Transcatheter Fontan completion: Creation of an extracardiac Fontan.

Patients with functionally univentricular hearts are usually palliated surgically. There have been several reports of successful attempts to complete the Fontan procedure without surgery. The pathways created at the time of the preconditioning were largely reminiscent of the lateral tunnel Fontan. However, this approach is still confidentially limited to a small number of centers. In 2013, we designed a circuit that mimics the actual surgical technique of extracardiac total cavopulmonary connection to allow for transcatheter completion in an animal study. A polytetrafluoroethylene conduit was connected between the pulmonary artery and the inferior vena cava (IVC). The superior anastomosis was occluded to avoid flow between IVC and superior vena cava (SVC). The conduit was connected to the right atrium (RA) and a large fenestration was created to allow free flow from the IVC to the RA. Extrapolating our approach, a center reported the successful transcatheter completion of an extracardiac Fontan in a 6-year-old child. However, this technique is not directly transposable to our population of patients who require preconditioning in infancy. We report here an innovative extension of this technique that may allow preparing patients in infancy, ideally at the time of the Glenn in the future, to receive an extracardiac Fontan at 2 years/11 kg without additional surgery.

Combined Percutaneous Transhepatic Lymphatic Embolization and Peroral Duodenal Mucosal Radiofrequency Ablation to Manage Protein-Losing Enteropathy.

Percutaneous transhepatic lymphatic embolization (PTLE) and peroral esophagogastroduodenoscopy (EGD) duodenal mucosal radiofrequency (RF) ablation were performed to manage protein-losing enteropathy (PLE) in patients with congenital heart disease. Five procedures were performed in 4 patients (3 men and 1 woman; median age, 49 years; range, 31-71 years). Transhepatic lymphangiography demonstrated abnormal periduodenal lymphatic channels. After methylene blue injection through transhepatic access, subsequent EGD evaluation showed methylene blue extravasation at various sites in the duodenal mucosa. Endoscopic RF ablation of the leakage sites followed by PTLE using 3:1 ethiodized oil-to-n-butyl cyanoacrylate glue ratio resulted in improved symptoms and serum albumin levels (before procedure, 2.6 g/dL [SD ± 0.2]; after procedure, 3.5 g/dL [SD ± 0.4]; P = .004) over a median follow-up of 16 months (range, 5-20 months). Transhepatic lymphangiography and methylene blue injection with EGD evaluation of the duodenal mucosa can help diagnose PLE. Combined PTLE and EGD-RF ablation is an option to treat patients with PLE.

Novel truncating mutations in CTNND1 cause a dominant craniofacial and cardiac syndrome.

CTNND1 encodes the p120-catenin (p120) protein, which has a wide range of functions, including the maintenance of cell-cell junctions, regulation of the epithelial-mesenchymal transition and transcriptional signalling. Due to advances in next-generation sequencing, CTNND1 has been implicated in human diseases including cleft palate and blepharocheilodontic (BCD) syndrome albeit only recently. In this study, we identify eight novel protein-truncating variants, six de novo, in 13 participants from nine families presenting with craniofacial dysmorphisms including cleft palate and hypodontia, as well as congenital cardiac anomalies, limb dysmorphologies and neurodevelopmental disorders. Using conditional deletions in mice as well as CRISPR/Cas9 approaches to target CTNND1 in Xenopus, we identified a subset of phenotypes that can be linked to p120-catenin in epithelial integrity and turnover, and additional phenotypes that suggest mesenchymal roles of CTNND1. We propose that CTNND1 variants have a wider developmental role than previously described and that variations in this gene underlie not only cleft palate and BCD but may be expanded to a broader velocardiofacial-like syndrome.

[Valve Replacement for Isolated Pulmonary Valve Infective Endocarditis without Congenital Heart Defects:Report of a Case].

We report a rare case of isolated pulmonary valve endocarditis in a 60-year-old man without congenital heart defects. He had a persistent fever and back neck pain after pulling out his teeth. Echo cardiography revealed a mobile vegetation (measuring 53 mm in size) adhering to the pulmonary valve and blood cultures showed the presence of Staphylococcus aureus( S. aureus). Because of mobile vegetation, pulmonic embolism and presence of S. aureus, surgical treatment was selected. During surgical procedure, we found that the vegetation had destroyed markedly pulmonary valve leaflets. After excising pulmonary valve leaflets, we implanted a bioprosthetic valve and enlarged the pulmonary artery with autologous pericardium. A year after surgery, the patient is stable with no sign of infection.

