Pediatric thoracoscopic lung resections: a comprehensive analysis of congenital lesion cases.

BACKGROUND: Congenital lung lesions in pediatric patients may be managed conservatively or by video assisted thoracoscopic surgery (VATS). This study aimed to determine the complications after VATS for congenital lung lesions in children. METHODS: All children undergoing a lung resection between January 2009 and June 2022 were retrospectively identified. Children undergoing a primary open lobectomy or a resection other than a congenital lung lesion were excluded. Both early (<30 days) and late postoperative pulmonary complications were determined. The primary endpoint was postoperative complications within 30 days. RESULTS: In total, 56 patients were included, with a median age of 13 months (IQR 9-37). A VATS lobectomy were performed in 46 patients (82%), an extralobar sequestration in 8 patients (14%), an wedge resection in 1 patient and a segment resection in 1 patient. During the COVID pandemic, fewer resections were performed with an increase in symptomatic patients. A conversion to open occurred in 6 patients (11%), of which a preoperative lung infection was associated with an increased risk thereof(p = 0.004). The median follow-up was 22 months (IQR 7-57) and all patients were alive. A postoperative complication (Clavien Dindo ≥3) occurred in 9 patients and complications without the need of intervention in 6 patients. During follow-up a pneumonia occurred in 11 patients. CONCLUSION: There seems to be a shift towards delayed surgery with an increase in symptomatic congenital lung malformations, which might lead to an increase in postoperative complications.

Psychosocial Impact of 8 Weeks COVID-19 Quarantine on Italian Parents and their Children.

OBJECTIVES: Italy was affected greatly by Coronavirus disease 2019 (COVID-19), emerging mainly in the Italian province of Lombardy. This outbreak led to profound governmental interventions along with a strict quarantine. This quarantine may have psychosocial impact on children and parents in particular. The aim of this study was to evaluate the impact of 8 weeks COVID-19 quarantine on psychosocial functioning of Italian parents and their children. METHODS: In this cross-sectional survey, we included parents and children resided in Italy during the 8 weeks COVID-19 quarantine. We evaluated social and emotional functioning, clinical symptoms possibly related to emotional distress, and change in perspectives using a questionnaire. RESULTS: The majority of 2315 parents (98% mothers) frequently experienced fear of getting ill (92%) and fluctuating moods (84%), the latter showing correlation to experiencing stress due to being in continuous close vicinity to their children (77%, r = 0.33). Parents reported a positive effect on the relationship with their partner (79%) and their children (89%). Irritability in children was frequent (74%) and correlated to parental fluctuating moods (r = 0.40). The vast majority of the participants (91%) reported that their perspectives for the future had changed. CONCLUSIONS FOR PRACTICE: Our findings suggest a profound impact of the COVID-19 quarantine on emotional functioning of parents and their children in Italy. Despite the protective measure of quarantine against national viral spread and subsequent infection, health care professionals should be aware of this emotional impact, in order to develop protective or therapeutic interventions.

Quality of life and colorectal function in Crohn's disease patients that underwent ileocecal resection during childhood.

Psychosocial and functional outcomes after intestinal resection in pediatric Crohn's disease (CD) are lacking. Therefore, we (I) assessed health-related quality of life (HRQOL), colorectal function, and satisfaction with surgery and (II) investigated their relationship with surgical outcomes, after ileocecal resection for CD. Crohn's patients that underwent ileocecal resection during childhood were included. HRQOL and colorectal function were assessed using SF-36 and COREFO, respectively, and compared with reference values. Satisfaction was scored on a 5-point Likert scale. In total, 80 patients (50% male, median age 23.0 years) were included. Physical HRQOL was impaired (SF-36 [mean]: CD, 47 vs. general, 54; p < 0.001), while mental HRQOL was similar to that in the general population. Overall colorectal function was impaired (COREFO [mean]: CD, 12.6 vs. normal, 7.2; p < 0.001). Worse colorectal function was associated with increasing clinical disease activity and longer interval since resection. Majority of patients was satisfied with surgery (81% satisfied/very satisfied, 11% neither satisfied nor dissatisfied, 8% dissatisfied/very dissatisfied). Decreased satisfaction with surgery was associated with increased clinical disease activity but not related to colorectal function.Conclusions: Physical HRQOL and colorectal function in CD patients who underwent ileocecal resection during childhood seem impaired and related to adverse surgical outcomes. This emphasizes the need for post-operative monitoring and prophylactic therapies. What is Known: • Up to 25% of pediatric-onset Crohn's disease (CD) patients undergo an intestinal resection within 5 years from diagnosis. • Many children and adults with CD experience disruption of their daily activities and health-related quality of life (HRQOL). What is New: • Physical HRQOL and colorectal function are impaired in patient with CD that underwent ileocecal resection during childhood. • Increasing clinical disease activity, a longer interval since surgery, severe complications related to surgery, and recurrent surgeries are all associated with worse colorectal function.

