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1.
Commun Med (Lond) ; 4(1): 111, 2024 Jun 11.
Artículo en Inglés | MEDLINE | ID: mdl-38862768

RESUMEN

BACKGROUND: Biliary atresia (BA) is an intractable disease of unknown cause that develops in the neonatal period. It causes jaundice and liver damage due to the destruction of extrahepatic biliary tracts,. We have found that heterozygous knockout mice of the SRY related HMG-box 17 (Sox17) gene, a master regulator of stem/progenitor cells in the gallbladder wall, exhibit a condition like BA. However, the precise contribution of hypoplastic gallbladder wall to the pathogenesis of hepatobiliary disease in Sox17 heterozygous embryos and human BA remains unclear. METHODS: We employed cholangiography and histological analyses in the mouse BA model. Furthermore, we conducted a retrospective analysis of human BA. RESULTS: We show that gallbladder wall hypoplasia causes abnormal multiple connections between the hilar hepatic bile ducts and the gallbladder-cystic duct in Sox17 heterozygous embryos. These multiple hilar extrahepatic ducts fuse with the developing intrahepatic duct walls and pull them out of the liver parenchyma, resulting in abnormal intrahepatic duct network and severe cholestasis. In human BA with gallbladder wall hypoplasia (i.e., abnormally reduced expression of SOX17), we also identify a strong association between reduced gallbladder width (a morphometric parameter indicating gallbladder wall hypoplasia) and severe liver injury at the time of the Kasai surgery, like the Sox17-mutant mouse model. CONCLUSIONS: Together with the close correlation between gallbladder wall hypoplasia and liver damage in both mouse and human cases, these findings provide an insight into the critical role of SOX17-positive gallbladder walls in establishing functional bile duct networks in the hepatic hilus of neonates.


Biliary atresia (BA) is a disease in newborns that causes a serious liver condition due to damage to the bile ducts (the pathways that carry bile juice). Although reduced function of a key gene called Sox17, which is essential for forming the gallbladder wall, has been observed in some BA cases, the link between gallbladder issues and liver damage is unknown. This study has shown how damage spreads through the bile ducts in the liver around the time of birth when there are problems in the gallbladder wall due to reduced SOX17 function. The findings indicate that proper growth of the gallbladder wall during this critical period is essential for forming a normal network of bile ducts in the developing liver. This discovery is promising for early diagnosis and better treatment of BA in newborns.

2.
J Ultrasound Med ; 43(8): 1521-1533, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-38708926

RESUMEN

OBJECTIVES: Extrapulmonary sequestration (EPS) within the diaphragm (ID-EPS) is rare and requires additional procedures such as incision or detachment of the diaphragm from the lesion for diagnosis. This study aimed to describe the imaging findings and evaluate the diagnostic accuracy of ultrasonography and computed tomography (CT) for ID-EPS. MATERIAL AND METHODS: Split diaphragm sign, shape of lesion edge, drainage vein into intra-abdomen on ultrasound and CT, and lesion characteristics on ultrasound were compared between patients with ID-EPS and with above-diaphragm EPS (AD-EPS) using Fisher's exact test. RESULTS: Three and nine patients were diagnosed with ID-EPS and AD-EPS, respectively. Significant differences were observed between the two groups in the split diaphragm sign on ultrasound (presence/absence in patients with ID-EPS vs AD-EPS, 2/1 vs 0/9, P = .046), shape of lesion edge on ultrasound/CT (round/beak in patients with ID-EPS vs AD-EPS, 3/0 vs 0/9, P = .005 on both CT and ultrasound), lesion characteristics on ultrasound (presence/absence of cystic area within lesion in patients with ID-EPS vs AD-EPS, 0/3 vs 7/2, P = .046), and the drainage vein into the abdomen on CT (presence/absence in patients with ID-EPS vs AD-EPS; 2/1 vs 0/9, P = .046). No drainage veins were visualized in the abdomen on ultrasonography and no significant differences in the presence/absence of the split-diagram sign on CT (presence/absence in patients with ID-EPS vs AD-EPS and ID-EPS; 0/3 vs 0/9, P > .999; 1/2 vs 0/9, P = .250) were observed between the two groups. CONCLUSION: A combination of postnatal ultrasonography and CT was useful in predicting EPS located within the diaphragm.


