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PURPOSE: Recently, children with trisomy 18 have been receiving more active treatment for malignancies. We report herein seven cases complete resection was achieved, and discuss multidisciplinary treatment for hepatoblastoma in patients with trisomy 18. METHOD: The medical records of children with trisomy 18 who were treated at the study center between 2010 and 2023 were reviewed. RESULT: Six of 69 patients had hepatoblastoma development, and three of these underwent multidisciplinary treatment. In addition, 6 patients had been referred by another hospital for treatment, and four of these underwent multidisciplinary treatment. Among the seven patients who underwent multidisciplinary treatment, three, two, and two were categorized in Pre-treatment Extent of Disease (PRETEXT) classification group I, II, and III, respectively. Neoadjuvant chemotherapy resulting in tumor reduction was performed in three cases. In all the cases, complete resection was achieved with pathologically safe margins. Perioperative complications included circulatory failure in one case and bile leakage in two cases. Adjuvant chemotherapy was administered in four cases. The postoperative observation period ranged from 3 months to 11 years, and all the patients are recurrence-free. CONCLUSION: Children with trisomy 18 complicated with hepatoblastoma whose cardiopulmonary conditions are stable may be good candidates for chemotherapy and surgery.
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Hepatoblastoma , Neoplasias Hepáticas , Síndrome de la Trisomía 18 , Humanos , Hepatoblastoma/genética , Hepatoblastoma/cirugía , Síndrome de la Trisomía 18/complicaciones , Síndrome de la Trisomía 18/genética , Síndrome de la Trisomía 18/cirugía , Masculino , Neoplasias Hepáticas/cirugía , Neoplasias Hepáticas/genética , Femenino , Lactante , Preescolar , Estudios Retrospectivos , Hepatectomía/métodos , Niño , Resultado del Tratamiento , Trisomía/genéticaRESUMEN
PURPOSE: Long-gap esophageal atresia (LGEA) is still a challenge for pediatric surgery. No consensus exists as to what constitutes a long gap, and few studies have investigated the maximum gap length safely repairable by primary anastomosis. Based on surgical outcomes at a single institution, we aimed to determine the gap length in LGEA with a high risk of complications. METHODS: The medical records of 51, consecutive patients with esophageal atresia (EA) with primary repair in the early neonatal period between 2001 and 2021 were retrospectively reviewed. Three, major complications were found in the surgical outcomes: (1) anastomotic leakage, (2) esophageal stricture requiring dilatation, and (3) GERD requiring fundoplication. The predictive power of the postsurgical complications was assessed using receiver operating characteristic analysis, and the area under the curve (AUC) and the cutoff value with a specificity of > 90% were calculated. RESULTS: Sixteen patients (31.4%) experienced a complication. The AUC of gap length was0.90 (p < 0.001), and the gap length cutoff value was ≥ 2.0 cm for predicting any complication (sensitivity: 62.5%, specificity: 91.4%). CONCLUSION: A gap length ≥ 2.0 cm was considered as defining LGEA and was associated with an extremely high complication rate after primary repair.
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Atresia Esofágica , Estenosis Esofágica , Fístula Traqueoesofágica , Recién Nacido , Niño , Humanos , Atresia Esofágica/cirugía , Atresia Esofágica/complicaciones , Estudios Retrospectivos , Resultado del Tratamiento , Estenosis Esofágica/etiología , Fístula Traqueoesofágica/cirugía , Fístula Traqueoesofágica/complicaciones , Anastomosis Quirúrgica/efectos adversosRESUMEN
OBJECTIVE: To identify patients with biliary atresia (BA) with extremely poor outcomes of bile drainage surgery using the infant BA liver fibrosis (iBALF) score, a liver fibrosis marker based on standard blood analysis. BACKGROUND: Although primary liver transplantation is beginning to be considered as an alternative to bile drainage surgery in patients with BA, those most likely to benefit from this procedure have not yet been identified. METHODS: The medical records of 380 patients with BA with bile drainage surgery between 2015 and 2019 were collected for retrospective analysis from 60 participating hospitals. To predict native liver survival at age 1 year, a receiver operating characteristic curve was drawn for the iBALF score. The cutoff value was determined as the point indicating >99% sensitivity. RESULTS: The median age at surgery was 56 days (range: 4-183 days), and native liver survival at age 1 year was achieved in 258 (67.9%) patients. An iBALF score of 5.27 was chosen as the cutoff, and 18 patients (4.7%) were found to have an iBALF score >5.27; of these, only 2 (95% CI: 1.4%-34.7%) had native liver survival at age 1 year, indicating a significantly poorer outcome than in the other patients (95% CI: 65.7%-75.4%). Moreover, patients with an iBALF score >5.27 had significantly higher mortality and younger age at salvage liver transplantation. CONCLUSIONS: Patients with BA having a preoperative iBALF score >5.27 had extremely poor outcomes of bile drainage surgery and may be considered candidates for primary LTx.