Stenting and overdilating small Gore-Tex vascular grafts in complex congenital heart disease.

BACKGROUND: Gore-Tex® grafts are integral in the management of congenital heart disease. Issues of graft stenosis or somatic outgrowth may precipitate high-risk early surgery, and catheter intervention is a relatively under-reported management option. OBJECTIVES: To assess efficacy, safety, and outcomes of stenting and overdilating small Gore-Tex® vascular grafts with the aim of optimizing surgical timing. METHODS: Retrospective analysis of single-center, 13-year experience of 93 graft stenting interventions or reintervention in 80 patients, with the aim of relieving stenosis ± overdilation, to depose surgical graft revision or optimize surgical timing and candidacy. RESULTS: Median preintervention graft diameter was 52% (IQR 43-63) of nominal size, postintervention this increased to median 102% (IQR 96-120) [P < 0.001]. Overdilation was achieved in 54%; of these, the median final internal lumen was 126% (IQR 113-132) [P < 0.001]. Mean oxygen saturations increased from 69% to 82% [P < 0.001]. Adverse event rate was 14.9%; this includes mortality (3.2%) and atrioventricular conduction block (8.5%). Univariate analysis and logistic regression showed a significant relationship between adverse events and young age [P < 0.01], low weight [P < 0.01], univentricular physiology [P < 0.001], use of femoral venous access alone [P = 0.03], and intervention on a right ventricle to pulmonary artery conduit [P = 0.03]. Within respective indication groups, 74% were bridged to elective cavopulmonary shunt, 95% were bridged to biventricular surgery, and 78% of long-term palliative care patients continue under follow-up. CONCLUSIONS: Gore-Tex® vascular grafts can be stented effectively and expanded beyond nominal diameters by around +26%. This improves oxygen saturations, providing excellent palliation and optimized surgical timing. Adverse events are most frequent in precavopulmonary shunt patients. CONDENSED ABSTRACT: Gore-Tex® grafts are widely used in the palliation of cyanotic congenital heart disease. Grafts may become stenosed and do not allow for somatic growth. Over a 13 year period, graft stenting was performed in 80 patients (93 technically successful interventions, 4 unsuccessful.) Median internal lumen change was +50% (of nominal graft size), mean oxygen saturation change +13%. Over-dilation was performed in more than half of the cohort, with a median gain of +26% on nominal graft size. In precavopulmonary shunt patients, there was a moderate incidence of serious complications. Clinically useful deferral of surgery was achieved for the majority.

Covidien micro vascular plug in congenital heart diseases and vascular anomalies: A new kid on the block for premature babies and older patients.

OBJECTIVES: To evaluate the safety, feasibility, and efficacy of the MVP™ microvascular plug(Covidien) for closure of vascular anomalies in patients with congenital heart diseases (CHD). BACKGROUND: The MVP™ is a novel device with PTFE integrated. The device has been recently introduced in the radiological field and reported exclusively for neurological anomalies. METHODS: All CHD patients receiving the device from April 2015 until July 2015 were included in the study and followed up clinically as well as by transthoracic echocardiography. Standard safety and follow-up such as vascular complications, embolization rate, and residual shunting were assessed. RESULTS: Twelve patients with a median age of 2.6-years (0.03-12.6 years) and a mean weight of 13 kg (2.8-34.2 kg) were included. Devices were delivered from the femoral artery in 10 and from the femoral vein in 2 patients. Devices were used for closure of patent ductus arteriosus (PDA) closure (n = 5), aorto-pulmonary collaterals or Blalock-Taussig shunt (n = 5), veno-venous fistula (n = 1), and coronary fistula (n = 1). One device was retrieved before release due to inappropriate size estimation (PDA spasm). The PDA was successfully closed using a PDA device (Lifetech, Medtronic). Immediate angiographic evaluation showed minimal or no shunt in 90% (10/11 patients) and 100% occlusion rate after a mean follow-up of 3.2-months. There was no device embolization, hemolysis, or any other complication following closure. CONCLUSIONS: The delivery of new MVP™ micro vascular plug system (Covidien) is safe and effective in patients with CHD for closure of a variety of vascular abnormalities. The low profile of the device and the sheathless nature of the procedure make it particularly interesting for PDA closure in premature babies. © 2016 Wiley Periodicals, Inc.

Correction of cortriatriatum sinister with classical Raghib's complex using an extracardiac conduit.

