Technical Aspects and Outcome of Multi-Staged and Single-Staged Thoracoabdominal Fenestrated Endovascular Aortic Repair.

PURPOSE: In some cases of endovascular thoracoabdominal or juxtarenal aortic aneurysm repair, a thoracic endograft in combination with a fenestrated renovisceral device may be needed in order to create a sufficient proximal landing zone. This study aimed to evaluate the technical aspects and postoperative morbidity of a single- or 2-stage approach. METHODS: Eighty-seven consecutive patients undergoing thoracic endovascular aortic repair (TEVAR) in combination with elective fenestrated repair (fenestrated endovascular aortic repair [FEVAR]; fenestrated Anaconda device) from 2015 to 2022 were included in this retrospective bicentric study. Underlying pathologies, aortic morphology, technical details, and postoperative morbidity were recorded. RESULTS: Single-staged ("1S," n=61) and 2-staged ("2S," n=26) interventions were compared. Indications were thoracoabdominal aneurysms (TAAAs) (Crawford I-IV) (n=56, 64%) and juxtarenal aneurysms (n=31, 36%). In 2S, the proportion of TAAA was higher than in 1S (2S: 77%, 1S: 59%; p=0.001). In 2S, the covered length of the descending aorta was longer (1S: 128±60 mm, 2S: 202±64 mm; p=0.003). Temporary aneurysm sack perfusion (TASP) was established in 11 (18%) of 1S and 1 (4%) of 2S patients (p=0.079), as well as cerebrospinal fluid (CSF) drainage catheter in 48 (79%) of 1S and 19 (73%) of 2S. The rate of spinal cord ischemia (SCI) and the severity of SCI were not different in both groups, with a total of 3 cases of persisting paraplegia. The rate of access complications was higher in 2S (n=6, 23%) than in 1S (n=4, 7%; p=0.027). Postoperative 30 day morbidity did not significantly differ in both groups and neither did 30 day mortality (4.6% in 1S vs 3.8% in 2S; p=0.083). CONCLUSION: The combination of TEVAR and FEVAR using a fenestrated endograft is feasible and safe. Aortic morphology does not change significantly after endovascular repair. A single-staged strategy is feasible with excellent results, especially in Crawford IV, Crawford V, or juxtarenal aneurysms. Two-staged repair is recommended in cases with long aortic coverage and a higher American Society of Anesthesiologists (ASA) class. Follow-up data are needed to evaluate the long-term stability of the TEVAR/FEVAR interconnection. CLINICAL IMPACT: Our study has revealed the safety and efficacy of the combination of TEVAR and FEVAR in the treatment of TAAAs and juxtarenal aneurysms with compromised supravisceral landing zones. A single-staged concept is not necessary in all cases. Staged procedures may reduce postoperative morbidity in cases with long aortic coverage and higher ASA class.

Pulmonary artery banding: still a role for staged bi-ventricular repair of intracardiac shunts?

OBJECTIVES: Although pulmonary artery banding remains a useful palliation in bi-ventricular shunting lesions, single-stage repair holds several advantages. We investigate outcomes of the former approach in high-risk patients. METHODS: Retrospective cohort study including all pulmonary artery banding procedures over 9 years, excluding single ventricle physiology and left ventricular training. RESULTS: Banding was performed in 125 patients at a median age of 41 days (2-294) and weight of 3.4 kg (1.8-7.32). Staged repair was undertaken for significant co-morbidity in 81 (64.8%) and anatomical complexity in 44 (35.2%). The median hospital stay was 14 days (interquartile range 8-33.5) and 14 patients (11.2%) required anatomical repair before discharge. Nine patients died during the initial admission (hospital mortality 7.2 %) and five following discharge (inter-stage mortality 4.8%). Of 105 banded patients who survived, 19 (18.1%) needed inter-stage re-admission and 18 (14.4%) required unplanned re-intervention. Full repair was performed in 93 (74.4%) at a median age of 13 months (3.1-49.9) and weight of 8.5 kg (3.08-16.8). Prior banding, 54% were below the 0.4th weight centile, but only 28% remained so at repair. Post-repair, 5/93 (5.4%) developed heart block requiring permanent pacemaker, and 11/93 (11.8%) required unplanned re-intervention. The post-repair mortality (including repairs during the initial admission) was 6/93 (6.5%), with overall mortality of the staged approach 13.6% (17/125). CONCLUSIONS: In a cohort with a high incidence of co-morbidity, pulmonary artery banding is associated with a significant risk of re-intervention and mortality. Weight gain improves after banding, but heart block, re-intervention, and mortality remain frequent following repair.