Asunto(s)
Secuestro Broncopulmonar , Diafragma , Tomografía Computarizada por Rayos X , Ultrasonografía , Humanos , Proyectos Piloto , Femenino , Masculino , Diafragma/diagnóstico por imagen , Ultrasonografía/métodos , Tomografía Computarizada por Rayos X/métodos , Reproducibilidad de los Resultados , Secuestro Broncopulmonar/diagnóstico por imagen , Adulto , Persona de Mediana Edad , Sensibilidad y Especificidad , Estudios Retrospectivos , Anciano , Adolescente
3.
J Laparoendosc Adv Surg Tech A ; 34(4): 376-379, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38407921

RESUMEN

Purpose: This study aimed to compare respiratory functions of patients after thoracoscopic lobectomy (TS) with those after thoracotomy (TR). Methods: This retrospective study was conducted in two centers, one of which adapted TS as a standard procedure in 2009 and the other performs it via TR. Data on patients who underwent lobectomy for congenital lung disease between 2009 and 2021 and underwent pulmonary function test (spirometry) were collected. Results: Ten patients underwent TS and 36 underwent TR. Distribution based on sex, prenatal diagnosis, pathological diagnosis, and resected lobe were similar between the two groups. The median [interquartile range] age at procedure in the TR group was significantly smaller than that in the TS group (13 [11-18] months versus 38 [13-79] months, P = .03). The procedure duration in the TR group was significantly shorter than that in the TS group (230 [171-264] minutes versus 264 [226-420] minutes, P = .02). Pulmonary function test was conducted at the age of eight in both groups, but the interval between the procedure, and the test was significantly shorter in the TS group (TR: 7 [5-8] years versus TS: 5 [2-7] years, P = .03). The ratio of forced vital capacity compared to predicted one (TR: 86.6 [76.6-95.3] versus TS: 88.7 [86.8-89.1], P = .58) and the ratio of forced expiratory volume in 1 second against that predicted (TR: 84.0 [80.5-88.7] versus TS: 88.7 [86.8-89.1], P = .08) were not significantly different between the two groups. Conclusions: Although TR was performed earlier than TS, respiratory function was similar between the two groups.


Asunto(s)
Neoplasias Pulmonares , Pulmón , Humanos , Lactante , Niño , Pulmón/cirugía , Neumonectomía/efectos adversos , Neumonectomía/métodos , Estudios Retrospectivos , Volumen Espiratorio Forzado , Capacidad Vital , Toracotomía/efectos adversos , Toracotomía/métodos , Neoplasias Pulmonares/cirugía , Resultado del Tratamiento , Cirugía Torácica Asistida por Video
4.
Int J Hematol ; 119(1): 99-103, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-37740816

RESUMEN

Thyroid involvement is rare in pediatric Langerhans cell histiocytosis (LCH). It may cause airway narrowing, leading to acute-onset respiratory distress. Severe cases may require emergent surgical interventions such as thyroidectomy, which should be avoided in children due to higher rates of complication, particularly in infancy. There is currently no consensus on the indications for surgical treatment in LCH with thyroid involvement. In this report, we describe the cases of two children who presented with tracheal stenosis caused by thyroid LCH, both of which were successfully treated by early induction of chemotherapy, and one of which was also treated for a shorter duration. Mutation analysis detected in-frame deletions of BRAF exon 12 in both cases. These cases suggest that timely diagnosis and administration of chemotherapy may alleviate severe airway obstruction and reduce the need for thyroidectomy in pediatric patients with thyroid LCH.


Asunto(s)
Histiocitosis de Células de Langerhans , Enfermedades de la Tiroides , Estenosis Traqueal , Humanos , Niño , Tiroidectomía , Enfermedades de la Tiroides/complicaciones , Enfermedades de la Tiroides/diagnóstico , Estenosis Traqueal/terapia , Estenosis Traqueal/complicaciones , Histiocitosis de Células de Langerhans/complicaciones , Histiocitosis de Células de Langerhans/terapia , Histiocitosis de Células de Langerhans/diagnóstico
5.
Asian J Surg ; 47(2): 968-972, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-38030485