We present a 32-year-old patient with cortriatriatum sinister with Raghib's complex (a left persistent superior vena cava draining into the left atrium with an absent coronary sinus and an atrial septal defect [ASD]) who underwent successful surgical correction with excision of the cortriatriatum, closure of the ASD, and establishing the drainage of the persistent left superior vena cava to the right atrium via interposition of an extracardiac 13-mm ringed polytetrafluoroethylene conduit.

Expanded polytetrafluoroethylene conduits with curved and handsewn bileaflet designs for right ventricular outflow tract reconstruction.

OBJECTIVE: This study reviewed the application of curved and bileaflet designs to pulmonary expanded polytetrafluoroethylene conduits with diameters of 10 to 16 mm and characterized this conduit on in vitro experiment, including particle image velocimetry. METHODS: All patients who received this conduit between 2010 and 2022 were evaluated. Three 16-mm conduits were tested in a circulatory simulator at different cardiac outputs (1.5-3.6 L/minute) and bending angles (130°-150°). RESULTS: Fifty consecutive patients were included. The median operative body weight was 8.4 kg (range, 2.6-12 kg); 10-, 12-, 14-, and 16-mm conduits were used in 1, 4, 6, and 39 patients, respectively. In 34 patients, the conduit was implanted in a heterotopic position. The overall survival rate was 89% at 8 years with 3 nonvalve-related deaths. There were 10 conduit replacements; 5 16-mm conduits (after 8 years) and 1 12-mm conduit (after 6 years) due to conduit stenosis, and the remaining 4 for reasons other than conduit failure. Freedom from conduit replacement was 89% and 82% at 5 and 8 years, respectively. Linear mixed-effects models with echocardiographic data implied that 16-mm conduits were durable with a peak velocity <3.5 m/second and without moderate/severe regurgitation until the patient's weight reached 25 kg. In experiments, peak transvalvular pressure gradients were 11.5 to 25.5 mm Hg, regurgitant fractions were 8.0% to 14.4%, and peak Reynolds shear stress in midsystolic phase was 29 to 318 Pa. CONCLUSIONS: Our conduits with curved and bileaflet designs have acceptable clinical durability and proven hydrodynamic profiles, which eliminate valve regurgitation and serve as a reliable bridge to subsequent conduit replacement.

Ellis van creveld syndrome: Cardiac anomalies and anesthetic implications.

Ellis-Van Creveld syndrome (EVC), also known as mesoectodermal dysplasia, is a rare autosomal recessive disorder with a tetrad of clinical features, comprising dwarfism, polydactyly, ectodermal dysplasia with sparse hair, hypoplastic nails and enamel, hypodontia and conical teeth and congenital heart disease (CHD). We report an 18-year-old girl with short stature and polydactyly, who got admitted to our hospital with shortness of breath on exertion for the last 2 years. On echocardiography, a partial atrioventricular canal (AV canal) defect was diagnosed, which was repaired surgically. The patient had an uneventful perioperative period.

Ectopia Cordis Sternal Cleft Repair With Mandibular Distractors: A Follow-Up Case Report.

A neonate with thoracic ectopia cordis presented following an uncomplicated delivery. A mandibular distractor was placed to bridge the sternal cleft and retracted (reverse distractor activation) over 24 days to facilitate sternal closure. Follow-up at five years postoperatively demonstrated a well-healed sternum. This novel approach to ectopia cordis repair facilitates slow, steady physiologic accommodation of the heart without hemodynamic instability or long-term complications.

Transcatheter Thoracic Duct Decompression for Multicompartment Lymphatic Failure After Fontan Palliation.

BACKGROUND: Lymphatic embolization therapy has proven effective for Fontan failure from plastic bronchitis or protein-losing enteropathy but not when multiple lymphatic compartments are involved; furthermore, embolization does not alter the underlying pathophysiology of lymphatic dysfunction. A technique for transcatheter thoracic duct decompression (TDD), rerouting the thoracic duct to the pulmonary venous atrium to treat multicompartment lymphatic failure is described and early outcomes presented. METHODS: Initially covered stents were used to channel the innominate vein flow inside of the cavopulmonary pathway into the pulmonary venous atrium. A modified approach was developed where covered stents redirected innominate vein directly to the left atrium via an extravascular course. Baseline and follow-up data on all patients undergoing TDD were reviewed. RESULTS: Twelve patients underwent TDD between March 2018 and February 2021 at a median age of 12 (range: 2-22) years. Lymphatic failure occurred in median of 3 compartments per patient (protein-losing enteropathy, ascites, pleural effusions, plastic bronchitis); 10 patients had lymphatic embolizations before TDD. TDD method was intra-Fontan tunnel in 4, direct approach in 7, and other in 1. There were no major procedural complications; 6 patients underwent subsequent procedures, most commonly to treat endoleaks. Lymphatic failure resolved in 6 patients, improved in 2, and was unchanged in 4 at 6 (range: 1-20) months follow-up. One patient died after TDD from Fontan failure. CONCLUSIONS: TDD is a promising new treatment for the failing Fontan physiology from multicompartment lymphatic failure. Additional work is needed to refine the technique and define optimal candidates.