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Atresia Biliar , Lactante , Humanos , Atresia Biliar/cirugía , Atresia Biliar/etiología , Estudios Retrospectivos , Portoenterostomía Hepática/efectos adversos , Portoenterostomía Hepática/métodos , Japón , Bilis , Cirrosis Hepática/cirugía , Cirrosis Hepática/etiología , DrenajeRESUMEN
PURPOSE: The transumbilical approach is widely used for minimally invasive surgery in children. We compared herein the postoperative cosmesis between two types of transumbilical approach: a vertical incision versus periumbilical incision. METHODS: Patients with a transumbilical laparotomy before age one year were prospectively enrolled between January 2018 and December 2020. A vertical incision or periumbilical incision was chosen at the surgeon's discretion. After excluding patients receiving a relaparotomy via another site, a questionnaire about the appearance of the umbilicus was completed by the patients' guardians at postoperative month 6 to assess satisfaction and determine the visual analog scale score. A photograph of the umbilicus was taken while the questionnaire was being administered for later assessment by surgeons blinded to the scar and umbilical shape. RESULTS: Forty patients were enrolled; 24 patients received a vertical incision while 16 received a periumbilical incision. The incision length was significantly shorter in the vertical incision group (median: 2.0; range: 1.5-3.0 cm vs. median: 2.75; range: 1.5-3.6 cm) (p = 0.001). The patients' guardians reported significantly higher satisfaction (p = 0.002) and higher scores on the visual analog scale (p = 0.046) in the vertical incision group (n = 22) than in the periumbilical incision group (n = 15). The surgeons' evaluation was associated with significantly more patients with a vertical incision than with a periumbilical incision achieving a cosmetically preferable outcome, including an invisible or fine scar and a normal umbilical shape. CONCLUSION: A vertical umbilical incision can provide better postoperative cosmesis than a periumbilical incision.
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BACKGROUND: The effects of disease classification and the patient's preoperative condition on the difficulty of performing a laparotomy for pediatric congenital biliary dilatation (CBD) have not been fully elucidated. METHODS: The present study retrospectively analyzed 46 pediatric CBD laparotomies performed at the study center between March 2010 and December 2021 and predictors of operative time. The patients were separated into a short operative time group (SOT) (≤ 360 min, n = 27) and a long operative time group (LOT) (> 360 min, n = 19). RESULTS: The preoperative AST and ALT values were higher, and the bile duct anastomosis diameter was larger, in the LOT. Correlation analysis demonstrated that the maximum cyst diameter, preoperative neutrophil-to-lymphocyte ratio, AST, ALT, AMY, and bile duct anastomosis diameter correlated positively with operative time. Multivariate analysis identified the maximal cyst diameter, preoperative AST, and bile duct anastomosis diameter as significant factors affecting surgical time. Postoperatively, intrapancreatic stones and paralytic ileus were observed in one patient each in the SOT, and mild bile leakage was observed in one patient in the LOT. CONCLUSIONS: The maximum cyst diameter, preoperative AST, and bile duct anastomosis diameter have the potential to predict the difficulty of performing a pediatric CBD laparotomy.
