Surgical Correction Research Articles

Operative Management of Y-Shaped Metatarsal with Biphalangeal Sixth Toe.

In the literature, there is no consensus regarding the surgical management of postaxial polydactyly, and few cases of polymetatarsia with polydactyly have been reported. Treatment of the complete deformity will prevent further foot and gait disorders. To identify literature relevant to the operative management of Y-shaped metatarsal with biphalangeal sixth toe and related skin and wound care to improve surgical treatment protocols from a clinical experience perspective. The authors searched several electronic databases in December 2022 for articles related to postaxial polysyndactyly in the feet and polymetatarsia. Databases searched included PubMed, SciELO, ScienceDirect, Cochrane Database of Systematic Reviews, and Google Scholar gray literature. Two independent researchers conducted the searches and read the article titles and abstracts. Studies were included if they were narrative reviews, case studies, or observational studies; written in English or Spanish; and published between 2012 and 2022. Nonhuman studies were excluded. Studies that met the inclusion criteria were fully evaluated. Disagreements between reviewers were resolved by consensus, and when there was no consensus, a senior researcher was consulted. The following data were extracted from the included studies using a standardized form: author and year of publication, study type, number of participants, sex, polydactyly location, polymetatarsia, type of polydactyly, participants' history of hereditary associated diseases or malformations, treatment, removal criteria, and timing of surgery. Authors evaluated 11 studies of postaxial polydactyly that included a total of 153 participants (64 men, 89 women). They also document their clinical experience with a surgical technique used in cases of bilateral postaxial polydactyly of the foot with a Y-shaped metatarsal with biphalangeal sixth toe. Surgical correction with lateral removal of the sixth toe is a resolutive treatment to improve the functionality of the foot, its aesthetic appearance, and the patient's quality of life. Case-specific treatment should be applied and tailored to meet the individual needs. The biomechanics of gait and shoe problems in these patients improve with surgical treatment, without presenting secondary aesthetic problems in skin care.

RECURRENCE AFTER MINIMALLY INVASIVE REPAIR OF INGUINAL HERNIA: INCIDENCE AND ASSOCIATED FACTORS

Abstract Background Minimally invasive laparoscopic surgery for inguinal hernia allows an approach to the entire myopectineal orifice, leading to an expected lower recurrence rate and/or incidence of occult hernias. Nevertheless, the literature reports recurrence rates of up to 12%, depending on the reported follow-up time. Methods Retrospective analysis of laparoscopic surgical corrections of inguinal hernias between January 2018 and March 2023. Evaluation of recurrence rate and factors associated with its development. Results During the analyzed period, 388 patients underwent laparoscopic surgical correction of inguinal hernia. In 183 patients, the surgical correction was bilateral, corresponding to 571 operated hernias (82% via TAPP and 18% via TEP). During an average follow-up of 22 months (6–65), the recurrence rate was 2.1%, diagnosed on average 14 months after surgery. The presence of postoperative wound complications and chronic pain were the only factors significantly associated with recurrence (p = 0.026, p <0.001, respectively). Factors such as gender (p = 0.155), age (p = 0.13), BMI (p = 0.3), smoking (p = 0.52), technique used (p = 0.391), recurrent hernia (p = 0.42) did not show an association with hernia recurrence. In multivariate analysis, the presence of postoperative complications was the only independent risk factor for recurrence (p = 0.04, OR 4). Conclusion In our study, the recurrence rate aligns with what is described in the literature. Limitations include the fact that only patients who returned to our hospital were diagnosed, and the short follow-up time in some cases. The learning curve of surgeons, both residents and specialists, during the implementation of the technique in our service may also impact these results.

Is there a place for endoscopic management in post-cholecystectomy iatrogenic bile duct injuries?

In this editorial we comment on the article by Emara et al published in the recent issue of the World Journal of Gastrointestinal Surgery . Previously, surgery was the primary treatment for bile duct injuries (BDI). The treatment of BDI has advanced due to technological breakthroughs and minimally invasive procedures. Endoscopic and percutaneous treatments have largely supplanted surgery as the primary treatment for most instances in recent years. Patient management, including the specific technique, is typically impacted by local knowledge and the kind and severity of the injury. Endoscopic therapy is a highly successful treatment for postoperative benign bile duct stenosis and offers superior long-term outcomes compared to surgical correction. Based on the damage features of BDI, therapeutic options include endoscopic duodenal papillary sphincterotomy, endoscopic nasobiliary drainage, and endoscopic biliary stent implantation.