Inspiratory muscle dysfunction and restrictive lung function impairment in congenital heart disease: Association with immune inflammatory response and exercise intolerance.

BACKGROUND: In adult patients with congenital heart disease (ACHD), both underlying disease and lung restriction contribute to exercise intolerance. In ACHD the yet incompletely understood mechanism underlying restricted ventilation may be inspiratory muscle weakness. Therefore, this study comprehensively evaluated inspiratory muscle function in ACHD and associations with systemic inflammation and the clinical severity of exercise intolerance. METHODS: 30 ACHD patients (21 men, 35 ± 12 years) and 30 healthy controls matched for age, gender and body mass index underwent spirometry, measurement of mouth occlusion pressures, and diaphragm ultrasound. Six-minute walking distance (6MWD) and New York Heart Association functional class were used to quantify exercise intolerance. Interleukin-6 (IL-6) and tumor necrosis factor-alpha (TNF-α) levels were measured using enzyme-linked immunosorbent assays. RESULTS: ACHD patients showed lower forced vital capacity (FVC), and maximum inspiratory (PImax) and expiratory (PEmax) pressures compared with controls (all p < 0.05). On ultrasound, ACHD patients showed a lower diaphragm thickening ratio (2.3 ± 0.5 vs. 2.8 ± 0.9, p < 0.01) and lower diaphragm excursion velocity during a voluntary sniff maneuver (5.7 ± 2.2 vs. 7.6 ± 2.0 cm/s, p < 0.01). Respiratory parameters, such as FVC (r = 0.53; p < 0.01) and PImax (r = 0.43; p = 0.02), correlated with 6MWD. Furthermore, amino terminal pro B-type natriuretic peptide levels were inversely correlated with FVC (r = -0.54; p < 0.01). Circulating pro-inflammatory cytokines were markedly increased, and IL-6 was correlated with 6MWD, dyspnea, and biomarkers of heart, lung and inspiratory muscle function (all p < 0.05). CONCLUSIONS: Our findings show that diaphragm dysfunction is present in ACHD and relates to restrictive ventilation disorder and exercise intolerance, possibly mediated by increased IL-6 levels.

An Unusual Complication of the "Supported" Ross Procedure.

We present a unique surgical complication in a 19-year-old patient who underwent a "supported" Ross procedure for congenital aortic stenosis. In the present case, herniation of pulmonary autograft material through coronary fenestrations in the Dacron supporting material can be appreciated. This case suggests a possible need to modify surgical technique to ensure that all autograft tissue remains contained within the Dacron bolster.

Pulmonary artery growth following arterial duct stenting in congenital heart disease with duct-dependent pulmonary circulation.

OBJECTIVES: The aim of this study was to assess the pulmonary artery (PA) growth following arterial duct (AD) stenting in neonates with congenital heart disease (CHD) with duct-dependent pulmonary circulation. BACKGROUND: AD stenting is increasingly deemed as a reliable alternative to surgical shunt in CHD with duct-dependent pulmonary circulation. Allowing the stent to conform to the PA anatomy might avoid any unbalance of lung perfusion and promote a uniform growth of the main PAs. METHODS: Of the 47 neonates and infants submitted to AD stenting as palliation of CHD with duct-dependent pulmonary circulation at our Institution, 15 underwent angiographic evaluation of the PA development before surgical repair due to progressive decrease of the systemic oxygen saturation. The Nakata and McGoon indexes as well as the left-to-right PA diameter ratio were considered as being indicative of global and balanced growth. RESULTS: Ductal stabilization was achieved with coronary stents dilated to 3.3 + or - 0.4 mm (range 2.5-4). The control angiography was performed 7 + or - 3 months (range 3-13) after the stenting procedure. At that time, the Nakata and McGoon indexes had significantly increased from 132 + or - 67 to 287 + or - 94 mm/m(2) and from 1.6 + or - 0.3 to 2.1 + or - 0.2 mm/m(2), respectively (P < 0.0001 for both comparisons), due to uniform vascular growth (left PA z-score from -0.7 + or - 1.6 to 1.4 + or - 1.2; right PA from -0.8 + or - 1.4 to 1.4 + or - 1.2, P < 0.0001 for both comparisons; final left-to-right PA ratio 0.9 + or - 0.1). CONCLUSIONS: Percutaneous AD stabilization with highly flexible coronary stents is effective in promoting a significant and balanced growth of the PAs in CHD with duct-dependent pulmonary circulation.