RESUMEN

BACKGROUND: The superiority of thoracoscopic repair (TR) over conventional open repair (COR) for esophageal atresia, especially in terms of long-term outcomes, remains to be investigated. The aim of this study was to compare short- and long-term results between TR and COR group. METHODS: Patients who underwent TR or COR for esophageal atresia without other predispositions to musculoskeletal deformities (2003-2016) and had been followed up for a minimum of 5 years were retrospectively reviewed. Musculoskeletal deformities (e.g., scoliosis, chest wall asymmetry, and rib deformities) were mainly evaluated based on the most recent chest radiographs. RESULTS: Nine and eight patients were included in the TR and COR groups, respectively; the mean follow-up period was 8.7 and 11.5 years, respectively (p = 0.14). These groups had similar epidemiological characteristics and rates of postoperative complications. Musculoskeletal deformities developed significantly less frequently in the TR group versus the COR group (11 % vs. 88 %, p < 0.05; scoliosis: 0 % vs. 38 %, p = 0.08; chest wall asymmetry: 11 % vs. 50 %, p = 0.14; and rib deformities: 11 % vs. 88 %, p < 0.05, respectively). CONCLUSION: TR was associated with a decreased incidence of musculoskeletal deformities and comparable complication rates versus COR for esophageal atresia repair. TR may achieve better long-term outcomes in this setting.


Asunto(s)
Atresia Esofágica , Escoliosis , Fístula Traqueoesofágica , Humanos , Atresia Esofágica/cirugía , Estudios Retrospectivos , Resultado del Tratamiento , Toracoscopía , Fístula Traqueoesofágica/cirugía
6.
J Vasc Surg Cases Innov Tech ; 9(4): 101332, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-38106343

RESUMEN

Central lymphatic diseases such as intractable chylothorax can be fatal. Lymphatic venous anastomosis at the venous angle level is expected to give a direct therapeutic effect because it opens the obstructed outlet of the main lymphatic vessels. However, the original methods resulted in some important issues, such as the potential for venous reflux. In the present case, we modified the original anastomosis method by interposing a vein graft with venous valves to increase the distance and prevent venous reflux. Collecting the lymphatic flow resulted in termination of the chylothorax with preserved postoperative patency for years, without any complications, including at the graft-harvested extremity.

7.
J Environ Manage ; 347: 119110, 2023 Dec 01.
Artículo en Inglés | MEDLINE | ID: mdl-37783076

RESUMEN

Ongoing global change makes it ever more urgent to find creative solutions for biodiversity preservation, but prioritizing sites for protection can be challenging. One shortcut lies in mapping the habitat requirements of well-established biodiversity indicators, such as top predators, to identify high-biodiversity sites. Here, we planned site protection for biodiversity conservation by developing a multi-scale species distribution model (SDM) for the raptorial Northern Goshawk (Accipiter gentilis; goshawk) breeding in an extensive megacity region of Japan. Specifically, we: (1) examined the determinants of top predator occurrence and thus of high-biodiversity value in this megacity setting, (2) identified the biodiversity hotspots, (3) validated whether they actually held higher biodiversity through an independent dataset, and (4) evaluated their current protection by environmental laws. The SDM revealed that goshawks preferred secluded sites far from roads, with abundant forest within a 100 m radius and extensive forest ecotones suitable for hunting within a 900 m radius. This multi-scale landscape configuration was independently confirmed to hold higher biodiversity, yet covered only 3.2% of the study area, with only 44.0% of these sites legally protected. Thus, a rapid biodiversity assessment mediated by a top predator quickly highlighted: (1) the poor development of biodiversity-friendly urban planning in this megacity complex, an aspect overlooked for decades of rapid urban sprawl, and (2) the extreme urgency of extending legal protection to the sites missed by the current protected area network. Exigent biodiversity indicators, such as top predators, could be employed in the early or late stages of anthropogenic impacts in order to proactively incorporate biodiversity protection into planning or flag key biodiversity relics. Our results confirm and validate the applied reliability of top predatory species as biodiversity conservation tools.