Successful treatment of Giant keloid on the right toes using trepanation combined with superficial radiotherapy (SRT-100): a case report with literature review

In dermatology, a keloid is one of the most common skin morphological abnormalities caused by excessive proliferation of fibroblasts. Keloids that are large or occur near important joint sites often cause varying degrees of physiological dysfunction in patients, therefore requiring medical treatment. A boy with congenital syndactyly developed huge keloids at the surgical site after undergoing surgical correction treatment. After treatment using trepanation combined with superficial radiotherapy (SRT-100) in our hospital, most of the boy’s keloids shrank and flattened. The affected foot returned to its normal appearance, and the boy could wear shoes normally. The boy did not complain of pain, numbness, or any other distinctive discomfort after completing the treatment. This suggested that the combination of trepanation and SRT-100 may be one of the options for treating hypertrophic keloids that cannot be treated by surgical excision.

Stages of motor development in children with cyanotic congenital heart defects after surgical treatment

Background. The article deals with the actual problem of pediatric neurology, pediatric cardiology, and pediatric cardiac surgery, in particular, the peculiarities of neurodevelopment in children with congenital heart defects (CHD). The purpose is to conduct a study of stages of stato-motor development (SMD) in patients with cyanotic congenital heart defects after surgical treatment and to determine the influence of pathogenic factors on neurodevelopmental delay. Materials and methods. Thirty-five patients with cyanotic CHD underwent examination and surgical treatment at the Center for Pediatric Cardiology and Cardiac Surgery of the Ministry of Health of Ukraine: 20 children — with transposition of the great vessels (TGV) and 15 children — with tetralogy of Fallot (TOF). The control group consisted of 35 healthy patients whose motor development corresponded to their age. Gestational age, body weight at birth, Apgar score, saturation, and duration of artificial blood circulation during surgical treatment for CHD were considered. Assessment of motor development was carried out with the help of the following scales: Munich Functional Developmental Diagnosis (G.J. Köhler, H.D. Egelkraut) and section 2 of the Hammersmith Infant Neurological Examination. Data were processed using the software Microsoft Excel version 16.0.12527.21236. Results. The gestational age of the studied group of patients with cyanotic CHD was 38 weeks, body weight — 3,500 g, which corresponded to the control group. Patients of the first subgroup with TGV were diagnosed with lower saturation indicators (р = 0.0001), a lower 1-minute Apgar score (p = 0.028) and a longer duration of artificial blood circulation — 146.00 ± 29.03 (р = 0.043) compared to the second subgroup with TOF. SMD delay was diagnosed in 29 % of cases, namely 12 % with TGV and 17 % with TOF. The average period of sitting with delayed SMD was 8.4 ± 2.2 months (p = 0.002), walking — 18.4 ± 6.3 months (p = 0.0001). Subsequently, children with TGV continue to develop without a significant delay compared to the TOF subgroup. Conclusions. 1. Patients with cyanotic CHD are born at 38 weeks of gestation, with sufficient body weight without a significant difference depending on the type of CHD and compared to the control group of healthy children. The predominance of the male gender is typical in cyanotic CHD. However, children with transposition of the great vessels are characterized by a lower 1-minute Apgar score and lower indicators of saturation at birth compared to those with tetralogy of Fallot. 2. Based on the conducted study, a delay of SMD was found in 29 % of patients with cyanotic CHD, namely 17 % with TOF and 12 % with TGV. The average duration of sitting with delay was 8.4 ± 2.2 months and walking was 18.4 ± 6.3 months. 3. Surgical treatment was carried out according to the detected indications, for transposition of the great vessels on the first day and for tetralogy of Fallot at 8 months of life. No pathogenic influence of the duration of artificial blood circulation and aortic clamping during cardiosurgical treatment on SMD in children was found. An increase in the duration of artificial blood circulation was noted during arterial switch operation in children with TGV, but no significant difference was found in children with SMD delay. 4. Timely early surgical treatment of critical CHD — TGV on the first day after birth, a developed route for pregnant women, techniques of performing operations — ensure a better SMD of this subgroup in the future. In patients with TOF, taking into account the long period of hypoxia before the start of surgical correction, neuropsychiatric disorders of varying degrees are more often noted. The development of an individual rehabilitation program is provided for this category of children.