Ellis van Creveld syndome.

Ellis-van Creveld syndrome (EVC) or chondroectodermal dysplasia is a rare autosomal recessive disorder. It is a tetrad of chondrodysplasia, ectodermal dysplasia, polydactyly, and congenital heart disease. In several case reports, dysplasia involving other organs has also been identified. The exact prevalence is unknown, but the syndrome seems more common among the Amish community. Many Indian cases have also been reported. This report describes a classical case of EVC syndrome in a 22 year old woman of Indian origin born of a consanginous marriage. The patient had chondrodysplasia of tubular bones resulting in disproportionate dwarfism, postaxial polydactyly, severely dystrophic nails, partially absent teeth, pectus excavatum with narrow chest, knock knees and AV canal defect.

Longitudinal growth in patients with single ventricle cardiac disease receiving tube-assisted feeds.

OBJECTIVE: Children with single ventricle cardiac disease (SVCD) have poor growth in early life. Tube-assisted feeding (TF) is used to improve weight gain, but its impact on long-term growth remains unknown. We sought to compare the longitudinal growth of SVCD patients receiving TF after initial cardiac surgery with those fed entirely by mouth. DESIGN: We conducted a retrospective cohort study of SVCD patients who underwent initial surgical palliation between 1999 and 2009. We defined TF as the use of nasogastric, gastrostomy, or jejunostomy TF. We compared maximal attained growth z-scores for each year of life between TF and non-TF patients. A secondary analysis compared surgical and clinical factors between groups. RESULTS: A total of 134 patients were included; 64% were male and 68% underwent the Norwood operation. One third of patients (44) received TF. Adjusting for age, TF patients had an average of 0.56 lower weight-for-age z-score (WAZ) than non-TF patients (P = 0.007) through the age of 6 years. Longitudinal height was not affected by TF status (P = 0.15). In a subanalysis of Norwood patients, TF patients had lower WAZ at initial hospital discharge despite longer LOS. TF patients had diminished WAZ after adjusting for complications, interstage hospitalizations, and timing of subsequent operations. CONCLUSIONS: In this single-center study, patients with SVCD requiring TF at discharge from initial surgical palliation had diminished WAZ at discharge and on long-term follow-up, despite controlling for other identifiable risk factors. Further investigation is needed to understand the mechanisms underlying this phenomenon and to risk stratify infants who go home on TF.

Modification of expanded polytetrafluoroethylene valved conduit using the thin-type leaflets.

OBJECTIVES: The expanded polytetrafluoroethylene conduit with fan-shaped leaflets and bulging sinuses for right ventricular outflow tract reconstruction was modified with a newly developed thin-type expanded polytetrafluoroethylene leaflet. The purpose of this study was to evaluate the clinical outcomes and hemodynamic performance of the modified conduit. METHODS: From January 2010 to December 2013, 149 patients underwent definitive right ventricular outflow tract reconstruction using the expanded polytetrafluoroethylene conduit; the 55 patients receiving a conventional conduit (group N) were compared with the 94 patients receiving a modified conduit (group T). RESULTS: There were no conduit-related deaths, operative deaths, or reimplantations for conduit failure. The overall survival and freedom from reintervention for conduit-related reasons at 3 years were 98.2% versus 95.6% (P = .438) and 94.7% versus 97.9% (P = .954) for groups N and T, respectively. The mean peak pressure gradients were 22.6 ± 15.6 mm Hg versus 18.2 ± 11.5 mm Hg (P = .161), and in the subanalysis within small-sized conduits, they were 30.2 ± 16.5 mm Hg versus 20.4 ± 10.7 mm Hg (P = .034). Regarding conduit insufficiency, the modified conduit showed a significantly worse grade of insufficiency (P = .014) only in the subanalysis within large-sized conduits. CONCLUSIONS: Although the clinical outcomes did not differ within midterm observation, the thin-type expanded polytetrafluoroethylene leaflet was considered to be suitable for the small-sized conduits, but not for large-sized conduits, based on the comparison of the hemodynamic performance. Long-term follow-up is necessary to address the appropriate sheet type for middle-sized conduits and to estimate the durability of the thin-type leaflet.