Choledochal Malformation in Children: Lessons Learned from a Dutch National Study.

INTRODUCTION: A choledochal malformation (CM) is a rare entity, especially in the Western world. We aimed to determine the incidence of CM in the Netherlands and the outcome of surgery for CM in childhood. METHODS: All pediatric patients who underwent a surgical procedure for type I-IV CM between 1989 and 2014 were entered into the Netherlands Study group on choledochal cyst/malformation. Patients with type V CM were excluded from the present analysis. Symptoms, surgical details, short-term (<30 days) and long-term (>30 days) complications were studied retrospectively. RESULTS: Between January 1989 and December 2014, 91 pediatric patients underwent surgery for CM at a median age of 2.1 years (0.0-17.7 years). All patients underwent resection of the extrahepatic biliary tree with restoration of the continuity via Roux-en-Y hepaticojejunostomy. Twelve patients (12%) were operated laparoscopically. Short-term complications, mainly biliary leakage and cholangitis, occurred in 20 patients (22%), without significant correlations with weight or age at surgery or surgical approach. Long-term postoperative complications were mainly cholangitis (13%) and anastomotic stricture (4%). Eight patients (9%) required radiological intervention or additional surgery. Surgery before 1 year of age (OR 9.3) and laparoscopic surgery (OR 4.4) were associated with more postoperative long-term complications. We did not observe biliary malignancies during treatment or follow-up. CONCLUSION: Surgery for CM carries a significant short- and long-term morbidity. Given the low incidence, we would suggest that (laparoscopic) hepatobiliary surgery for CM should be performed in specialized pediatric surgical centers with a wide experience in laparoscopy and hepatobiliary surgery.

Erratum: Missed Proximal Tracheoesophageal Fistula (TEF) in a Neonate with Type D Esophageal Atresia.

[This corrects the article DOI: 10.1055/a-2227-6389.].

Missed Proximal Tracheoesophageal Fistula (TEF) in a Neonate with Type D Esophageal Atresia.

We present the case of a patient with the rare type D esophageal atresia (EA), diagnosed after correction of an EA initially diagnosed as type C. Routine postoperative contrast esophagogram showed a missed proximal tracheoesophageal fistula. This case report illustrates the potential difficulties to diagnose type D EA.

Intercostal Nerve Cryoablation or Epidural Analgesia for Multimodal Pain Management after the Nuss Procedure: A Cohort Study.

BACKGROUND: Nuss procedure for pectus excavatum is a minimally invasive, but painful procedure. Recently, intercostal nerve cryoablation has been introduced as a pain management technique. MATERIALS AND METHODS: In this cohort study, we compared the efficacy of multimodal pain management strategies in children undergoing a Nuss procedure. The effectiveness of intercostal nerve cryoablation combined with patient-controlled systemic opioid analgesia (PCA) was compared with continuous epidural analgesia (CEA) combined with PCA. The study was conducted between January 2019 and July 2022. Primary outcome was length of stay (LOS), and secondary outcomes were operation room time, postoperative pain, opioid consumption, and gabapentin use. RESULTS: Sixty-six consecutive patients were included, 33 patients in each group. The cryoablation group exhibited lower Numeric Rating Scale (NRS) pain scores on postoperative day 1 and 2 (p = 0.002, p = 0.001) and a shorter LOS (3 vs. 6 days (p < 0.001). Cryoablation resulted in less patients requiring opioids at discharge (30.3 vs. 97.0%; p < 0.001) and 1 week after surgery (6.1 vs. 45.4%; p < 0.001)). In the CEA group, gabapentin use was more prevalent (78.8 vs. 18.2%; p < 0.001) and the operation room time was shorter (119.4 vs. 135.0 minutes; p < .010). No neuropathic pain was reported. CONCLUSIONS: Intercostal nerve cryoablation is a superior analgesic method compared with CEA, with reduced LOS, opioid use, and NRS pain scores. The prophylactic use of gabapentin is redundant.