How and When Should Tetralogy of Fallot be Palliated Prior to Complete Repair?

The controversy regarding the best or ideal surgical management of Tetralogy of Fallot (ToF) stems from the recognition of there being a spectrum of morphology and associated lesions, each of which require a different approach to achieve the three goals of minimizing mortality preserving right ventricular function long-term and minimizing reinterventions. A one-size-fits-all approach to ToF needs to be replaced by a considered and personalized approach in order to yield the best outcomes possible for individual patients. The great majority of patients with ToF undergo primary complete repair between age 3-9 months with excellent outcomes. However, the greatest challenge is the severely cyanotic neonates where primary repair is still associated with high mortality and reintervention rates. Risk factors are low weight and small/poorly developed pulmonary vasculature. High-risk neonates have better outcomes with palliation-but mortality is still high. Palliative interventions in the catherization lab are showing better outcomes than traditional BT shunt and the RVOT stent is emerging as potential game-changer. Primary neonatal repair is still recommended if weight >3 kg and Nakata >100 mm2/m2. However, neonates with low weight, small pulmonary arteries or multiple comorbidities (including ToF/AVSD and anomalous LAD) may do better with a staged approach, There is good argument for RVOT stenting as a bridge to complete repair due to its stable circulation without diastolic run off and volume loading of the circulation, and its potential to allow branch PA growth.

Delayed presentation of anorectal malformations in a tertiary care hospital in India.

AIM: To study delayed presentation of ARMs, management and its effect on surgical and functional complications. METHODS: It is a retrospective study from March 2015 to March 2020. All the patients satisfying the criteria of delayed ARMs, i.e., presenting 7 days after birth were included. Information regarding type of ARM, mode of presentation, time of presentation, associated anomalies, management strategy, postoperative complications and functional outcome was noted. Minimum follow-up period was 6 months. RESULTS: Out of 102 patients with ARM, 44 patients presented late. Among the 44 patients, 9 were males and 35 were females. Associated comorbidities observed are low birth weight (n = 9) and preterm (n = 13). Associated anomalies observed were cardiac (n = 18), renal (n = 8), other gastrointestinal (n = 5) and skeletal (n = 1). (1) Male: rectourethral fistula-2 (staged repair), anal stenosis-3 (anoplasty) and anocutaneous fistula-4 (anoplasty). (2) Female: vestibular fistula: 15 (6 primary definitive surgery + 9 staged repair), ectopic anus: 3 (staged repair), anal stenosis: 2 (anoplasty), urogenital sinus: 4 (staged repair), H-type ARM: 8 (staged repair) and persistent cloaca: 3 (staged repair). Primary repair was done in 15 patients (34%), and staged repair was done in 29 patients (65.9%). Anoplasty was done in 9 patients, ASARP (modified tsuchida's procedure) in 8 patients and PSARP in 27 patients. Postoperative complications observed were constipation (n = 21, 47.7%), fecal incontinence (n = 12, 27.27%) with perianal excoriation in 2 patients, anal stenosis (n = 3, 6.8%) and rectal mucosal prolapse (n = 2, 4.5%) CONCLUSION: Delayed presentation of ARMs is not uncommon and is more common in females. Management is almost similar to those who present early. Those who present with chronic constipation and megarectum require staged repair. Complications were more frequent with delayed presentation. Hence, every newborn should have careful examination of perineum and screened for ARM to avoid possible morbidity and mortality.

Is two-staged repair for truncus arteriosus type A3 unpractical?

BACKGROUND: A complex and rare form of persistent truncus arteriosus needs careful attention when choosing the optimal strategy for repair. AIM OF THE STUDY: We herein describe our surgical strategy of a small infant having this malformation concomitantly with right aortic arch, unusual pulmonary artery branching and a left superior vena cava. METHODS: The patient underwent initially bilateral pulmonary arterial banding followed by the Rastelli type definitive repair. The pulmonary arteries were unified in front of the left superior vena cava, and the right ventricular outflow tract was readily reconstructed. RESULTS: The patient is doing well with an excellent hemodynamic status. CONCLUSIONS: We considered the two-stage approach was sensible in this particular patient.

Surgical Options for Pulmonary Atresia with Ventricular Septal Defect in Neonates and Young Infants.