Prenatally Diagnosed Congenital High Airway Obstruction Syndrome: Perinatal Management and Outcome—A Single Tertiary Care Center Experience

AbstractTo report our experience with the management of prenatally diagnosed cases of congenital high airway obstruction syndrome (CHAOS) and the postnatal outcome of those who underwent an ex utero intrapartum treatment (EXIT) procedure. This is a single center, retrospective observational study of prenatally diagnosed CHAOS cases using two-dimensional ultrasound from December 2017 to December 2022 in a tertiary care facility. Of the total nine fetuses prenatally diagnosed with CHAOS, three (33.3%) were associated with multiple congenital anomalies, seven out of nine (77.8%) developed ascites, and one had fetal hydrops. Five (55.6%) underwent medical termination of pregnancy and two were lost to follow-up (22.2%). The remaining two continued pregnancy and required EXIT tracheostomy at the time of delivery (22.2%). Microarray was performed in both which was normal. Postnatally, both infants are tracheostomy dependent with one requiring frequent ventilator support. CHAOS even when isolated generally has poor prognosis without intervention. Performing an EXIT procedure at birth can significantly improve postnatal survival by minimizing hypoxic damage. However, the long-term medical and surgical challenges for survivors remain numerous especially speech disorders, even after lifesaving fetal intervention and surgical correction. Therefore, an accurate prenatal diagnosis is necessary to give the couple an option of continuing pregnancy after realistic counseling regarding the prognosis and postnatal outcome.

Inconsistency in the Reporting Terminology of Adverse Events and Complications in Hallux Valgus Reconstruction: A Systematic Review.

Surgical complications are inevitable in any surgical subspecialty. Throughout the years, many classification systems have been developed to better understand and report such complications. The aim of this systematic review is to investigate the variability and frequency of reporting terms used to describe adverse events and complications in hallux valgus reconstruction. We hypothesized that the terms used would be highly inconsistent, which further promotes a need for a standardized terminology reporting system. Studies related to hallux valgus reconstruction outcomes that met our predetermined inclusion criteria were investigated to identify and report the related adverse terms and complications. Adverse terms and complications were grouped into 9 categories. Of the 142 studies included, 376 distinct terms that described adverse events or complications related to hallux valgus reconstruction were identified. Of these, 73.4% (276/376) were mentioned only once in their respective studies. Five of 376 terms were mentioned in at least 25% of the papers, and only 2 of 376 were mentioned in at least 50%. The most frequently reported adverse events were "Recurrence," mentioned in 77 of 142 studies (54%), followed by "Nonunion," mentioned in 76 of 142 studies (53%). The most reported category was "Bone/Joint" with 135 related terms, mentioned in 135 of 376 of the papers (95.1%). The terminology used in reporting adverse events and complications in surgical hallux valgus correction was highly inconsistent and variable. This represents yet another barrier in accurate reporting of these terms, and subsequently a difficult analysis of the outcomes related to hallux valgus reconstruction. To overcome these challenges, we suggest developing a standardized terminology reporting system.Levels of Evidence: Level III; systematic review of Level III studies and above.

Surgical Management of Postoperative Nipple Necrosis After Inverted Nipple Correction: Experiences From a Series of 25 Cases.

The inverted nipple is a condition that affects approximately 10% of women and can have negative cosmetic and psychological implications. Surgical correction is a common approach to address this concern; however, this method can lead to complications, such as nipple necrosis. As comprehensive guidelines are currently lacking for postoperative nipple necrosis management, this study reports our experience in the management of postoperative nipple necrosis following initial attempt at surgical management. A retrospective chart review was conducted and included female patients who experienced postoperative nipple necrosis after inverted nipple correction between 2018 and 2021. Cases of recurrent nipple retraction following partial necrosis and cases of complete nipple necrosis were evaluated. Recurrent nipple retraction was managed using various inverted nipple correction techniques, while complete necrosis required a modified C-V flap for nipple reconstruction. A total of 25 patients with a total of 42 affected nipples were included. Thirteen cases (26 nipples) experienced recurrent nipple retraction following partial necrosis, while 12 cases (16 nipples) exhibited complete necrosis. No significant predictive variables for these complications were found. Notably, all patients achieved successful healing following single-stage surgical repair. At 6 months postoperation, the treated nipples exhibited satisfactory healing and appearance and an absence of infection or papillary necrosis. Seven reconstructed nipples showed a mean loss of projection (2.7 ± 0.98) compared with only 2 nipples in the inverted nipple correction group. Distinguishing between recurrent nipple retraction after partial necrosis and complete nipple necrosis is crucial and should be taken into consideration when managing patients following inverted nipple correction.

Apical Suspension Repair for Vaginal Vault Prolapse: A Randomized Clinical Trial.