No Pathogenic DICER1 Gene Variants in a Cohort Study of 28 Children With Congenital Pulmonary Airway Malformation.

BACKGROUND: Distinguishing congenital pulmonary airway malformations (CPAMs) from pleuropulmonary blastoma (PPB) can be challenging. Previously diagnosed patients with CPAM may have been misdiagnosed and we may have missed DICER1-associated PPBs, a diagnosis with important clinical implications for patients and their families. To gain insight in potential misdiagnoses, we systematically assessed somatic DICER1 gene mutation status in an unselected, retrospective cohort of patients with a CPAM diagnosis. METHODS: In the Amsterdam University Medical Center (the Netherlands), it has been standard policy to resect CPAM lesions. We included all consecutive cases of children (age 0-18 years) with a diagnosis of CPAM between 2007 and 2017 at this center. Clinical and radiographic features were reviewed, and DICER1 gene sequencing was performed on DNA retrieved from CPAM tissue samples. RESULTS: Twenty-eight patients with a surgically removed CPAM were included. CPAM type 1 and type 2 were the most common subtypes (n = 12 and n = 13). For 21 patients a chest CT scan was available for reassessment by two pediatric radiologists. In 9 patients (9/21, 43%) the CPAM subtype scored by the radiologists did not correspond with the subtype given at pathology assessment. No pathogenic mutations and no copy number variations of the DICER1 gene were found in the DNA extracted from CPAM tissue (0/28). CONCLUSIONS: Our findings suggest that the initial CPAM diagnoses were correct. These findings should be validated through larger studies to draw conclusions regarding whether systematic DICER1 genetic testing is required in children with a pathological confirmed diagnosis of CPAM or not. LEVEL OF EVIDENCE: Level IV.

Laparoscopic pyloromyotomy, the tail of the learning curve.

BACKGROUND: The debate whether laparoscopic pyloromyotomy (LP) is superior to open pyloromyotomy (OP) remains actual. A recent review showed no benefit between the LP or OP, but in the studied randomized, controlled trials the learning curve was not addressed. Comparing LP including the learning curve with OP after the learning curve is questionable. In previous research, the learning curve of LP was analyzed. It was concluded that the plateau was reached after 35 procedures with a steep decrease in complications when comparing before and after 35 procedures. This study was designed to retrospectively analyze the results obtained in HPS patients after the learning curve in LP has been reached. The results will be compared with results of OP in HPS patients in the same period. METHODS: A retrospective analysis in 106 OP and 57 LP was performed from September 2008 to June 2012. RESULTS: The overall complication rate in the OP group was significantly higher than in the LP group (18% vs. 3.5%, p = 0.012). Also there was a higher major complication rate in the OP group (10.4% vs. 1.7%, p = 0.045). The median time to operate was with 28.5 min in OP and 30.0 min in LP (not significant), whereas the LOS was 2 days in both groups (not significant). CONCLUSION: In this study, a further decline in overall and major complications after the learning curve is seen in the LP group, the tail of the learning curve. The debate whether LP is superior to OP is not finished as long as it is not clear whether the minimally invasive operation is beyond the initial or tail of the learning curve.

Dynamic Compression Therapy for Pectus Carinatum in Children and Adolescents: Factors for Success.