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Enfermedades de las Vías Biliares , Procedimientos Quirúrgicos del Sistema Biliar , Quiste del Colédoco , Humanos , Niño , Quiste del Colédoco/cirugía , Estudios Retrospectivos , Enfermedades de las Vías Biliares/cirugía , Laparotomía , Dilatación Patológica/cirugíaRESUMEN
PURPOSE: The present study aimed to review the treatment experience and outcomes of Currarino syndrome (CS) complicated with anorectal stenosis to evaluate the current treatment strategies. METHODS: Seven cases of CS complicated with anorectal stenosis, treated at our hospital between 1998 and 2021, were retrospectively investigated. This is a case series article from a single institution. RESULTS: In six and three cases and one case, the presacral mass was a mature teratoma, meningocele, and lipoma, respectively. Resection of the lesion was performed in all six cases of mature teratoma, and duraplasty was performed before resection in all three cases of meningocele. Moreover, surgery for anorectal stenosis was performed simultaneously in four patients. Surgery was performed for six cases of anorectal stenosis, with the remaining case relieved by dilation using a metal bougie. The surgical methods used were a partial resection with end-to-end anastomosis, anorectal strictureplasty, pull-through, posterior sagittal anorectoplasty, and cutback after mass resection. Pathological analysis of the anorectal stenoses revealed disorganized and rough smooth muscle fibers and the replacement of the stroma by an increased quantity of collagen fibers. CONCLUSIONS: The clinical outcomes of CS can be improved by establishing a treatment flow chart and understanding the complicated pathophysiology of the disease.
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PURPOSE: The slide tracheoplasty (STP) is the standard treatment for severe congenital tracheal stenosis (CTS). Understanding the features of the tracheal stenosis in each case and choosing an appropriate incision design are very important for successfully executing the procedure. The present study aimed to evaluate the advantages of three-dimensional (3D) printed models of the trachea for improving CTS. METHODS: Three-D tracheal models were created using computed tomography (CT) data from ten patients undergoing STP for CTS. Simulated surgery was performed using the hollow models after reinforcing with them with a coating of gum spray. Clinical outcomes, including patient survival, postoperative surgical interventions, and time required for STP, were compared with the corresponding values in the last ten patients before the introduction of 3D model simulations. RESULTS: All ten patients for whom simulated surgery using a 3D tracheal model were conducted achieved good airway patency after their STP. The surgeons reported feeling that the 3D model simulations were highly effective although there was no significant difference in the clinical outcomes of the groups with or without simulated STP. The models were useful not only for surgical planning but also for sharing important information among the multidisciplinary team and the patients' family. CONCLUSION: Our experience using 3D tracheal models demonstrated several features enabling improvement in the surgical treatment of CTS.
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Procedimientos de Cirugía Plástica , Tráquea , Humanos , Lactante , Tráquea/diagnóstico por imagen , Tráquea/cirugía , Tráquea/anomalías , Estudios Retrospectivos , Resultado del Tratamiento , Procedimientos de Cirugía Plástica/métodosRESUMEN
PURPOSE: Radical surgery for congenital tracheal stenosis (CTS) is technically demanding. CTS combined with tracheal bronchus (TB) and pulmonary artery (PA) sling is a particularly challenging condition. We herein report our successfully modified surgical techniques for CTS combined with TB and PA sling. METHODS: Nine patients treated at our institution from July 2010 to December 2020 for CTS with TB and PA sling were enrolled. The patients' characteristics, operative results, and clinical outcomes were reviewed and analyzed retrospectively. RESULTS: The mean age at the operation and body weight were 8.0 ± 4.4 months old and 6.5 ± 0.8 kg, respectively. The mean tracheal diameter and length of the stenotic lesion were 3.2 ± 1.0 mm (mean stenosis rate 46.2%) and 25.4 ± 4.9 mm, respectively. All cases were complicated with PA sling at bifurcation stenosis with tracheobronchomalacia. All patients underwent modified posterior-anterior slide tracheoplasty with an inverted Y-shaped incision at the bifurcation and repositioning of the PA. The mean postoperative intubation period was 25.0 ± 32.1 days. There were no major intraoperative or postoperative complications, including hypoxic-ischemic encephalopathy. The mean hospital stay was 92.2 ± 73.4 days. All patients were discharged home without tracheostomy or oxygen support. CONCLUSION: Our slide tracheoplasty technique for CTS with TB and PA sling achieved excellent outcomes. LEVEL OF EVIDENCE: Level IV.