BACKGROUND: The optimal surgical strategy for pulmonary atresia with ventricular septal defect (PA/VSD) in neonates and young infants is controversial. Staged repair may be associated with a higher risk of inter-stage mortality, while primary repair may lead to frequent post-repair re-interventions. METHODS: From 2004 to 2017, 65 patients with PA/VSD who underwent surgical intervention before 90 days of age were identified and enrolled in this retrospective study. The cohort was divided into two groups: group-SR, who underwent initial palliation with staged repair (n = 50), and group-PR who underwent primary repair (n = 15). RESULTS: There were three post-palliation in-hospital mortalities, four inter-stage mortalities, and one post-repair in-hospital mortality in group-SR. In group-PR, there was one in-hospital death and one late death. Five-year survival rates were comparable between the two groups (group-SR: 83.6%; group-PR: 86.7%; p = 0.754). During the median follow-up duration of 44.7 months (Inter-quartile range, 19-109 months), 40 post-repair re-interventions (22 in group-SR, 18 in group-PR) were performed in 26 patients (18 in group-SR, 8 in group-PR). On Cox proportional hazards model, primary repair was identified as the only risk factor for decreased time to death/1st post-repair re-intervention (Hazard ratio (HR): 2.3, p = 0.049) and death/2nd post-repair re-intervention (HR 2.91, p = 0.033). CONCLUSIONS: A staged repair strategy, compared with primary repair, was associated with comparable overall survival with less frequent re-interventions after repair in young infants with PA/VSD. Lowering the inter-stage mortality after initial palliation by vigilant outpatient care and aggressive home monitoring may be the key to better surgical outcomes in this subset. Surgical outcomes of PA with VSD according to the surgical strategies. Patient 1 (birth weight: 2.7 kg) underwent primary Rastelli-type repair at post-natal day # 50 (body weight: 3.8 kg) using Contegra® 12 mm. The postoperative course was rocky, with long ventilatory support (10 days), ICU stay (14 days), and hospital stay (20 days). Cardiac CT scan at 9 months post-repair showed severe branch pulmonary artery stenosis, which necessitated LPA stenting at 12 months post-repair and RV-PA conduit replacement with extensive pulmonary artery reconstruction at 25 months post-repair. Patient 2 (birth weight: 2.5 kg) underwent RMBT at post-natal day #30 (body weight: 3.4 kg) using 4 mm PTFE vascular graft and staged Rastelli-type repair at post-natal 11 months using a hand-made Gore-Tex valved conduit (14 mm). No post-repair re-intervention has been performed. Cardiac CT scan at 90 months post-repair showed no branch pulmonary artery stenosis.CT computed tomography, ICU intensive care unit, LPA left pulmonary artery, PA pulmonary atresia, PTFE polytetrafluoroethylene, RMBT right modified Blalock-Taussig shunt, RV-PA right ventricle to pulmonary artery, VSD ventricular septal defect.

A Novel Technique for Prevention of Gastroesophageal Reflux in Staged Repair of Long Gap Esophageal Atresia with Tracheoesophageal Fistula.

AIM: The objective of the study is to report a novel technique of preventing gastroesophageal reflux and air leak from fistula to stomach in patients of tracheoesophageal fistula with long gap atresia, to buy time for the staged procedure. METHODS: Seven patients of tracheoesophageal fistula with upper pouch of esophagus at 2nd thoracic vertebra were selected for the staged procedure. Weight ranged from 1.7 to 1.8 kg. During the 1st stage surgery for gastrostomy, midline strip of linea alba attached to xiphoid process was harvested and slinged around the gastroesophageal junction, along with right cervical esophagostomy. After radio-nuclear scan, the demonstration of abolition of gastroesophageal reflux, gastrostomy feed was started. The 2nd stage surgery performed after 6 weeks, included mobilization of esophagostomy, release of sling, thoracotomy, and tension-free esophageal anastomosis. Outcome measurement includes (1) prevention of air leak from esophagus into the stomach, (2) abolition of gastroesophageal reflux, (3) ability to start gastrostomy feeds, and (4) reversal of occlusion after release of the sling. RESULTS: The placement of linea alba sling and elevation of gastroesophageal junction, abolished air leak from fistula to stomach in all. Radio nuclear scan demonstrated abolition of gastroesophageal reflux in 6 with weight gain after gastrostomy feeding. One patient expired due to sepsis. One patient underwent final repair with reversal of occlusion with release of the sling. CONCLUSION: Using a sling of the linea alba around the cardioesophageal junction, prevents gastroesophageal reflux and escape of air from esophagus into the stomach, gives time to improve the respiratory and nutritional status of the patient, for a subsequent safer delayed primary anastomosis.