Asunto(s)
Biodiversidad , Ecosistema , Animales , Reproducibilidad de los Resultados , Bosques , Conducta Predatoria , Conservación de los Recursos Naturales/métodos
8.
Lipids Health Dis ; 22(1): 172, 2023 Oct 14.
Artículo en Inglés | MEDLINE | ID: mdl-37838679

RESUMEN

Mead acid (MA, 5,8,11-eicosatrienoic acid) is an n-9 polyunsaturated fatty acid (PUFA) and a marker of essential fatty acid deficiency, but nonetheless generally draws little attention. MA is distributed in various normal tissues and can be converted to several specific lipid mediators by lipoxygenase and cyclooxygenase. Recent pathological and epidemiological studies on MA raise the possibility of its effects on inflammation, cancer, dermatitis and cystic fibrosis, suggesting it is an endogenous multifunctional PUFA. This review summarizes the biosynthesis, presence, metabolism and physiological roles of MA and its relation to various diseases, as well as the significance of MA in PUFA metabolism.


Asunto(s)
Ácido 8,11,14-Eicosatrienoico , Ácidos Grasos Insaturados , Humanos , Ácidos Grasos Insaturados/metabolismo , Inflamación
9.
Pediatr Surg Int ; 39(1): 271, 2023 Sep 09.
Artículo en Inglés | MEDLINE | ID: mdl-37684432

RESUMEN

PURPOSE: To ensure the safe spread of pediatric endoscopic surgery, it is essential to build a training curriculum, and a survey of the current situation in Japan is necessary. The present study assessed an efficient training curriculum by clarifying instructor class pediatric surgeons' experiences, including autonomy when performing advanced endoscopic surgeries. METHODS: An online nationwide questionnaire survey was conducted among pediatric surgeons who had Endoscopic Surgical Skill Qualification (ESSQ) and board-certified instructors who had skills comparable to ESSQ. We assessed participants' training experience, opinions concerning the ideal training curriculum, and the correlation between surgical experience and the level of autonomy. The Zwisch scale was used to assess autonomy. RESULTS: Fifty-two participants responded to the survey (response rate: 86.7%). Only 57.7% of the respondents felt that they had received sufficient endoscopic surgery training. Most respondents considered an educational curriculum for endoscopic surgery including off-the-job training essential during the training period. Autonomy had been acquired after experiencing two to three cases for most advanced endoscopic surgeries. CONCLUSION: This first nationwide survey in Japan showed that instructor class pediatric surgeons acquired autonomy after experiencing two to three for most advanced endoscopic surgeries. Our findings suggest that training, especially off-the-job training, has been insufficient.


Asunto(s)
Especialidades Quirúrgicas , Cirujanos , Humanos , Niño , Japón , Curriculum , Endoscopía
10.
Case Rep Pediatr ; 2023: 5593369, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37711640

RESUMEN

Inguinal hernia is the most common surgical disease in pediatric patients, and urgent intervention such as manual reduction is needed for incarcerated inguinal hernia. Torsion of undescended testes, inguinal herniated ovarian torsion, and purulent lymphadenitis are mimickers of this condition. If these mimicker lesions are misdiagnosed as incarcerated inguinal hernia, manual reduction usually fails, and edematous and erythematous changes may occur in these mimicker lesions due to manual reduction. For physicians in the emergency department, prompt decisions and familiarity with the sonographic appearance of different contents within an inguinal hernia are important to accurately diagnose these mimickers. In this case series, we present sonographic images of a typical case of incarcerated inguinal hernia (an 11-month-old male with right incarcerated inguinal hernia) and three cases of mimicker lesions (a 7-month-old female with herniated ovarian torsion, a 7-year-old boy with undescended testicular torsion, and a 2-month-old male with purulent lymphadenitis). The incidence of incarcerated inguinal hernia is reported to be higher in males (80%), on the right side (60%), and in infants and toddlers. This information is important for diagnosing mimicker lesions. In addition, to prevent manual reduction in mimicker diseases, point-of-care ultrasound before manual reduction in suspected cases of incarcerated inguinal hernia is important.