The optimal surgical repair of vaginal vault prolapse after hysterectomy remains undetermined. To compare the efficacy and safety of 3 surgical approaches for vaginal vault prolapse after hysterectomy. This was a multisite, 3-arm, superiority and noninferiority randomized clinical trial. Outcomes were assessed biannually up to 60 months, until the last participant reached 36 months of follow-up. Settings included 9 clinical sites in the US National Institute of Child Health and Human Development (NICHD) Pelvic Floor Disorders Network. Between February 2016 and April 2019, women with symptomatic vaginal vault prolapse after hysterectomy who desired surgical correction were randomized. Data were analyzed from November 2022 to January 2023. Mesh-augmented (either abdominally [sacrocolpopexy] or through a vaginal incision [transvaginal mesh]) vs transvaginal native tissue repair. The primary outcome was time until composite treatment failure (including retreatment for prolapse, prolapse beyond the hymen, or prolapse symptoms) evaluated with survival models. Secondary outcomes included patient-reported symptom-specific results, objective measures, and adverse events. Of 376 randomized participants (mean [SD] age, 66.1 [8.7] years), 360 (96%) had surgery, and 296 (82%) completed follow-up. Adjusted 36-month failure incidence was 28% (95% CI, 20%-37%) for sacrocolpopexy, 29% (95% CI, 21%-38%) for transvaginal mesh, and 43% (95% CI, 35%-53%) for native tissue repair. Sacrocolpopexy was found to be superior to native tissue repair (adjusted hazard ratio [aHR], 0.57; 99% CI, 0.33-0.98; P = .01). Transvaginal mesh was not statistically superior to native tissue after adjustment for multiple comparisons (aHR, 0.60; 99% CI, 0.34-1.03; P = .02) but was noninferior to sacrocolpopexy (aHR, 1.05; 97% CI, 0-1.65; P = .01). All 3 surgeries resulted in sustained benefits in subjective outcomes. Mesh exposure rates were low (4 of 120 [3%] for sacrocolpopexy and 6 of 115 [5%] for transvaginal mesh) as were the rates of dyspareunia. Among participants undergoing apical repair for vaginal vault prolapse, sacrocolpopexy and transvaginal mesh resulted in similar composite failure rates at study completion; both had lower failure rates than native tissue repair, although only sacrocolpopexy met a statistically significant difference. Low rates of mesh complications and adverse events corroborated the overall safety of each approach. ClinicalTrials.gov Identifier: NCT02676973.

Minimally Invasive Controlled Growing Rods for the Surgical Treatment of Early-Onset Scoliosis—A Surgical Technique Video

Background: Spinal deformities in children and adolescents can be easily divided into those occurring and diagnosed before the age of 10—early-onset scoliosis—and those occurring and diagnosed after the age of 10—late-onset scoliosis. When the curvature continues to progress and exceeds a Cobb angle of more than 60–65 degrees, surgical treatment should be considered. The most common treatment procedure for EOS is the surgical correction of the deformity using standard growing rods (SGRs), and in the case of congenital defects with additional hemivertebrae, it is the resection of the hemivertebra and short fusion. Minimally invasive controlled growing rods (MICGRs) need to be distracted every 6–9 months through a minimally invasive approach that involves sedation and neuromonitoring to obtain the best possible correction while minimizing complications. The aim of our study is to present a less-invasive surgical technique for MICGR implantation based on a two-case presentation—early-onset idiopathic scoliosis and congenital kyphosis. The surgical technique is the less-invasive percutaneous and subfascial implantation of MICGRs without long incisions in the back. Conclusions: The use of MICGRs is an alternative and safe surgical technique for patients undergoing surgical treatment for EOS. Without the risk of metallosis, like in other implant systems, and the need for replacement after 2 years of use, like in using magnetically controlled growing rods (MCGRs), the MICGR system can be used as a less-invasive procedure, allowing for the avoidance of many periodic invasive procedures in children with a wider opening of the spine (like in using standard growing rods), minimizing the number of planned hospitalizations, reducing the length of hospital stays, and reducing the physical and mental burdens on young patients, parents, and families.

Scimitar Syndrome in a Pediatric Cohort.