Editor's Choice - Outcomes After One Stage Versus Two Stage Open Repair of Type II Thoraco-abdominal Aortic Aneurysms.

OBJECTIVE: This study compared the outcomes of open one stage with open two stage repair of type II thoraco-abdominal aortic aneurysms (TAAA). METHODS: This retrospective study included 94 patients (68 men) with a mean ± SD age of 54.5 ± 14 years who underwent open type II TAAA repair from March 2006 to January 2016. The mean aneurysm diameter was 65 ± 14.4 mm. The median follow up was 42 months (range 12-96). Seventy-six patients received one stage open repair and 18 patients were treated in two steps: 12 received two open procedures (thoracic and abdominal) and six received hybrid repair (one open and one endovascular procedure). This study focused on the comparison of open one stage and open two stage TAAA repair. The median time between the two steps was 31.5 days (range 1-169). RESULTS: In hospital mortality after open one stage repair versus open two stage type II repair was 22.4% versus 0% (odds ratio 7.352, 95% confidence interval [CI] 0.884-959.1]; p = .19). The one year survival rate after one stage repair versus open two stage repair was 74.7% (95% CI 62.7-83.3) versus 90.9% (95% CI 50.8-98.7 [p = .225]). The five year survival rate after one stage repair versus open two stage repair was 53.0% (95% CI 37.2-66.5) versus 90.9% (95% CI 50.8-98.7 [p = .141]). The hazard ratio for survival after one stage repair and after open two stage repair was 4.563 (95% CI 96.9-81.4 [p = .137]). Paraplegia was observed after open one stage repair versus open two stage in 10.5% vs. 8% (p = 1). Acute kidney injury requiring permanent dialysis and myocardial infarction were assessed for after open one stage repair and open two stage and were seen in 3.9% vs. 0% (p = 1) and in 5.3% vs. 0% (p = 1), respectively. CONCLUSION: Open two stage repair may be recommended as a treatment option for type II TAAAs if anatomically feasible, as it has a lower mortality and similar complication rates to one stage repair.

Staged abdominal wall reconstruction in the setting of complex gastrointestinal reconstruction.

PURPOSE: Literature on one- versus two-staged abdominal wall reconstruction (AWR) with complex gastrointestinal reconstruction (GIR) is limited to single-arm case series with a focus on patients who complete all planned stages. Herein, we describe our experience with both one- and two-staged approaches to AWR/GIR, with attention to those who did not complete both intended stages. METHODS: A retrospective review of prospectively collected data was conducted to identify patients who underwent a one- or two-stage approach to GIR/AWR from 2013 to 2020. The one-stage approach included GIR and definitive sublay mesh herniorrhaphy. The two-stage approach included Stage 1 (S1)-GIR and non-definitive herniorrhaphy and Stage 2 (S2)-definitive sublay mesh herniorrhaphy. RESULTS: Fifty-four patients underwent GIR/AWR: 20 (37.0%) underwent a planned 1-stage operation while 34 (63.0%) underwent S1 of a planned 2-stage approach. Patients assigned to the 2-stage approach were more likely to be smokers, have a history of mesh infection, have an enterocutaneous fistula, and a contaminated wound class (p<0.05). Of the 34 patients who underwent S1, 12 (35.3%) completed S2 during the mean follow-up period of 44 months while 22 (64.7%) did not complete S2. Of these, 10 (45.5%) developed hernia recurrence but did not undergo S2 secondary to elective nonoperative management (40%), pending preoperative optimization (30%), additional complex GIR (10%), hernia-related incarceration requiring emergent surgery (10%), or unrelated death (10%). No differences in outcome including SSI, SSO, readmission, and recurrence were noted between the 12 patients who completed the two-stage approach and the 20 patients who completed a one-stage approach, despite increased risk factors for complications in the 2-stage group (p>0.05). CONCLUSION: Planned two-stage operations for GIR/AWR may distribute operative complexity and post-operative morbidity into separate surgical interventions. However, many patients may never undergo the intended definitive S2 herniorrhaphy. Future evaluation of 1- versus 2-stage GIR/AWR is needed to clarify indications for each approach. This work must also consider the frequent deviations from intended clinical course demonstrated in this study.