11.
Surg Today ; 53(12): 1363-1371, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37087700

RESUMEN

PURPOSE: This study aimed to evaluate the learning curve of thoracoscopic repair of tracheoesophageal fistula (TEF) by a single surgeon using a cumulative sum (CUSUM) analysis. METHODS: Prospective clinical data of consecutive Gross type-C TEF repairs performed by a pediatric surgeon from 2010 to 2020 were recorded. CUSUM charts for anastomosis and operating times were generated. The learning curves were compared with the effect of accumulation based on case experience. RESULTS: For 33 consecutive cases, the mean operative and anastomosis times were 139 ± 39 min and 3137 ± 1110 s, respectively. Significant transitions beyond the learning phase for total operating and anastomosis times were observed at cases 13 and 17. Both the total operating time and anastomosis time were significantly faster in the proficiency improvement phase than in the initial learning phase. Postoperative complications significantly decreased after the initial anastomosis learning phase but not after the initial total operating learning phase. CONCLUSIONS: Thoracoscopic repair of TEF is considered safe and feasible after 13 cases, where the surgeon can improve their proficiency with the total operation procedure, and 17 cases, which will enable the surgeon to achieve proficiency in anastomosis. Postoperative complications significantly decreased after gaining familiarity with the anastomosis procedure through the learning phase.


Asunto(s)
Atresia Esofágica , Cirujanos , Fístula Traqueoesofágica , Niño , Humanos , Fístula Traqueoesofágica/cirugía , Fístula Traqueoesofágica/complicaciones , Atresia Esofágica/cirugía , Estudios Prospectivos , Anastomosis Quirúrgica , Complicaciones Posoperatorias/epidemiología , Curva de Aprendizaje , Toracoscopía/métodos , Estudios Retrospectivos
12.
Surg Today ; 53(11): 1269-1274, 2023 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-37017869

RESUMEN

PURPOSE: Postoperative anastomotic leakage is the most frequent short-term complication of esophageal atresia repair in neonates. We conducted this study using a nationwide surgical database in Japan to identify the risk factors for anastomotic leakage in neonates undergoing esophageal atresia repair. METHODS: Neonates diagnosed with esophageal atresia between 2015 and 2019 were identified in the National Clinical Database. Postoperative anastomotic leakage was compared among patients to identify the potential risk factors, using univariate analysis. Multivariable logistic regression analysis included sex, gestational age, thoracoscopic repair, staged repair, and procedure time as independent variables. RESULTS: We identified 667 patients, with an overall leakage incidence of 7.8% (n = 52). Anastomotic leakage was more likely in patients who underwent staged repairs than in those who did not (21.2% vs. 5.2%, respectively) and in patients with a procedure time > 3.5 h than in those with a procedure time < 3.5 h (12.6% vs. 3.0%, respectively; p < 0.001). Multivariable logistic regression analysis identified staged repair (odds ratio [OR] 4.89, 95% confidence interval [CI] 2.22-10.16, p < 0.001) and a longer procedure time (OR 4.65, 95% CI 2.38-9.95, p < 0.001) as risk factors associated with postoperative leakage. CONCLUSION: Staged procedures and long operative times are associated with postoperative anastomotic leakage, suggesting that leakage is more likely after complex esophageal atresia repair and that such patients require refined treatment strategies.


Asunto(s)
Atresia Esofágica , Fístula Traqueoesofágica , Recién Nacido , Humanos , Atresia Esofágica/cirugía , Fuga Anastomótica/epidemiología , Fuga Anastomótica/etiología , Estudios Retrospectivos , Anastomosis Quirúrgica/efectos adversos , Anastomosis Quirúrgica/métodos , Factores de Riesgo , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Fístula Traqueoesofágica/complicaciones , Fístula Traqueoesofágica/cirugía
13.
J Clin Ultrasound ; 51(5): 819-826, 2023 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-36870044

RESUMEN

OBJECTIVES: To describe the incidence and diagnostic performance of ultrasound for perianal abscess or fistula-in-ano in pediatric patients with perianal inflammation. METHODS: We included 45 patients with perianal inflammation who underwent ultrasonography. To demonstrate the diagnostic performance of ultrasound for fistula-in-ano, a definite diagnosis of perianal abscess, and fistula-in-ano was determined as that proven through magnetic resonance imaging (MRI) or computed tomography (CT). The absence or presence of perianal abscess and fistula-in-ano on ultrasonography was recorded. RESULTS: Among the 45 patients, on ultrasound, perianal abscess and fistula-in-ano were detected in 22 (48.9%) and 30 (68.2%) patients, respectively. Nine patients had MRI or CT and a definite diagnosis of perianal abscess or fistula-in-ano; accuracy, negative predictive value, and positive predictive value of ultrasound for perianal abscess were 77.8% (7/9; 95% confidence interval [CI]: 40.0%-97.1%), 66.7% (2/3; 95% CI: 9.4%-99.2%), 83.3% (5/6; 95% CI: 35.9%-99.6%), and those of fistula-in-ano were 100% (9/9; 95% CI: 66.4%-100%), 100% (8/8; 95% CI: 63.1%-100%), and 100% (1/1; 95% CI: 2.5%-100%), respectively. CONCLUSIONS: Perianal abscess and fistula-in-ano were detected by ultrasound in half of the patients with perianal inflammation. Accordingly, ultrasound has an acceptable diagnostic performance for perianal abscess and fistula-in-ano.