BACKGROUND: Pectus carinatum (PC) is a congenital chest wall deformity. In childhood, it is increasingly treated with dynamic compression therapy. Factors for success for dynamic brace therapy are relatively unknown. METHODS: Between 2013 and 2020, 740 patients treated with the Dynamic Compression System (DCS), were studied. This included the effect of age, gender, pectus height, symmetry and pectus rigidity on treatment time and symptoms with linear multiple regression analyses. RESULTS: Carinatum height and high pressure of initial correction at the start of treatment were associated with a prolonged duration of treatment. For each cm increase in carinatum height, the total treatment duration increased with 1.9 months (p-value= 0.002, 95% CI: 0.70-3.13). An initial correction pressure of ≥7.6 pounds per square inch (psi), increased the treatment duration with 3.5 months (p-value 0.006, 95% CI: 1.04-6.01) compared to an initial correction pressure of ≤5.0 psi. A high initial pressure of correction of ≥7.6 psi increased the odds of having somatic symptoms with 1.19 (p-value= 0.012, 95% CI: 1.04-1.45) and psychosocial symptoms with 1.13 (p-value= 0.04, 95% CI: 1.01-1.27) compared to a low initial pressure of correction of ≤5.0 psi. An initial pressure of correction of 5.1-7.5 psi increased the odds of having somatic symptoms with 1.14 (p-value 0.046, 95% CI: 1.00-1.29) compared to an initial pressure of correction of ≤5.0 psi. Patients with asymmetric chests were more likely to abandon therapy CONCLUSIONS: High carinatum height and high initial pressure of correction are associated with prolonged bracing treatment and a higher failure rate. LEVEL OF EVIDENCE: III.

Clinical significance of concomitant pectus deformity and adolescent idiopathic scoliosis: systematic review with best evidence synthesis.

Background: A misbalance in forces is proposed for causing adolescent idiopathic scoliosis (AIS). AIS is therefore correlated to adjacent musculoskeletal pathologies. Its concomitance with idiopathic pectus deformities (PD) is underexposed. This systematic review analyzes the clinical significance and predictive factors of PD-associated AIS. Methods: A search was performed in PubMed, UpToDate, Embase, and Cochrane. A study was included if it: assessed the association between PD and scoliosis (category I), reported a prevalence of scoliosis in PD patients (category II), or addressed other topics about PD-associated AIS (category III). Studies in category I discussing predictive factors were appraised using the Quality in Prognosis Studies tool. Because of heterogeneity among the studies, predictive factors were analyzed according to a best evidence synthesis. A mean prevalence of scoliosis in PD patients was calculated using category I and II. Category III was narratively reviewed. Results: Forty-eight studies were included (I:19, II:21, III:8). Category I comprised 512 patients with PD-concomitant scoliosis. Thirteen studies reported predictive factors, of which 15 concerned the prevalence of scoliosis in PD patients and 12 Cobb Angle (CA) change after PD correction. Compared with AIS, PD seems to develop earlier in adolescence, and PD with concomitant AIS was more frequently reported in older patients. Evidence remained conflicting regarding the association between the severity of PD and that of scoliosis. As opposed to at a younger age, late PD correction is not associated with a postoperative increase of CA. Limited evidence showed that patients with a high CA undergoing PD correction do not experience an increase in CA, though, strong evidence indicated that it would not lead to a decrease in CA. The mean probable prevalence of AIS in PD patients was 13.1%. Conclusion: Current literature confirms the association between PD and AIS in patients with an indication for PD correction.Level of evidence: III.

Ravitch Surgery or Dynamic Compression Bracing for Pectus Carinatum: A Retrospective Cohort Study.

BACKGROUND: Pectus carinatum is a pediatric condition that can be treated by dynamic compression system (DCS) bracing or surgery. Several publications on DCS bracing or surgery are available; however, they do not compare both treatments. METHODS: Over a 10-year period, 738 patients with pectus carinatum were treated at the Amsterdam Pectus Center (Amsterdam, The Netherlands). This study describes this 10-year experience and the results of both treatments. RESULTS: Of the 631 patients who underwent DCS bracing treatment, 553 finished treatment, and 78 patients are still under treatment. A total of 73.8% (n = 408) of these patients finished treatment successfully, 13.6% (n = 75) experienced treatment failure, and 12.7% (n = 70) were lost to follow-up. The success rate decreased with an increasing pressure of initial compression (84.2%-67.3%). Marfan syndrome and Poland syndrome were associated with unfavorable results. Ravitch surgery was performed in 105 patients, with a success rate of 92.4%. Complications occurred in 32.4% of patients, and 6.7% of patients had complications for which surgery was needed. No relationship was found between osteotomy or sternal fixation and outcomes or complications. The Abramson procedure was performed in 2 patients. CONCLUSIONS: DCS bracing should be the treatment of choice in patients with pectus carinatum because of its noninvasiveness, good results, and lower complication rate compared with surgery. Besides pressure of initial correction, motivation is an important factor influencing outcomes, and compliance remains a major challenge in treating pectus carinatum using DCS bracing. Bracing patients before their growth spurt should be discouraged. Patients with a higher pressure of initial compression (>8.0-8.5 psi) and Marfan syndrome or Poland syndrome have poorer outcomes. In those patients, surgery may be considered.