Cumulative Radiation Exposure and Lifetime Cancer Risk in Patients With Tetralogy of Fallot Requiring Early Intervention.

Background: Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention, utilizing either a staged repair (SR) or primary repair (PR) approach. They are exposed to several sources of low-dose ionizing radiation, which may contribute to increased cancer risk. Objectives: The purpose of this study was to compare cumulative radiation exposure and associated lifetime attributable risk (LAR) of cancer between treatment strategies in sTOF. Methods: Neonates with sTOF who underwent SR or PR from 2012 to 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. Radiation exposure from all radiologic studies prior to 18 months of age was converted to organ-equivalent doses and projected LAR of cancer incidence using the National Cancer Institute dosimetry tools. Results: There were 242 neonates from 8 centers, including patients with 146 SR and 96 PR. Cumulative total effective dose was significantly higher for SR (median 8.3 mSv, IQR: 3.0-17.4 mSv) than PR (2.1 mSv, IQR: 0.8-8.5 mSv; P < 0.001). Cumulative organ-level doses were significantly higher in SR compared to PR. Regardless of treatment strategy, LARs were higher in females compared to males. Among organs with median exposure >1 mGy in females, the LAR was highest for breast in SR (mean 1.9/1,000 patients). The highest proportion of cancers attributable to radiation exposure was projected for thyroid cancer in females undergoing SR (7.3%). Conclusions: Cumulative radiation exposure and LARs were higher among those undergoing SR compared to PR. This will be an important factor to consider in determining the preferred neonatal treatment strategy and should substantiate efforts to reduce radiation exposure in this vulnerable population.

Staged repair of a ruptured thoracoabdominal aortic aneurysm: a case report.

BACKGROUND: A ruptured thoracoabdominal aortic aneurysm (rTAAA) represents a considerable challenge for surgeons. To date, endovascular procedures have not been able to completely replace open repair when debranching is required. CASE PRESENTATION: A 73-year-old man was admitted to our hospital after complaining of left lateral abdominal pain. Enhanced computed tomography revealed a left retroperitoneal hematoma and a large, ruptured Crawford type IV TAAA. We first performed emergency resuscitative surgery to close the lacerated foramen. A graft replacement was performed 1 month after the initial surgery when the patient had stabilized. At 5 years postoperatively, neither occlusion nor anastomotic pseudoaneurysm was noted on computed tomography. CONCLUSIONS: We provide an update on the perioperative management of patients undergoing open rTAAA repair. This procedure can be considered to ensure complete repair of an rTAAA.

Staged repair for complete atrioventricular septal defect in patients weighing less than 4.0 kg.

OBJECTIVE: This study compared the mortality, left atrioventricular valve-related reoperation, and left atrioventricular valve competence in symptomatic neonates and small infants who underwent staged repair incorporating pulmonary artery banding or primary repair for complete atrioventricular septal defect. METHODS: Patients weighing less than 4.0 kg at the time of undergoing staged (n = 37) or primary (n = 23) repair for balanced complete atrioventricular septal defect between 1999 and 2022 were reviewed. The mean follow-up period was 9.1 years. Freedom from moderate or greater left atrioventricular valve regurgitation was estimated with the Kaplan-Meier method. RESULTS: The staged group included smaller children (median weight, 2.9 vs 3.7 kg) and a higher proportion of neonates (41% vs 4%). All patients in the staged group survived pulmonary artery banding and underwent intracardiac repair (median weight, 6.8 kg). After pulmonary artery banding, the severity of left atrioventricular valve regurgitation improved in 10 of 12 patients (83%) without left atrioventricular valve anomaly who had mild or greater left atrioventricular valve regurgitation and a left atrioventricular valve Z score greater than 0. Although survival and freedom from left atrioventricular valve-related reoperation at 15 years (P = .195 and .602, respectively) were comparable between the groups, freedom from moderate or greater left atrioventricular valve regurgitation at 15 years was higher in the staged group (P = .026). CONCLUSIONS: Compared with primary repair, staged repair for complete atrioventricular septal defect in children weighing less than 4.0 kg resulted in comparable survival and reoperation rates and better left atrioventricular valve competence. Pulmonary artery banding may mitigate secondary left atrioventricular valve regurgitation unless a structural valve abnormality exists. Selective deferred intracardiac repair beyond the neonatal and small-infancy period may still play an important role in low-weight patients.

Two big bones, one big decision: When to fix bilateral femur fractures.