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Cardiopatías Congénitas , Procedimientos de Cirugía Plástica , Anomalías del Sistema Respiratorio , Estenosis Traqueal , Bronquios/anomalías , Bronquios/cirugía , Constricción Patológica/etiología , Constricción Patológica/cirugía , Cardiopatías Congénitas/cirugía , Humanos , Lactante , Arteria Pulmonar/cirugía , Procedimientos de Cirugía Plástica/métodos , Anomalías del Sistema Respiratorio/cirugía , Estudios Retrospectivos , Tráquea/anomalías , Tráquea/cirugía , Estenosis Traqueal/congénito , Estenosis Traqueal/cirugía , Resultado del TratamientoRESUMEN
PURPOSE: The application of slide tracheoplasty (STP) in the treatment of congenital tracheal stenosis (CTS) has improved patient outcomes over the past few decades. We reviewed our experiences with the procedure, elucidated risk factors, and discussed important aspects of perioperative management to improve outcomes. METHOD: Patients with CTS undergoing STP between July 1998 and December 2020 were enrolled. Patient characteristics, perioperative condition, management, operative details, and outcomes, including mortality and postoperative intervention, were collected from medical records. RESULTS: Eighty patients underwent STP. Sixty-five patients (81.3%) had an associated cardiovascular anomaly. Thirteen patients (16.3%) had unilateral lung agenesis or hypoplasia. Preoperative mechanical ventilation was necessary in 54 (67.5%) patients, and extracorporeal membrane oxygenation (ECMO) was required in eight patients (10.0%). An endotracheal tube was placed before the stenotic entrance to avoid granulation. During STP, the trachea was dissected as little as possible to maintain the blood supply. The one-year survival rate was 88.8% (nine patients died). One patient (1.3%) required postoperative balloon dilation, and none required stenting or granulation removal. Of the survivors, 62 (92.5%) achieved successful extubation without tracheostomy. Multivariable analysis revealed complex cardiovascular anomaly (P = 0.05) and preoperative ECMO (P = 0.019) to be adverse predictors of survival. CONCLUSION: Although STP can be performed successfully in CTS patients, surgeons and families should be aware of factors that may lead to a more difficult postoperative course or increase the mortality. Meticulous, perioperative positioning of the endotracheal tube and preserving the tracheal blood flow can minimize the need for postoperative intervention.
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Procedimientos de Cirugía Plástica , Estenosis Traqueal , Niño , Constricción Patológica , Humanos , Procedimientos de Cirugía Plástica/métodos , Estudios Retrospectivos , Tráquea/anomalías , Tráquea/cirugía , Estenosis Traqueal/congénito , Estenosis Traqueal/cirugía , Resultado del TratamientoAsunto(s)
Fístula Biliar , Fístula Bronquial , Fístula Biliar/diagnóstico , Fístula Bronquial/diagnóstico , Humanos , EsputoRESUMEN
BACKGROUND: The optimal management method for pediatric pancreatic trauma is controversial. Moreover, the efficacy of stent placement via endoscopic retrograde pancreatography (ERP) remains poorly documented. METHODS: The present, retrospective review of pediatric patients with pancreatic trauma was conducted from 2010 to 2020 at a single institution. RESULTS: Ten, male children with the median age of 9.5 years (range 4-14 years) with a grade I (n = 2), II (n = 4) or III (n = 4) pancreatic injury were identified. Of six of these patients in whom ERP was performed, four had a pancreatic duct injury (PDI). Pancreatic stent placement was performed in all the patients with ERP at a site proximal to the injury in four patients and across the injury in two patients. A pseudocyst or pancreatic fluid collection was detected in five patients, of these, two with a grade II injury were managed successfully with conservative therapy while three with PDI required surgery. In the four patients with PDI, only one in whom the stent was placed across the PDI was able to avoid surgery. CONCLUSION: Therapeutic ERP might be effective even if a patient has a PDI, therefore, early ERP should be considered as a treatment option.