Volvulus as a complication of chronic intestinal pseudo-obstruction syndrome.

UNLABELLED: Chronic intestinal pseudo-obstruction syndrome (CIPS) is a severe motility disorder of the gastrointestinal tract that presents with continuous or recurrent symptoms and signs of intestinal obstruction without evidence of a structural lesion occluding the intestinal lumen. Mechanical obstruction might occur in these patients as well but is typically difficult to distinguish from an exacerbation of CIPS. We report two pediatric cases in which mechanical obstruction by volvulus mimicked an exacerbation of CIPS, requiring surgical intervention. CONCLUSION: Awareness of the possibility of true mechanical obstruction in CIPS patients during an exacerbation episode is needed, as this is a severe condition and usually requires surgical intervention.

A modified surgical technique for aortopexy in tracheobronchomalacia.

OBJECTIVES: Tracheobronchomalacia (TBM) is characterized by collapse of trachea, bronchi or both, leading to dyspnoea, expiratory stridor, coughing or recurrent airway infections. Surgical treatment with aortopexy is warranted for severe TBM. We describe a modified aortopexy technique with aortic wall strap sutures that evenly distributes the traction force over the full width of the aortic arch. The aim of this study was to determine the outcomes of this modified anterior aortopexy technique. METHODS: Retrospective chart review of all patients undergoing aortopexy with aortic wall strap sutures for TBM between January 2010 and June 2020 in 2 tertiary hospitals in the Netherlands. RESULTS: Twenty-four patients [median age 9 months (interquartile range 2-117 months); 71% male] underwent aortopexy with the modified technique for TBM (52%), tracheomalacia (40%) or bonchomalacia (8%). Aortopexy was successful in 91.7%, defined as relief or decrease of respiratory symptoms and no need for respiratory support. Complications occurred in 8.3% and mortality was 4%. CONCLUSIONS: Aortopexy with non-absorbable strap sutures seems an effective and safe treatment for severe TBM. This study supports the hypothesis that strap sutures provide a solid and reliable traction force, but future comparative studies should confirm the benefit of strap sutures over conventional techniques.

Robotic Central Pancreatectomy with Roux-en-Y Pancreaticojejunostomy.

Central pancreatectomy is a parenchyma-sparing alternative to distal pancreatectomy in patients with a benign or low-grade malignant tumor in the body of the pancreas. The aim of central pancreatectomy is to prevent postoperative life-long endocrine and exocrine insufficiency. The downside of central pancreatectomy is the high rate of postoperative pancreatic fistula, which is the main reason that many surgeons do not routinely use central pancreatectomy in eligible patients. Most studies report open or laparoscopic central pancreatectomy with a pancreatico-gastrostomy anastomosis in adults. This is the first description of a standardized approach to robotic central pancreatectomy with Roux-en-Y pancreaticojejunostomy reconstruction in an adolescent (16-year-old boy) with a pseudopapillary tumor in the body of the pancreas. The operation time was 248 min with 20 mL of blood loss. The postoperative course was uneventful except for the short-term medical treatment for a grade B pancreatic fistula. Robotic central pancreatectomy can be safely applied in selected patients in experienced centers.

Pasireotide treatment for severe congenital hyperinsulinism due to a homozygous ABCC8 mutation.