PURPOSE: For polytrauma patients with bilateral femoral shaft fractures (BFSF), there is currently no consensus on the optimal timing of surgery. This study assesses the impact of early (≤ 24 h) versus delayed (>24 h) definitive fixation on clinical outcomes, especially focusing on concomitant versus staged repair. We hypothesized that early definitive fixation leads to lower mortality and morbidity rates. METHODS: The 2017-2020 Trauma Quality Improvement Program was used to identify patients aged ≥16 years with BFSF who underwent definitive fixation. Early definitive fixation (EDF) was defined as fixation of both femoral shaft fractures within 24 h, delayed definitive fixation (DDF) as fixation of both fractures after 24 h, and early staged fixation (ESF) as fixation of one femur within 24 h and the other femur after 24 h. Propensity score matching and multilevel mixed effects regression models were used to compare groups. RESULTS: 1,118 patients were included, of which 62.8% underwent EDF. Following propensity score matching, 279 balanced pairs were formed. EDF was associated with decreased overall morbidity (12.9% vs 22.6%, p = 0.003), lower rate of deep venous thrombosis (2.2% vs 6.5%, p = 0.012), a shorter ICU LOS (5 vs 7 days, p < 0.001) and a shorter hospital LOS (10 vs 15 days, p < 0.001). When compared to DDF, early staged fixation (ESF) was associated with lower rates of ventilator acquired pneumonia (0.0% vs 4.9%, p = 0.007), but a longer ICU LOS (8 vs 6 days, p = 0.004). Using regression analysis, every 24-hour delay to definitive fixation increased the odds of developing complications by 1.05, postoperative LOS by 10 h and total hospital LOS by 27 h. CONCLUSION: Early definitive fixation (≤ 24 h) is preferred over delayed definitive fixation (>24 h) for patients with bilateral femur shaft fractures when accounting for age, sex, injury characteristics, additional fractures and interventions, and hospital level. Although mortality does not differ, overall morbidity and deep venous thrombosis rates, and length of hospital and intensive care unit stay are significantly lower. When early definitive fixation is not possible, early staged repair seems preferable over delayed definitive fixation.

Primary repair vs Delayed staged repair in infants with primary obstructive megaureters and their long term outcomes - A single centre experience.

BACKGROUND: There is paucity of literature in the management of infants with primary obstructive megaureters undergoing upfront primary repair with an extravesical technique of ureteral re implantation (with or without ureteral tailoring). OBJECTIVE: To compare 2 different approaches in the management of infants with unilateral primary obstructive megaureters. STUDY DESIGN: This was a retrospective analysis of a prospectively maintained data base between 2005 and 2021. Infants <1 year with unilateral primary obstructive megaureter were included. They were divided into 2 groups: those who underwent an upfront extravesical ureteric reimplantation with or without ureteral tailoring during infancy -Primary Repair (PR), and those who initially underwent a low end cutaneous ureterostomy during infancy followed by take down of ureterostomy and intravesical ureteric reimplantation after 1 year of age -Delayed staged repair (DSR). Children presenting with sepsis, in whom a diversion was imperative, were excluded. All children were followed up annually after their definitive repair with a renal ultrasound, diuretic renogram, estimated glomerular filtration rate and assessment of voiding dysfunction if present. The 1st year and 3rd year follow up details were collated and analysed. Failure was defined as persistent obstructive pattern on renogram with worsening differential renal function or presence of high grade reflux with recurrent breakthrough urinary tract infection; both of which necessitated a redo reimplantation following the definitive surgery. RESULTS: There were 18 infants in Primary repair and 16 infants in Delayed Staged Repair. Urinary tract infections was the commonest presenting symptom amongst both groups i.e. > 50%.The post operative complication rate was 11% in Primary repair and 31% in Delayed Staged Repair. One child in each of the groups (2 girls) required redo reimplantation (5.8%). At the end of the 3rd year follow up (from the definitive repair) there was significant reduction in the hydronephrosis, improvement in the differential renal function with no evidence of obstruction and improvement in the estimated glomerular filtration rate amongst all in both groups which was statistically significant i.e. p < 0.05. The success rate was 94.4% in Primary Repair and 93.75% in Delayed Staged Repair. The mean follow up was 9.7 years amongst those undergone Primary Repair and 9 years amongst those undergone Delayed Staged Repair. DISCUSSION AND CONCLUSION: Primary extravesical ureteral reimplantation may be considered as the preferred line of management of unilateral obstructed megaureters during infancy.