Scimitar syndrome is a rare form of congenital heart disease (CHD) characterized by anomalous pulmonary venous drainage of the right lung to the inferior vena cava. We describe the presentation, diagnosis, therapeutic management and long-term follow-up of 10 pediatric patients with Scimitar Syndrome. We performed a retrospective observational study of all pediatric patients from our institution with scimitar syndrome (March 1996-July 2023). Patients underwent systematic evaluation including medical and family history, chest x-ray, 12-lead electrocardiogram, echocardiogram, angiography and/or computed tomography; or magnetic resonance angiography. Ten patients with scimitar syndrome were included. The median age at diagnosis was 10.4 [0.1-150.2] months and the median follow-up time was 7.7 [1.3-15.3] years. Eight patients presented with aortopulmonary collateral arteries which were embolized. Two patients had dual connections to the inferior vena cava and left atrium; embolization of the inferior vena cava connection was only feasible in one of them. No patients underwent surgery of the scimitar vein. Three patients had surgical correction of CHDs. There were no deaths related to scimitar syndrome during follow-up. All patients with scimitar syndrome need prompt cardiovascular evaluation and follow-up. Our study demonstrates that a conservative approach with aortopulmonary collateral artery embolization, scimitar vein embolization when dual drainage to the left atrium is identified, along with correction of concomitant CHDs might have good results in patients with scimitar syndrome in order to postpone surgical correction of the anomalous pulmonary venous return to an older age when clinically or hemodynamically indicated. Further studies with longer-term follow-up and a larger sample size are needed to more effectively determine treatment strategy.

THE IMPORTANCE OF ULTRASONOGRAPHY IN THE DETECTION OF CONGENITAL HEART DISEASES IN CHILDREN

Over the last few years, remarkable progress has been made in the field of congenital heart diseases. Improvements considering diagnostic modalities, especially imaging, in surgical and interventional techniques, as well as in postoperative therapy and care, have contributed to a significant reduction in mortality and morbidity. One of the most important applications of medical imaging techniques in children is the detection and treatment of congenital heart anomalies. Objective of this article is to show the importance of ultrasonography in the detection of congenital heart diseases in children. The study was conducted on children with simple and complex congenital heart diseases and was conducted on the Pediatric Clinic, UKCS. The research is descriptive on a representative sample. In our study, 166 children were observed, of which 148 children (77 boys, 71 girls) with simple congenital heart diseases, and 18 children (8 boys, 10 girls) with complex congenital heart diseases. Out of the total number of observed children, 115 had a surgical correction, 97 children with simple congenital heart diseases (45 boys, 52 girls) and 18 children with complex congenital heart diseases (8 boys, 10 girls). The number of children monitored through the Cardiac Counseling Center who didn’t undergo surgical correction was 51, all with simple congenital heart diseases. Out of the total number of observed children who were frequently coming for follow-ups, 28 children had changes on the ECG, and 138 of them had no changes on the ECG, 93 were surgically treated, and 73 of them were on conservative therapy. Based on the results of the research, we conclude that ultrasonography is an important method in the detection and treatment of congenital heart diseases.

New diagnostic criteria for metopic ridges and trigonocephaly: a 3D geometric approach

BackgroundTrigonocephaly occurs due to the premature fusion of the metopic suture, leading to a triangular forehead and hypotelorism. This condition often requires surgical correction for morphological and functional indications. Metopic ridges also originate from premature metopic closure but are only associated with mid-frontal bulging; their surgical correction is rarely required. Differential diagnosis between these two conditions can be challenging, especially in minor trigonocephaly.MethodsTwo hundred seven scans of patients with trigonocephaly (90), metopic rigdes (27), and controls (90) were collected. Geometric morphometrics were used to quantify skull and orbital morphology as well as the interfrontal angle and the cephalic index. An innovative method was developed to automatically compute the frontal curvature along the metopic suture. Different machine-learning algorithms were tested to assess the predictive power of morphological data in terms of classification.ResultsWe showed that control patients, trigonocephaly and metopic rigdes have distinctive skull and orbital shapes. The 3D frontal curvature enabled a clear discrimination between groups (sensitivity and specificity > 92%). Furthermore, we reached an accuracy of 100% in group discrimination when combining 6 univariate measures.ConclusionTwo diagnostic tools were proposed and demonstrated to be successful in assisting differential diagnosis for patients with trigonocephaly or metopic ridges. Further clinical assessments are required to validate the practical clinical relevance of these tools.