Polytetrafluoroethylene conduits versus homografts for right ventricular outflow tract reconstruction in infants and young children: An institutional experience.

OBJECTIVE: Our institution uses a valved polytetrafluoroethylene conduit as an alternative to homografts. The objective of this study was to investigate the performance of bicuspid valved polytetrafluoroethylene conduits used for right ventricular outflow tract reconstruction in children aged less than 2 years and to evaluate risk factors for earlier conduit explant. METHODS: We performed an Institutional Review Board-approved retrospective chart review of all patients aged less than 2 years who underwent surgical right ventricular outflow tract reconstruction with a bicuspid valved polytetrafluoroethylene conduit or homograft conduit from July 2004 to December 2014. The end points of the study were defined as conduit explant, conduit explant or reintervention, conduit stenosis, and conduit insufficiency. RESULTS: Fifty-four patients underwent 65 right ventricular outflow tract reconstructions with a bicuspid valved polytetrafluoroethylene conduit (n = 39) or a homograft conduit (n = 26, 23 pulmonary, 3 aortic). The majority of diagnoses were truncus arteriosus (n = 28) and tetralogy of Fallot with pulmonary atresia (n = 19). Median age of patients at surgery was 134 (8-323) days and 128 (7-384) days in the PTFE and homograft groups, respectively. There was no difference in demographic data between the 2 groups. Time-to-event analysis demonstrated no difference in time to explant (P = .474) or time to explant or reintervention (P = .206) between the 2 conduit types. Younger age at surgery was the only independent risk factor for conduit explant (subdistribution hazard ratio 1.104 per 30 days younger, P < .001). There was no significant influence of conduit type on the development of moderate conduit stenosis (P = .931) or severe conduit insufficiency (P = .880). Larger conduit z score was protective for the development of moderate conduit stenosis (subdistribution hazard ratio, 0.46; P = .001). CONCLUSIONS: Bicuspid valved polytetrafluoroethylene conduits are a satisfactory choice for right ventricular outflow tract reconstruction in patients aged less than 2 years. Their availability, low cost, and lack of potential sensitization make them an appealing alternative to homograft conduits.

Ultrasound biomicroscopy image patterns in normal upper eyelid and congenital ptosis in the Indian population.

PURPOSE: To study the features of upper eyelid in healthy individual and different types of congenital ptosis in the Indian population using ultrasound biomicroscopy (UBM). METHODS: This was a prospective observational study at a tertiary care center. Eyelid structure of healthy individuals with no eyelid abnormalities (n = 19); simple congenital ptosis (n = 33) cases; Marcus Gunn jaw-winking ptosis (MGJWP, n = 7) cases, and blepharophimosis-ptosis-epicanthus inversus syndrome (BPES, n = 20) cases were studied on a vertical UBM scan using 50-MHz probe. Lid-thickness, tarsal-thickness, orbicularis oculi and levator-Muller-orbital septum-conjunctival (LMSC) complex were measured in primary gaze. Comparison was made between four groups and results were statistically analyzed using ANOVA test. In normal individuals, LMSC measurements were repeated in down-gaze imaging. RESULTS: Skin with subcutaneous tissue, LMSC complex and pre-aponeurotic fat-pad appeared echodense while orbicularis oculi and tarsus appeared echolucent. In primary gaze, mean thickness (± standard deviation) of the eyelid, tarsus, orbicularis oculi and LMSC, respectively, were: 1.612 ± 0.205, 0.907 ± 0.098, 0.336 ± 0.083, and 0.785 ± 0.135 mm in normal individual. LMSC showed 46.64% increase in thickness on down-gaze. The mean eyelid thickness and LMSC were thicker in MGJWP and BPES as compared to normal. In different types of congenital ptosis cases, various patterns of UBM imaging were observed. CONCLUSION: UBM allows noninvasive imaging of eyelid structures with good anatomical correspondence in normal eyelids and study the structural alterations of eyelids in different types of congenital ptosis. UBM can be used to highlight the anatomical difference in normal eyelids that may help modify the surgery for better cosmetic outcomes. Furthermore, it has the potential to be used in preoperative evaluation and operative planning in certain types of acquired ptosis, which needs to be evaluated.