Outcome analysis of staged preputial graft technique for primary proximal hypospadias with and without post-operative vacuum physiotherapy.

PURPOSE: Management of proximal hypospadias remains challenging. We assessed the results of staged preputial graft repairs (SPG) for proximal hypospadias and hypothesize that post-operative vacuum physiotherapy (VP) improves graft suppleness and overall outcomes. MATERIALS AND METHODS: Retrospective analysis of n = 71 patients with proximal hypospadias and severe ventral penile curvature (PC) of ≥50° after degloving. PC was corrected using ventral transverse incisions of the tunica albuginea (VTITA) without applying a tourniquet, taking care to avoid injuring the underlying erectile tissue. The ventral raw area at the penile shaft, including VTITA, were covered with either divided and partially mobilized urethral plate, or with the inner preputial graft itself. During the second stage, a tunica vaginalis flap was often used to cover the tubularized neourethra. Outcomes and post-op complications were assessed after each stage, comparing patients who received vacuum physiotherapy (VP+, n = 49) with those who did not (VP-, n = 22). RESULTS: Mean PC was 66°, average follow-up duration was 13.01 months, and overall complication rate was 22.5%. Only 6 of 49 VP + patients experienced complications (12.24%; 4 fistulas; 2 urethral strictures) and no recurrence of PC after second stage was observed in this group. VP- patients displayed a significantly higher rate of complications, with 10 of 22 cases (45.45%) exhibiting fistula development (n = 5) and glans dehiscence (n = 5). Recurrence of mild PC after first-stage repair was comparable between patient groups (12% VP+, 18% VP-) and easily corrected by simple graft tubularization or dorsal plication during second-stage repair. CONCLUSIONS: Staged repair using VTITA is effective for correcting proximal hypospadias with severe chordee. VP appears to promote and expedite graft suppleness and significantly improves patient outcomes.

Long-term outcomes of staged repair of tetralogy of Fallot.

BACKGROUND: The optimal management strategy for symptomatic young infants with tetralogy of Fallot (TOF) is yet to be determined. We aimed to evaluate the long-term outcomes of a staged approach with initial shunt palliation followed by complete repair. METHODS: Between January 1993 and July 2021, 160 children with TOF underwent a systemic-to-pulmonary shunt at our institution, including 65 neonates (41%). The mean duration of follow-up was 12.3 ± 8.1 years. RESULTS: Hospital mortality was 3% (4 of 160), all occurring in patients with a shunt size-to-weight ratio ≥1.2 mm/kg. Composite morbidity-defined as cardiac arrest, postoperative mechanical circulatory support, or unplanned reoperation-occurred in 21% (33 of 160). On multivariable analysis, a shunt size-to-weight ratio ≥1.2 mm/kg and prematurity were independent predictors of composite morbidity. Interstage mortality was 3% (4 of 156). A limited transannular patch was used in 75% (113 of 150) of TOF repairs. Actuarial survival at 20 years after shunt was 90% (95% confidence interval [CI], 79%-95%). Actuarial freedom from reinterventions at 20 years after TOF repair was 40% (95% CI, 28%-52%). Neonates had comparable composite morbidity, mortality, and late risk of reinterventions to older children. CONCLUSIONS: Staged repair of TOF in symptomatic young infants results in low mortality but high rates of reinterventions at long-term follow-up. A shunt size-to-weight ratio ≥1.2 mm/kg is a significant risk factor for mortality and morbidity prior to complete repair. Neonates undergoing shunt insertion have comparable outcomes to older children.

Comparison of treatment strategies for neonates with tetralogy of Fallot and pulmonary atresia.