ABCC8 and KCJN11 mutations cause the most severe diazoxide-resistant forms of congenital hyperinsulinism (CHI). Somatostatin analogues are considered as secondline treatment in diazoxide-unresponsive cases. Current treatment protocols include the first-generation somatostatin analogue octreotide, although pasireotide, a second-generation somatostatin analogue, might be more effective in reducing insulin secretion. Herein we report the first off-label use of pasireotide in a boy with a severe therapy-resistant form of CHI due to a homozygous ABCC8 mutation. After partial pancreatectomy, hyperinsulinism persisted; in an attempt to prevent further surgery, off-label treatment with pasireotide was initiated. Short-acting pasireotide treatment caused high blood glucose level shortly after injection. Long-acting pasireotide treatment resulted in more stable glycemic control. No side effects (e.g., central adrenal insufficiency) were noticed during a 2-month treatment period. Because of recurrent hypoglycemia despite a rather high carbohydrate intake, the boy underwent near-total pancreatectomy at the age of 11 months. In conclusion, pasireotide treatment slightly improved glycemic control without side effects in a boy with severe CHI. However, the effect of pasireotide was not sufficient to prevent near-total pancreatectomy in this case of severe CHI.

[Dynamic compression brace for pectus carinatum: 5 years on]. / Dynamische-compressiebrace bij pectus carinatum.

Pectus carinatum is a congenital chest-wall deformity with a prevalence of 0.3-0.7%. This chest-wall deformity can lead to functional, cosmetic and psychosocial problems. For many years, the only available treatment was an invasive surgical procedure. A less-invasive treatment, namely external compression of the sternum with a brace, showed disappointing results due to discomfort, a high percentage of skin complications and low compliance. The introduction of the dynamic compression brace has meant that the pressure of sternal compression can be measured and adjusted, which has improved comfort and compliance and resulted in an increased success rate of 92% and a decrease in the percentage of skin complications to just 8%. Despite the fact that wearing a compression brace for a longer period of time is not an easy undertaking for an adolescent, the use of the dynamic compression brace results in a high success rate, and is a safe and an efficient treatment.

Risk factors for refractory anastomotic strictures after oesophageal atresia repair: a multicentre study.

OBJECTIVE: To determine the incidence of refractory anastomotic strictures after oesophageal atresia (OA) repair and to identify risk factors associated with refractory strictures. METHODS: Retrospective national multicentre study in patients with OA born between 1999 and 2013. Exclusion criteria were isolated fistula, inability to obtain oesophageal continuity, death prior to discharge and follow-up <6 months. A refractory oesophageal stricture was defined as an anastomotic stricture requiring ≥5 dilations at maximally 4-week intervals. Risk factors for development of refractory anastomotic strictures after OA repair were identified with multivariable logistic regression analysis. RESULTS: We included 454 children (61% male, 7% isolated OA (Gross type A)). End-to-end anastomosis was performed in 436 (96%) children. Anastomotic leakage occurred in 13%. Fifty-eight per cent of children with an end-to-end anastomosis developed an anastomotic stricture, requiring a median of 3 (range 1-34) dilations. Refractory strictures were found in 32/436 (7%) children and required a median of 10 (range 5-34) dilations. Isolated OA (OR 5.7; p=0.012), anastomotic leakage (OR 5.0; p=0.001) and the need for oesophageal dilation ≤28 days after anastomosis (OR 15.9; p<0.001) were risk factors for development of a refractory stricture. CONCLUSIONS: The incidence of refractory strictures of the end-to-end anastomosis in children treated for OA was 7%. Risk factors were isolated OA, anastomotic leakage and the need for oesophageal dilation less than 1 month after OA repair.

Two Cases of Hepatoblastoma in Young Adults.

Adult hepatoblastoma (AHB) is a rare liver tumor with a poor prognosis in adolescents and adults. This contrasts with hepatoblastoma in children and is not fully understood. Here we describe two adolescents with AHB who were treated in our hospital. Adolescents are likely to receive less intensive chemotherapy protocols and are treated in hospitals with less experience in pediatric oncology, resulting in poor outcome. More research is necessary for optimal treatment of AHB in adolescents. Adolescents with AHB should be referred to hospitals experienced in pediatric oncology and receive intensive chemotherapy, followed by hemihepatectomy.

Diagnosis and treatment of cutaneous zygomycosis.

Cutaneous zygomycosis is a rare but severe fungal infection with high risk of dissemination. Early recognition, deep surgical biopsy for diagnosis, aggressive treatment with repeated surgical debridement, and targeted pharmacotherapy are essential and can prevent dissemination and fatal outcome. We present case reports of 2 patients.