MITRACLIP TREATMENT FOR CARDIOGENIC SHOCK DUE TO ACUTE MITRAL REGURGITATION IN A PATIENT WITH POSTINFARCTION PAPILLARY MUSCLE RUPTURE

Abstract A 85–year–old woman presented to ED with worsening dyspnea for 24 hours; no other related symptom. Her medical history included hypertension, dyslipidemia, type 2 DM and surgical coronary revascularization 13 years before for multiple critical coronary artery disease (LIMA to LAD and sequential SVG to PDA and OM branch). The ECG promptly revealed a subacute infero–lateral STEMI. Transthoracic echocardiography (TTE) showed inferior and postero–lateral wall akinesia with severely reduced left ventricle ejection fraction (LVEF), mildly reduced right ventricle systolic function and severe mitral regurgitation (MR) due to posterior papillary muscle (PM) rupture (Fig.1). Clinical scenario was complicated by pulmonary edema and cardiogenic shock treated with diuretics and sodium nitroprusside infusion, non–invasive ventilation and IABP insertion. Urgent coronary angiography revealed a thrombotic occlusion of the venous graft for OM branch. Surgical correction was excluded due to hemodynamic instability. Graft occlusion was treated with manual thromboaspiration, balloon dilatations and abciximab infusion. After an initial stabilization, transesophageal echocardiography (TEE) was performed confirming severe MR resulting from rupture of one head of a bifid posterior PM (red arrows, Fig.1) and prolapse of the anterior leaflet combined with restrictive systolic movement of the posterior leaflet due to wall motion abnormalities. 3 days later a transcatheter mitral valve edge–to–edge repair (MTEER) was performed using a MitraClip device (1 XTR clip and 1 NTR clip, Fig.2). TEE with 3D and TrueVue vision confirmed the correct placement of clips (Fig.3), a mild–to–moderate residual MR, absence of significant transmitral gradient and a mildly reduced LVEF. No clinical events were reported during recovery; patient was discharged after 2 weeks. Conclusion MTEER is an established therapy for reducing degenerative and functional MR. Little data is available on its use in the setting of PM rupture. This rare complication of AMI carries a mortality rate of 80% during the first week without surgical correction but more than half of patients are excluded because of prohibitive operative risk. TEER may be an alternative for these patients. In this case report we described the utility of MTEER in the setting of cardiogenic shock secondary to acute ischemic MR with papillary muscle partial rupture. This procedure can be useful with acceptable safety and procedural success.

Clinical and radiological evaluation of caudal regression syndrome.

Caudal regression syndrome is a form of segmental spinal dysgenesis involving the caudal spinal column, ranging from segmental coccygeal agenesis to extensive thoracolumbar agenesis with varying degrees of spinal cord dysgenesis. A majority of caudal regression cases are sporadic but maternal pre-gestational diabetes mellitus is an important risk factor. Imaging is an integral part of management of caudal regression syndrome. Antenatal diagnosis on obstetric ultrasound and evaluation with fetal MRI is ideal. Early postnatal diagnosis and/or detailed evaluation with MRI is essential for early management to improve outcomes. Pang classification categorizes caudal regression syndrome into two categories based on the position of the conus while Renshaw classification is based on the degree of vertebral column agenesis. Caudal regression syndrome may be associated with several additional anomalies, both spinal and extraspinal. A number of genitourinary and gastrointestinal anomalies have been described in association with caudal regression syndrome. The field of view of MRI of the lumbosacral spine in caudal regression syndrome needs to be extended to visualize the retroperitoneal structures without the use of a saturation band. Syndromic associations may be suspected, and additional imaging performed, based on findings of extended field of view MRI of the spine. Associated sacral masses and filar abnormalities need to be identified and may also require surgical treatment. The multisystem nature of this disease necessitates a multimodality approach to the evaluation and management of caudal regression syndrome with close cooperation between pediatric neuroradiologists and body radiologists as well as multiple clinical teams. Appropriate early management with surgical correction as necessary can significantly improve prognosis and survival in caudal regression syndrome.

THE SIGN OF THE HORNS: A RARE CASE OF DOUBLE PERI–COMMISSURAL MITRAL FLAIL

Abstract A 80–year–old man with history of hypertension and dyslipidemia was admitted to our Cardiology Unit for exertional dyspnea (NYHA III) occurred during the last few days. The physical examination showed a systolic heart murmur (4/6 on Levine scale) and signs of pulmonary congestion. Transthoracic echocardiography revealed a massive mitral regurgitation (Fig.1) together with severe dilation of the left atrium, mild–to–moderate aortic regurgitation, high probability of pulmonary hypertension and normal biventricular systolic function. A transesophageal echocardiography (TEE) was performed to clarify mitral valve anatomy. A double flail of the posterior leaflet was present involving the peri–commissural portions due to a single chordal rupture on P1 scallop and a double chordal rupture on P3 scallop (Fig.2). P2 scallop was prolapsing and there was a billowing of the anterior leaflet. In addiction mitral annulus was dilated (antero–posterior diameter of 43 mm and intercommissural diameter of 45 mm). We named this finding “sign of the horns” for his 3D echocardiographic appearance (Fig.3) given the double flail resembling two fingers. A surgical correction was performed with 3 GoreTex neochords implantation on the posterior leaflet and an annuloplasty ring (Physio II n. 34) implantation during extracorporeal circulation through right anterior minithoracotomy. Surgical and echocardiographic results were optimal. The patient was discharged after 7 days of hospitalization. Conclusion Coexistence of a double peri–commissural flail in the same leaflet is a very rare echocardiographic finding and we named it “double horn sign”. TEE is an essential examination in this scenario in order to understand the exact mechanism of the regurgitation for an optimal preoperative planning.