OBJECTIVE: Neonates with tetralogy of Fallot and pulmonary atresia (TOF/PA) but no major aorta-pulmonary collaterals are dependent on the arterial duct for pulmonary blood flow and require early intervention, either by primary (PR) or staged repair (SR) with initial palliation (IP) followed by complete repair (CR). The optimal approach has not been established. METHODS: Neonates with TOF/PA who underwent PR or SR were retrospectively reviewed from the Congenital Cardiac Research Collaborative. Outcomes were compared between PR and SR (IP + CR) strategies. Propensity scoring was used to adjust for baseline differences. The primary outcome was mortality. Secondary outcomes included complications, length of stay, cardiopulmonary bypass and anesthesia times, reintervention (RI), and pulmonary artery (PA) growth. RESULTS: Of 282 neonates, 106 underwent PR and 176 underwent SR (IP: 144 surgical, 32 transcatheter). Patients who underwent SR were more likely to have DiGeorge syndrome and greater rates of mechanical ventilation before the initial intervention. Mortality was not significantly different. Duration of mechanical ventilation, inotrope use, and complication rates were similar. Cumulative length of stay, cardiopulmonary bypass, and anesthesia times favored PR (P ≤ .001). Early RI was more common in patients who underwent SR (rate ratio, 1.42; P = .003) but was similar after CR (P = .837). Conduit size at the time of CR was larger with SR. Right PA growth was greater with PR. CONCLUSIONS: In neonates with TOF/PA, SR is more common in greater-risk patients. Accounting for this, SR and PR strategies have similar mortality. Perioperative morbidities, RI, and right PA growth generally favor PR, whereas SR allows for larger initial conduit implantation.

The outcome and complications of modern staged repair surgery in newborns with classic bladder exstrophy in different genders: A retrospective study.

INTRODUCTION: Classic bladder exstrophy (CBE) is a rare anterior midline birth defect that remains a challenge for pediatric surgeons. Despite multiple reconstructive methods, outcomes vary widely in various reports. This study aims to compare the success rate and complications of modern staged repair of exstrophy (MSRE) in each gender and compare together. METHODS: This retrospective cross-sectional study included cases of CBE between 2010 and 2020 that underwent MSRE. Short-term follow-up results, including incontinence rate, vesicoureteral reflux (VUR), urinary infections, deformed genitalia, and so on, were measured in each gender, and their differences were reported. RESULTS: Among the 40 newborns with CBE who underwent MSRE, 25 (62.5%) were boys, while the others had non-male genitalia. The rates of incontinence, VUR, dehiscence, and fistulas did not differ significantly between genders. However, chronic urinary tract infections (UTIs) were more frequent in girls, and boys were more likely to have malformed genitalia (p < .05). CONCLUSION: Our findings indicate a similar rate of complications in each gender. However, chronic UTIs and external genitalia deformities were significantly more common in girls and boys, respectively. Further large-sized controlled trials may be needed to corroborate these findings.

Contemporary patterns of the management of truncus arteriosus (primary versus staged repair): outcomes from the Japanese National Cardiovascular Database.

OBJECTIVES: Although primary repair in early infancy has for decades been the prevalent strategy for management of truncus arteriosus (TA), recent concerns about the levels of morbidity and mortality have led to consideration of a staged surgical approach. Our goal was to describe recent patterns of management, to characterize patients who underwent primary or staged repair and to evaluate risk factors associated with operative mortality in a contemporary multicentre cohort. METHODS: In the Japanese Cardiovascular Surgery Database, we identified all cases of TA undergoing an initial surgical procedure from 2008 to 2018. Operative mortality was defined as death within 30 days of an operation or in-hospital death regardless of the length of hospital stay. The hospital volume was defined by the average volume of TA repairs per year. RESULTS: The total number of patients undergoing initial surgery for TA was 286. Sixty-eight (24%, 68/286) underwent primary repair (primary repair group). The remaining 218 (76%, 218/286) underwent initial bilateral pulmonary artery banding as part of a planned staged approach (staged repair group). One hundred sixty-two patients out of 218 initially banded patients underwent the repair of TA during this study period. Concomitant diagnoses in the entire cohort included interrupted aortic arch repair in 36 patients and truncal valve regurgitation in 32. No centres handling an average of ≥2 truncus cases/year of the repair of TA were identified in this cohort. A total of 30% (85/286) of the cases were performed at centres that handled an average of ≥1 and <2 cases/year. The remaining 70% were at centres with <1 case/year. Overall, 37 patients (12.9%; 37/286) died. The operative mortality rates in the primary and staged repair groups were similar: that for the primary repair group was 16.2% (11/68) versus 11.9% for the staged repair group (26/218; P = 0.41). With multivariable logistic regression analysis, the factors most strongly associated with operative mortality were preoperative heart failure requiring catecholamine support (odds ratio, 4.18; 95% confidence interval 1.96-8.96) and the repeat bilateral pulmonary artery banding (odds ratio, 3.89; 95% confidence interval 1.08-14.07). CONCLUSIONS: The staged repair of TA has emerged as the preferred option for surgical timing at most of the centres participating in the Japanese Cardiovascular Surgery Database. The management outcomes of the patients with TA were favourable, even for the patients at low-volume centres.