA hypoplastic thumbs were treated with abductor pollicis brevis rerouting. The mean follow-up period was 70 months (range, 12-172 months). We assessed preoperative to postoperative changes in the angles of the first and second metacarpal axes and the longitudinal axis of the first metacarpal and proximal thumb phalanx as well as grip and pinch strengths...

OBJECTIVES: To assess the early outcomes and risk factors of paediatric patients requiring extracorporeal membrane oxygenation after cardiac surgery (post cardiotomy). METHODS: Retrospective binational cohort study from the Australia and New Zealand Congenital Outcomes Registry for Surgery database. All patients younger than 18 years of age who underwent a paediatric cardiac surgical procedure from 1 January 2013 to 31 December 2021 and required post-cardiotomy extracorporeal membrane oxygenation in the same hospital admission were included in the study...

PURPOSE: Fibular hemimelia has denoted a spectrum of postaxial longitudinal deficiency with fibular aplasia/hypoplasia; the term "terminal hemimelia" is reserved for patients with postaxial longitudinal deficiency having a normal fibula. We aimed to delineate the characteristics of terminal hemimelia. METHODS: In total, 30 patients with postaxial longitudinal deficiency who had a normal or hypoplastic fibula and visited our institution between 1992 and 2022 were reviewed...

BACKGROUND: In liver transplant patients with hypoplastic portal vein (PV), when the narrowed segment is extended too deep into the dorsal side of the pancreas, it is difficult and dangerous to reconstruct the interposition graft from the upper part of the pancreas. Herein, we present a case of PV reconstruction with the autologous mesosystemic shunt vessel from the caudal side of the pancreas in a situation where the narrowed PV was deep, and we discuss the technical details. CASE PRESENTATION: A 25-year-old woman presented with cholestatic liver cirrhosis due to biliary atresia after Kasai procedure...

Renal endometriosis is a rare disorder of cases of urinary tract endometriosis. A 42-year-old woman presented at our outpatient department with an incidental painless mass on her left hypoplastic kidney revealed on an abdominal ultrasound. Abdominal and pelvic examinations revealed no abnormal findings. A computed tomography (CT) scan revealed an anterolateral slightly enhanced left renal mass that measured 1.2 cm in diameter. Furthermore, CT did not reveal any evidence of abdominal or thoracic metastasis. There are a few case reports in the literature of tumors in specimens from patients who underwent nephrectomy for hypoplastic kidneys, but discriminating between benign and malignant masses is difficult unless a nephrectomy is performed...

PURPOSE: Hypoplastic pedicles of the thoracolumbar spine (<5 mm diameter) are often found in syndromic deformities of the spine and pose a challenge in pedicle screw instrumentation. 3D-printed patient-specific guides might help overcome anatomical difficulties when instrumenting pedicles with screws, thereby reducing the necessity for less effective fixation methods such as hooks or sublaminar wires. In this study, the surgical feasibility and clinical outcome of patients with hypoplastic pedicles following pedicle screw instrumentation with 3D-printed patient-specific guides were assessed...

A variety of surgical approaches exist to treat aortic coarctation in neonates and infants. Our institutional approach is designed to match the surgical approach to the individual anatomy of the patient. The objective of this study is to evaluate operative characteristics and outcomes of all neonates and infants who underwent surgical repair of coarctation of the aorta or hypoplastic aortic arch at University of Florida from 2006 to 2021, inclusive, either in isolation or with concomitant repair of atrial septal defect (ASD) and/or ventricular septal defect (VSD)...