Long-Term Follow-Up after Ureteral Reimplantation in Children: A 12-Year Analysis

Abstract Background Ureteral reimplantation (UR) is a well-established procedure, yet there exists a notable absence of evidence-based guidelines for postoperative follow-up. Existing literature suggests that routine follow-up extending beyond one year may not yield significant advantages, particularly for asymptomatic children. However, it is crucial to acknowledge that many studies supporting this assertion rely on relatively short observation periods. Aims This study seeks to address the potential emergence of late-presenting complications post UR. Our objective is to comprehensively evaluate our patient cohort for the incidence of conditions warranting the establishment of standardized, long-term follow-up practices after UR. Methods Retrospective review of 137 medical records from children who underwent UR at our institution between January 2006 and June 2013. Inclusion criteria required a minimum 10-year postoperative follow-up. Results Fifty-three renal units in 34 children met inclusion criteria. Ureterovesical junction (UVJ) obstruction occurred in 7.5% of the units and was diagnosed within eight months after UR. Obstruction occurred exclusively after Politano-Leadbetter reimplantation and was silent in all cases. Postoperative UTIs occurred in 9 children. Notably, these were all singular events without signs for recurrent reflux. A decade after surgery, one child developed chronic kidney disease (CKD) due to preexisting reflux nephropathy (RN), while one child was newly diagnosed with RN during follow-up despite the timely surgical correction of vesicoureteral reflux (VUR). or hypertension linked to RN were recorded. Conclusion Children with uncomplicated postoperative recoveries within the first-year, devoid of evident UVJ obstruction, exhibit a low subsequent risk, suggesting limited benefit from repeated scheduled long-term follow-up. Nevertheless, we recommend that general practitioners conduct regular monitoring, considering individual risk factors, to enhance the detection of late-onset hypertension or proteinuria as potential indicators of RN during adolescence.

Degenerative mitral regurgitation due to flail leaflet: sex-related differences in presentation, management, and outcomes.

Presentation, outcome, and management of females with degenerative mitral regurgitation (DMR) are undefined. We analysed sex-specific baseline clinical and echocardiographic characteristics at referral for DMR due to flail leaflets and subsequent management and outcomes. In the Mitral Regurgitation International Database (MIDA) international registry, females were compared with males regarding presentation at referral, management, and outcome (survival/heart failure), under medical treatment, post-operatively, and encompassing all follow-up. At referral, females (n = 650) vs. males (n = 1660) were older with more severe symptoms and higher MIDA score. Smaller cavity diameters belied higher cardiac dimension indexed to body surface area. Under conservative management, excess mortality vs. expected was observed in males [standardized mortality ratio (SMR) 1.45 (1.27-1.65), P < .001] but was higher in females [SMR 2.00 (1.67-2.38), P < .001]. Female sex was independently associated with mortality [adjusted hazard ratio (HR) 1.29 (1.04-1.61), P = .02], cardiovascular mortality [adjusted HR 1.58 (1.14-2.18), P = .007], and heart failure [adjusted HR 1.36 (1.02-1.81), P = .04] under medical management. Females vs. males were less offered surgical correction (72% vs. 80%, P < .001); however, surgical outcome, adjusted for more severe presentation in females, was similar (P ≥ .09). Ultimately, overall outcome throughout follow-up was worse in females who displayed persistent excess mortality vs. expected [SMR 1.31 (1.16-1.47), P < .001], whereas males enjoyed normal life expectancy restoration [SMR 0.92 (0.85-0.99), P = .036]. Females with severe DMR were referred to tertiary centers at a more advanced stage, incurred higher mortality and morbidity under conservative management, and were offered surgery less and later after referral. Ultimately, these sex-related differences yielded persistent excess mortality despite surgery in females with DMR, while males enjoyed restoration of life expectancy, warranting imperative re-evaluation of sex-specific DMR management.

