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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.
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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.
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Malformação Adenomatoide Cística Congênita do Pulmão , Blastoma Pulmonar , Criança , Humanos , Recém-Nascido , Lactente , Pré-Escolar , Adolescente , Estudos de Coortes , Estudos Retrospectivos , Blastoma Pulmonar/diagnóstico , Blastoma Pulmonar/genética , Blastoma Pulmonar/cirurgia , Malformação Adenomatoide Cística Congênita do Pulmão/diagnóstico por imagem , Malformação Adenomatoide Cística Congênita do Pulmão/genética , Malformação Adenomatoide Cística Congênita do Pulmão/cirurgia , DNA , Ribonuclease III/genética , RNA Helicases DEAD-box/genéticaRESUMO
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.
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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.
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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.
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COVID-19 , Quarentena , COVID-19/epidemiologia , COVID-19/prevenção & controle , Criança , Estudos Transversais , Feminino , Humanos , Itália/epidemiologia , Pais/psicologia , Quarentena/psicologia , SARS-CoV-2RESUMO
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.
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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.
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Neoplasias Pancreáticas , Procedimentos Cirúrgicos Robóticos , Adolescente , Adulto , Humanos , Masculino , Pancreatectomia/efeitos adversos , Fístula Pancreática/etiologia , Fístula Pancreática/prevenção & controle , Fístula Pancreática/cirurgia , Neoplasias Pancreáticas/patologia , Neoplasias Pancreáticas/cirurgia , Pancreaticojejunostomia/efeitos adversos , Estudos RetrospectivosRESUMO
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.
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Braquetes , Pectus Carinatum/terapia , Criança , Feminino , Humanos , Masculino , Pressão , Resultado do TratamentoRESUMO
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.
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Atresia Esofágica/cirurgia , Estenose Esofágica/etiologia , Complicações Pós-Operatórias , Anastomose Cirúrgica/efeitos adversos , Fístula Anastomótica/etiologia , Criança , Pré-Escolar , Dilatação/estatística & dados numéricos , Estenose Esofágica/terapia , Feminino , Humanos , Incidência , Lactente , Masculino , Estudos Retrospectivos , Fatores de RiscoRESUMO
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.
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Cisto do Colédoco/cirurgia , Adolescente , Anastomose em-Y de Roux/métodos , Procedimentos Cirúrgicos do Sistema Biliar/métodos , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Laparoscopia/métodos , Masculino , Complicações Pós-Operatórias/etiologia , Estudos RetrospectivosRESUMO
INTRODUCTION: Choledochal malformations (CMs) are increasingly diagnosed antenatally. There is a dilemma between early surgery to prevent CM-related symptoms and postponing surgery to reduce complications. We aimed to identify the optimal timing of surgery in asymptomatic neonates with antenatally diagnosed CM and to identify predictors for development of symptoms. METHODS: Using the Netherlands Study group on CHoledochal Cyst/malformation (NeSCHoc) we retrospectively collected demographic, biochemical and surgical data from all Dutch patients with an antenatally detected CM. RESULTS: Between 1989 and 2014, antenatally suspected CM was confirmed in 17 patients at a median age of 10days (1day-2months). Four patients developed symptoms directly after birth (24%). Thirteen patients (76%) remained asymptomatic. Two of these progressed to symptoms before surgical intervention at 0.7 and 2.1months resp. Postoperatively, four patients developed short-term complications and three developed long-term complications. Patients <5.6kg (the series median) showed more short-term complications (66%) when compared to patients >5.6kg (0%, p=0.02). CONCLUSION: When not symptomatic within the first days of life, the majority of children with antenatally detected CM remains asymptomatic. Surgery might safely be delayed to the age of 6months or a weight of 6kg. Postponing surgery in the clinically and biochemical asymptomatic patient might decrease the complication rate. LEVELS OF EVIDENCE: Level III.