Asunto(s)
Enfermedades del Ano , Fístula Rectal , Humanos , Niño , Absceso/diagnóstico por imagen , Incidencia , Enfermedades del Ano/diagnóstico por imagen , Enfermedades del Ano/epidemiología , Enfermedades del Ano/complicaciones , Fístula Rectal/diagnóstico por imagen , Fístula Rectal/epidemiología , Ultrasonografía/efectos adversos
14.
Australas J Ultrasound Med ; 26(1): 26-33, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-36960131

RESUMEN

Purpose: To investigate and determine the sonographic findings obtained from manually distorted testes to predict testicular atrophy following manual detorsion. Materials and methods: Twenty-two patients who had been diagnosed with testicular torsion and undergone manual detorsion were included. These patients were classified according to the presence or absence of testicular atrophy. The duration of symptoms, presence or absence of hyperperfusion within the entire affected testis, and echogenicity (homogeneous or heterogeneous) within the affected testis were compared using the Mann-Whitney U-test or Fisher's exact test, as appropriate. Results: Testicular atrophy was detected in seven patients. There was a significant difference in the frequency of hyperperfusion within the entire affected testis (with atrophy [present/absent] vs. without atrophy [present/absent] = 0/7 vs. 8/7, P = 0.023) between patients with and without testicular atrophy. No significant differences in the duration of symptoms (with atrophy vs. without atrophy = 7 ± 3.3 h vs. 4.7 ± 3.6 h, P = 0.075) or frequency of echogenicity within the testis (with atrophy [heterogeneous/homogeneous] vs. without atrophy [heterogeneous/homogeneous] = 2/5 vs. 2/13, P = 0.565) were observed between the groups. Conclusions: This small cohort study suggests that the presence of hyperperfusion within the entire affected testis immediately after successful manual detorsion is useful in predicting the avoidance of testicular atrophy.

15.
Pediatr Blood Cancer ; 70(1): e30030, 2023 01.
Artículo en Inglés | MEDLINE | ID: mdl-36184783

RESUMEN

BACKGROUND: Biopsies for diagnosis before chemotherapy is common in children with malignant solid tumors. Wound healing is delayed by chemotherapy; however, the ideal interval between biopsy and chemotherapy remains unknown. We aimed to summarize the relationship between chemotherapy timing and postoperative surgical complications. PROCEDURE: We retrospectively reviewed patients with malignant solid tumors who underwent chemotherapy after surgical biopsy at our institution between January 2014 and August 2020. The primary outcomes were postoperative surgical complications (within 30 days) and the timing of chemotherapy. RESULTS: Forty-three patients were analyzed. The types of tumors were neuroblastoma (n = 20), hepatoblastoma (n = 10), Ewing sarcoma (n = 5), germ cell tumor (n = 3), angiosarcoma (n = 1), clear cell sarcoma (n = 1), ganglioneuroblastoma (n = 1), rhabdoid tumor (n = 1), and rhabdomyosarcoma (n = 1). The operative procedures were thoracoscopy (n = 5), laparotomy (n = 17), laparoscopy (n = 14), and superficial (n = 7). The median time [range] to chemotherapy after biopsy was 4 [0-21] days. No surgical complications occurred before chemotherapy, and two (4.7%) patients experienced complications after chemotherapy. These included postoperative hemorrhage (grade 3) and surgical site infection (grade 1). Chemotherapy was initiated 1 and 6 days after biopsy, respectively, in these cases. Complications occurred 10 and 23 days after biopsy, respectively. CONCLUSION: The rate of postoperative surgical complications related to biopsy seems acceptable, even when chemotherapy was initiated in the early postoperative period. Early initiation of chemotherapy after biopsy may be a suitable option, particularly in children with bulky or symptomatic malignant solid tumors.