CASE PRESENTATION: A COMPLEX CLINICAL SCENARIO IN A 43–YEAR–OLD MALE WITH DEVICE–RELATED COMPLICATIONS

Abstract A 43 yo male presented with an exposed pocket of his ICD in the right prepectoral and axillary region, along with total exposure of the lead electrodes up to the fixation sleeves.The patient, a known carrier of double–outlet right ventricle, underwent surgical correction. At the age of one, he received a dual–chamber pacemaker in the left prepectoral region due to AVB. Due to recurrent sustained ventricular tachycardias an ICD was implanted in the right prepectoral region, with removal of the left–sided pacemaker and concurrent abandonment of the ventricular pacing lead. In October 2019, he was admitted for a recurrence of pocket erosion.Device extraction was proposed but declined by the patient. Subsequently, he was referred to our institution for device removal. After heart team discussion, the patient was offered a transvenous lead extraction, with potential cardiothoracic surgical intervention if transvenous extraction proved to be challenging.An attempt was made to extract the leads using a transvenous approach. The procedure proceeded without complications, and the leads were released from adhesions up to the superior vena cava. However, there was an adhesion point at this location that was not overcome by mechanical dilation.Due to this limitation, a few days later, a cardiothoracic surgical intervention was performed to complete the lead wires‘ extraction using an invasive approach. In this context, a cardiac resynchronization device CRT–P was reimplanted in the left prepectoral region with epicardial electrodes. The patient underwent submuscular subclavian implantation of a SCID.The patient presented with a new pocket erosion of the CRT–P.Considering the high infection risk, It was therefore decided to implant a leadless device following electroanatomic mapping of the right ventricle, as there were surgical patches in the septal region. This approach aimed to identify the optimal anchoring site for the device. Subsequently, he will go extraction of the generator of the CRT–P.The decision to forgo biventricular stimulation was made, because of the elevated infectious risk.Complications associated with implantable devices pose an increasingly significant challenge in managing young patients with cardiac arrhythmias. Only through an integrated approach and proactive sharing of knowledge and expertise can we significantly improve clinical outcomes and the quality of life for this increasingly relevant population.

A 20–YEAR FOLLOW–UP OF SUCCESSFUL SURGICAL MANAGEMENT OF DOUBLE–OUTLET RIGHT VENTRICLE WITH PULMONARY STENOSIS: A CASE REPORT

Abstract A 25–year–old man, suffering from worsening exertional dyspnea for 4 months, was referred to our Cardiology Unit. The patient was previously diagnosed with a late diagnosis of double outlet right ventricle (DORV), as well as pulmonary stenosis, VSD, and right–sided aortic arch for which he underwent a first surgical procedure around the age of 2 involving an intersternal anastomosis through a left lateral thoracotomy approach. A second intervention took place 2 years later for a radical correction of the anomaly through a median sternotomy. The patient had sporadic follow–up since then. A baseline ECG was performed, showing evidence of sinus rhythm, normal AV with a bifid P wave in lead II, slight RAD, incomplete RBBB, and asymmetric negative T waves from V1 to V3 and in aVL, and biphasic T waves in V4–V5. We performed an echocardiogram which showed: a dilated RA, a normal–sized LA, an apparently intact interatrial septum without evident shunts; a normal sized LV; EF 60%; a moderately dilated, hypertrophic and hypertrabeculated RV; FAC 33%; normal aortic flow; a minimal MR; a massive residual pulmonary insufficiency; a moderate TR; a dilatated pulmonary trunk; a normal–sized and collapsible IVC upon inspiration; PAPs of 70 mmHg; no pericardial effusion. A maximal exercise stress test appeared to be normal throughout. However, due to the severe pulmonary hypertension he was referred to our PH centre. While these patients are commonly affected by residual early or late left ventricular outflow tract obstruction, fortunately our patient was not. DORV is a relatively common congenital heart defect characterized by an abnormal connection between the ventricles and arteries, in which both the aorta and the pulmonary trunk are connected completely or predominantly to the right ventricle. The anatomical spectrum of this anomaly is quite large, and it can be associated with the presence of a VSD that often extends towards one or both of the arterial valves, allowing the flow of blood from the left ventricle to the aorta. An accurate diagnosis through echocardiography or CMR is essential in order to guide the surgical repair and the appropriate therapeutic approach for individuals affected by this anomaly. A successful surgical correction of DORV is essential, but it is important to be aware of the potential complications of post–operative PH (especially in patients with preoperative PH), which necessitates close surveillance and appropriate therapeutic management.