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Cisto do Colédoco/cirurgia , Diagnóstico Pré-Natal , Adulto , Criança , Cisto do Colédoco/diagnóstico por imagem , Estudos de Coortes , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Países Baixos , Estudos Retrospectivos , Fatores de Tempo , Resultado do TratamentoRESUMO
An inguinal hernia is a protrusion of the intra-abdominal contents (in most cases the small bowel) via the inguinal canal towards the labium or the scrotum and is possible due to the fact that the processus vaginalis has not obliterated. It occurs in 1-2% of children. If surgical repair is not performed within a short period of time, the consequences can be far-reaching. Incarceration may occur, with the risk of ischaemia and necrosis. Morbidity and mortality due to inguinal hernias can be prevented by well-timed surgical interventions. The diagnosis can be made by the patient's history, and examination and palpation. Ultrasonography or other radiological tests are not indicated, although often performed. In this clinical lesson the Dutch guideline on inguinal hernias in children will be explicated with reference to three cases.
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Hérnia Inguinal/diagnóstico , Hérnia Inguinal/cirurgia , Feminino , Hérnia Inguinal/complicações , Humanos , Lactente , Recém-Nascido , Masculino , Palpação/métodos , Escroto , Resultado do TratamentoRESUMO
Gynaecomastia, breast enlargement in men, is common in all age groups. It is operated on by plastic surgeons, general surgeons and paediatric surgeons. It is therefore possible that there is a difference in the populations treated, the indications for surgery and the management used by the different practitioners. We performed a survey in order to assess the approach to treatment of gynaecomastia by the different disciplines. An electronic survey questionnaire was sent to members of the Dutch societies of surgery, paediatric surgery and plastic surgery. We received 105 responses from plastic surgeons, 95 from general surgeons and 15 from paediatric surgeons, representing respective response rates of 38.7%, 23.8% and 42.8%. Plastic surgeons operated on gynaecomastia most frequently. The diagnostic criteria and workup were similar for all disciplines, although general surgeons used more imaging. There was a difference in the side operated on. General surgeons and paediatric surgeons operated mainly on unilateral cases (74% and 52%), while plastic surgeons operated mainly on bilateral cases (85%). Pharmaceutical treatment with Tamoxifen was reported only by general surgeons (13%). All disciplines used mainly the periareolar incision. Plastic surgeons reported more often the use of other surgical approaches as well as adjunctive liposuction and they did not always submit tissue for pathological examination. Perioperative antibiotics, drains and pressure garments were not always used. All disciplines agreed that the most common complication was bleeding, followed by seroma, infection, insufficient results, inverted nipple and nipple necrosis. This survey highlights some differences in the practice of gynaecomastia surgery. The findings appear to point to the fact that the indications are different, being more aesthetic in the case of plastic surgeons. The results of this survey are important in establishing the standard of care and may be helpful for setting guidelines.
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Ginecomastia/cirurgia , Mamoplastia/métodos , Padrões de Prática Médica , Antibacterianos/uso terapêutico , Drenagem , Antagonistas de Estrogênios/uso terapêutico , Cirurgia Geral , Ginecomastia/diagnóstico , Ginecomastia/tratamento farmacológico , Humanos , Masculino , Mamoplastia/efeitos adversos , Países Baixos , Cirurgia Plástica , Inquéritos e Questionários , Tamoxifeno/uso terapêuticoRESUMO
BACKGROUND: The aim of this study is to evaluate if symptomatic or asymptomatic PVT, as diagnosed with ultrasonography (US), occurs more often in children after the introduction and implementation of LS compared to open splenectomy. METHODS: A retrospective cohort of 76 splenectomized patients for benign hematological disease were analyzed, 24 after open splenectomy (OS) and 52 after LS. RESULTS: In six of the OS and 40 after LS a postoperative US was obtained. In two patients after LS, a PVT was seen on US. Both patients were symptomatic and also underwent a laparoscopic cholecystectomy. The length of stay in the hospital was significantly shorter for LS (median 4.5 days, range 2-12) compared to OS (median 7 days, range 5-12), (P=.00). Median operation time of OS was 65 min (range 35-130 min) and of LS 170 min (range 85-275 min) (P=.00). There was no difference in postoperative complications. CONCLUSION: The risk of developing a PVT after laparoscopic splenectomy seems low, and thus LS is not contraindicated in patients with benign hematological disease. When combining LS and laparoscopic cholecystectomy, prophylactic heparin might be considered.