Asunto(s)
Hepatoblastoma , Neoplasias Hepáticas , Neuroblastoma , Niño , Humanos , Estudios Retrospectivos , Hepatoblastoma/cirugía , Biopsia , Complicaciones Posoperatorias/etiología , Neuroblastoma/cirugía , Neoplasias Hepáticas/cirugía
16.
Int J Hematol ; 117(5): 759-764, 2023 May.
Artículo en Inglés | MEDLINE | ID: mdl-36469185

RESUMEN

Liver cirrhosis due to secondary sclerosing cholangitis caused by Langerhans cell histiocytosis (LCH) has a poor prognosis, and liver transplantation is the definitive treatment. However, the optimal timing has not been established. We report a 2-year-old girl with LCH-related liver cirrhosis who successfully underwent liver transplantation before progressing to severe liver dysfunction. Physical examination revealed a tumor on her palate. Biopsy was performed, and a diagnosis of LCH was established, together with hepatomegaly, splenomegaly, and rashes. Percutaneous liver biopsy before treatment revealed extreme fibrosis and absence of LCH cells. After beginning chemotherapy, she experienced several delays in treatment and dose reductions because of unacceptable bone marrow suppression, worsening liver dysfunction, and cholangitis. However, tumor shrinkage was observed in both magnetic resonance imaging and BRAF V600E mutant allele titers in her plasma. Given the good treatment response, liver transplantation was conducted. The postoperative course was uneventful, and chemotherapy was resumed 34 days after liver transplantation. Subsequent maintenance treatment was completed with no severe adverse effects. To prevent perioperative complications due to exacerbation of liver dysfunction and possible discontinuation of chemotherapy, liver transplantation should be considered before development of end-stage liver failure, provided that the original disease is well controlled.


Asunto(s)
Colangitis Esclerosante , Histiocitosis de Células de Langerhans , Hepatopatías , Trasplante de Hígado , Humanos , Femenino , Preescolar , Trasplante de Hígado/efectos adversos , Colangitis Esclerosante/complicaciones , Colangitis Esclerosante/patología , Hepatopatías/etiología , Cirrosis Hepática/complicaciones , Histiocitosis de Células de Langerhans/complicaciones , Histiocitosis de Células de Langerhans/terapia , Histiocitosis de Células de Langerhans/diagnóstico
17.
J Laparoendosc Adv Surg Tech A ; 32(12): 1234-1236, 2022 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-36350681

RESUMEN

Aim: To evaluate the impact of previous infection on perioperative outcomes in patients undergoing thoracoscopic lobectomy for congenital lung anomalies. Methods: This was a single-institution retrospective observational study for which patients who underwent thoracoscopic lobectomy for congenital lung disease between 2009 and 2021 were enrolled, and patients with extralobar sequestration were excluded. Patient background and data related to the surgery were compared between patients who had an infection before surgery (Group 1) and those who did not (Group 2). Results: This study included 34 patients, 13 in Group 1 and 21 in Group 2. The sex-based distribution and pathological diagnosis were similar between the two groups. Malformations were prenatally diagnosed in 1 patient in Group 1 (7.7%) and 18 patients in Group 2 (86%; P < .001). The median age and weight at the time of the procedure and procedure duration were comparable between the two groups. The amount of blood loss was significantly higher in Group 1 (60 mL) than in Group 2 (20 mL; P = .0042). Four patients in Group 2 required reoperation due to air leakage, pyothorax, and cardiac tamponade, whereas none of the Group 1 patients required reoperation (P = .12). No conversion to thoracotomy was required in either group. The duration of postoperative admission was similar between the two groups (Group 1: 6 days versus Group 2: 6 days; P = .14). Conclusions: Preceding infection increased the amount of bleeding during thoracoscopic lobectomy but had little effect on other outcomes.