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Traumatismos Abdominales , Enfermedades Pancreáticas , Adolescente , Niño , Preescolar , Colangiopancreatografia Retrógrada Endoscópica , Humanos , Masculino , Conductos Pancreáticos/cirugía , Estudios RetrospectivosRESUMEN
BACKGROUND: Patients with severe motor and intellectual disabilities often suffer from tracheal stenosis due to chest deformation and brachiocephalic artery compression, which sometimes leads to serious complications, such as dying spell and tracheobrachiocephalic artery fistula. We herein described our experience of performing a novel and simple thoracoplastic procedure combined with brachiocephalic artery transection in two patients with severe chest deformation and tracheal stenosis. CASE PRESENTATION: The patients were a 12-year-old female with cerebral palsy due to periventricular leukomalacia and a 21-year-old male with subacute sclerosing panencephalitis stage IV in the Jabbour classification following a laryngotracheal separation. Both patients showed severe chest deformation and symptoms of airway stenosis resulting in dying spells. The sternum was laterally transected between the manubrium and the sternal body, and a manubriotomy was performed longitudinally, ending with an inverse T-shaped sternotomy. Since the clavicle and the first rib remained attached to the halves of the divided manubrium, the sternum was allowed to be left open, resulting in improvement of the mediastinal narrowing and tracheal stenosis. Postoperative computed tomography (CT) showed that the distance between the halves of the manubrium was maintained at 10-11 mm, and that the mediastinal narrowing in both patients improved; the sternocervical spine distance increased from 20 mm to 22 and 13 mm to 16 mm, respectively. The patients' tracheal stenosis below the sternal end of the clavicle and the manubrium and respiratory symptoms improved, and the patients are currently at home in a stable condition with no chest fragility and no upper limb movement disorder 1 year after surgery. CONCLUSIONS: Our observations suggested that the inverse T-shaped sternotomy combined with brachiocephalic artery transection may relieve symptoms of tracheal stenosis due to severe chest deformation in patients with severe motor and intellectual disabilities.
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Etilefrine, a sympathomimetic agent, is reportedly effective against postoperative chylothorax. However, its effectiveness in treating congenital chylothorax was unknown. We report herein a case of refractory congenital chylothorax treated with etilefrine in a late preterm neonate with massive fetal chylous pleural effusion. The chylothorax was unresponsive to previous treatments, including dietary and pharmacological treatment and thoracic duct ligation. The pleural effusion decreased after intravenous etilefrine was begun on day of life (DOL) 84 and resolved after the addition of chemical pleurodesis with OK-432 on DOL 90. This combination therapy may be a viable treatment option for cases of congenital chylothorax that are unresponsive to other treatments.
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PURPOSE: Lingual thyroglossal duct cysts (L-TGDCs) are rare and sometimes lethal owing to their association with asphyxia. We aimed to analyze our single institutional experience with L-TGDCs. METHODS: Twelve L-TGDC cases treated at our institution between January 2010 and December 2017 were investigated. RESULTS: The male/female ratio was 6/6. The age at the diagnosis was 2⯱â¯1.4â¯months (7â¯days to 6â¯months), and 3 patients were diagnosed in the neonatal period. The patients presented with stridor (nâ¯=â¯12; 100%), growth retardation (nâ¯=â¯5; 42%), apnea (nâ¯=â¯3; 25%), and vomiting (nâ¯=â¯1; 8.3%). Lateral X-rays were obtained in 8 cases (66.7%); a lingual mass was suspected in 7 (87.5%). Transoral marsupialization of the cyst was performed under direct vision in all cases. All cases were nasally and orally intubated using a laryngoscope, bronchoscope, or airway scope. The mean operative time was 18⯱â¯2.9â¯min. The mean cyst size was 10.5⯱â¯1.8â¯mm. No recurrence was observed during the follow-up period (37.5⯱â¯18â¯months). CONCLUSION: L-TGDC requires a precise diagnosis and rapid intervention because of the risk of asphyxia resulting in sudden death. Transoral marsupialization under direct vision is an effective and secure approach. L-TGDC should be considered when patients younger than six months of age present with respiratory distress. TYPE OF STUDY: Retrospective Study. LEVEL OF EVIDENCE: Level IV.