Asunto(s)
Enfermedades Pulmonares , Neoplasias Pulmonares , Anomalías del Sistema Respiratorio , Humanos , Neumonectomía/métodos , Tiempo de Internación , Resultado del Tratamiento , Enfermedades Pulmonares/cirugía , Anomalías del Sistema Respiratorio/complicaciones , Anomalías del Sistema Respiratorio/cirugía , Estudios Retrospectivos , Pulmón/patología , Neoplasias Pulmonares/cirugía , Cirugía Torácica Asistida por Video/métodos , Toracoscopía/métodos
18.
Pediatr Surg Int ; 38(12): 1785-1791, 2022 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-36102983

RESUMEN

PURPOSE: This study aimed to compare the perioperative outcomes of laparoscopically assisted anorectoplasty (LAARP) and conventional procedures (CPs) for anorectal malformation (ARM) using a national inpatient database in Japan. METHODS: Using the Diagnosis Procedure Combination database, we identified patients who underwent anorectoplasty for high- or intermediate-type ARMs from 2010 to 2019. Primary outcomes were postoperative rectal prolapse, anal stenosis, and general complications. Secondary outcomes were the duration of anesthesia and length of hospital stay. We performed 1:2 propensity score-matched analyses to compare the outcomes between the LAARP and CP groups. RESULTS: We identified 1005 eligible patients, comprising 286 and 719 patients who underwent LAARP and CP, respectively. The propensity score-matched groups included 281 patients with LAARP and 562 with CP. The LAARP group showed a higher proportion of rectal prolapse (21.4% vs. 8.5%; odds ratio, 2.91; 95% confidence interval [CI], 1.89-4.48; p < 0.001) and longer duration of anesthesia (462 min vs. 365 min; difference, 90 min; 95% CI 43-137; p < 0.001) than the CP group. No significant differences were found in other outcomes. CONCLUSION: LAARP had worse outcomes than CP in terms of rectal prolapse. Thus, we propose that LAARP may require technical refinement to improve patient outcomes.


Asunto(s)
Malformaciones Anorrectales , Laparoscopía , Prolapso Rectal , Humanos , Lactante , Malformaciones Anorrectales/cirugía , Estudios Retrospectivos , Prolapso Rectal/cirugía , Laparoscopía/métodos , Resultado del Tratamiento , Recto/cirugía , Recto/anomalías , Canal Anal/cirugía , Canal Anal/anomalías
19.
Pediatr Int ; 64(1): e15208, 2022 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-35831265

RESUMEN

BACKGROUND: To determine the incidence and risk factors for late severe intestinal complications after surgical repair for intra-abdominal congenital intestinal atresia/stenosis. METHODS: We included 51 patients who underwent surgical repair for congenital intestinal atresia/stenosis. Late severe intestinal complications included adhesive ileus, incisional hernia, or volvulus. Whether surgical intervention was urgent or not was recorded. The location of the atresia/stenosis was classified into two groups: atresia/stenosis located at the oral or anal side from the Treitz ligament. The type of atresia/stenosis was classified as low-risk types (type I, mucosal web/II, fibrous cord/IIIa, mesenteric gap defect) and high-risk types (IIIb, apple peel/IV, multiple atresia). We compared the incidence of late intestinal complications between the location of intestinal atresia/stenosis at the oral and anal side of Treitz ligament, and between low- and high-risk types of atresia/stenosis using Fisher's exact test. RESULTS: Eight (15.7%) had late intestinal complications, all of which occurred in patients with intestinal atresia/stenosis located on the anal side of the ligament of Treitz. Urgent surgical intervention was needed in four cases. There was a significant difference in the location of atresia/stenosis (with vs. without late intestinal complications at oral/anal side of the Treitz ligament: 0/8 vs. 24/19; P = 0.005) and the type of intestinal atresia/stenosis (with vs. without that accompanying low-/high-risk type: 5/3 vs. 41/2; P = 0.023). CONCLUSIONS: Physicians should consider the presence of intestinal complications that require surgical intervention in patients undergoing surgical reconstruction for jejunal and ileal atresia/stenosis with abdominal symptoms.


Asunto(s)
Atresia Intestinal , Obstrucción Intestinal , Constricción Patológica , Humanos , Incidencia , Atresia Intestinal/epidemiología , Atresia Intestinal/cirugía , Obstrucción Intestinal/etiología , Yeyuno/anomalías , Yeyuno/cirugía
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