RESUMO
PURPOSE: Surgical procedures for anorectoplasty for anorectal malformations (ARMs), particularly rectourethral fistula (RUF), depend on the institution. We investigated the diagnosis and treatment of RUF in male patients with ARMs in Japan using a questionnaire survey. METHODS: An online survey inquiring about the diagnosis and treatment (diagnostic modalities, surgical approaches, fistula dissection devices, and fistula closure techniques) of each type of ARM in male patients was conducted among institutional members of the Japanese Study Group of Anorectal Anomalies. Fisher's exact test was used to compare surgical methods between posterior sagittal anorectoplasty (PSARP) and laparoscopy-assisted anorectoplasty (LAARP). RESULTS: Sixty-one institutions (100%) completed the survey. LAARP was the preferred approach for high-type ARM (75.4%). PSARP was preferred for intermediate-type ARM (59.0%). Monopolar devices were most commonly used (72.1%) for RUF dissection. Blunt dissection was more frequent in the PSARP group (PSARP vs. LAARP: 55.6 vs. 20.0%, p < 0.005). Cystoscopy/urethroscopy to confirm the extent of dissection was used more frequently in the LAARP group (70.0% vs. 25.0%, p < 0.005). Clips and staplers were used more frequently in the LAARP group (p < 0.05). CONCLUSION: Distinct fistula management strategies for PSARP and LAARP were revealed. Further studies are needed to investigate the postoperative outcomes associated with these practices.
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Malformações Anorretais , Fístula Retal , Doenças Uretrais , Fístula Urinária , Humanos , Masculino , Fístula Retal/cirurgia , Fístula Retal/diagnóstico , Japão , Malformações Anorretais/cirurgia , Fístula Urinária/cirurgia , Fístula Urinária/diagnóstico , Inquéritos e Questionários , Doenças Uretrais/cirurgia , Doenças Uretrais/diagnóstico , Anus Imperfurado/cirurgia , Anus Imperfurado/diagnóstico , Laparoscopia/métodosRESUMO
PURPOSE: Anorectal malformations are associated with other organ malformations. Proximodistal elongation of the cloacal plate and anal opening at its distal end are essential for anal development. However, the anal developmental stage in which Wnt5a is directly involved remains unelucidated. Here, we attempted to identify this developmental stage; since Wnt5a is expressed in the mesoderm, and the striated muscle complex (SMC) in mice develops from the mesoderm, we also examined Wnt5a contribution to SMC development. METHODS: We established conditional knockout (CKO) mice in which Wnt5a could be knocked out using an appropriate tamoxifen dose. We evaluated the macroscopic appearance and histopathological features of Wnt5aCKO and wild-type mouse embryos. RESULTS: Wnt5aCKO mice showed phenotypes typical of Wnt5a constitutional knockout mice when Wnt5a was knocked out at E8-E11. Furthermore, the anus failed to open when Wnt5a was knocked out at E8 but opened when it was knocked out at E9 or thereafter. The caudal end of the SMC was dysplastic in Wnt5aCKO mice induced at E8, but was unaffected when mice were induced at E9 or thereafter. CONCLUSION: We suggest a critical role for Wnt5a in anal opening and SMC formation at a very early stage of embryonic development.
Assuntos
Canal Anal , Malformações Anorretais , Desenvolvimento Embrionário , Proteína Wnt-5a , Canal Anal/anormalidades , Animais , Malformações Anorretais/genética , Cloaca , Desenvolvimento Embrionário/genética , Feminino , Camundongos , Camundongos Knockout , Gravidez , Proteína Wnt-5a/genéticaRESUMO
PURPOSE: Hepatobiliary scintigraphy is a minimally invasive imaging method that evaluates bile flow dynamics. At our hospital, it has been performed for postoperative evaluation of patients with choledochal cysts (CC). This study evaluated the usefulness of biliary scintigraphy for predicting late complications in patients with CCs. METHODS: The study included pediatric patients with CC who underwent surgery at Chiba University Hospital from 1978 to 2020, followed by postoperative biliary scintigraphy and subsequent radiologic evaluation. The patients were divided into two groups according to the presence or absence of "biliary cholestasis" on biliary scintigraphy. RESULTS: The study included 108 patients, with a median age at surgery of 2 years and 11 months. The median follow-up period was 5203 days, with 11 hepatolithiasis cases and 8 cholangitis cases. No patients had cholangiocarcinoma. Twelve patients were considered to have "cholestasis" following biliary scintigraphy evaluation. There was no significant difference in the occurrence of hepatolithiasis between the cholestasis and non-cholestasis groups (p = 0.47), but cholangitis was significantly more common in the cholestasis group (p = 0.016). CONCLUSION: Biliary cholestasis on postoperative hepatobiliary scintigraphy was a risk factor for cholangitis in patients with CCs. These particular patients should be monitored carefully.
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Colangite , Cisto do Colédoco , Colestase , Litíase , Hepatopatias , Humanos , Criança , Cisto do Colédoco/complicações , Cisto do Colédoco/diagnóstico por imagem , Cisto do Colédoco/cirurgia , Litíase/complicações , Hepatopatias/complicações , Cintilografia , Colangite/diagnóstico por imagem , Colangite/etiologia , Complicações Pós-Operatórias/epidemiologiaRESUMO
PURPOSE: Although the impairment of regulatory T-cells (Tregs) has been shown in the liver or portal area of biliary atresia (BA) the frequency and function of circulating Tregs in BA patients is poorly understood. We aimed to investigate the frequency and function of circulating Tregs in BA patients. METHODS: Peripheral blood mononuclear cells were collected from 25 BA patients and 24 controls. Treg frequency was measured by flow cytometry; function was determined by T-cell proliferation assay. We also assessed the association between Treg frequency/function and clinical parameters in BA cases. RESULTS: There was no significant difference between the two groups in both frequency (BA: 3.4%; control: 3.2%; p = 0.97) and function (BA: 22.0%; control: 7.5%; p = 0.23) of Tregs. We further focused on 13 preoperative BA patients and 14 age-matched controls. Neither Treg frequency nor function were significantly different (frequency: BA: 4.6%; control: 3.4%; p = 0.38, function: BA: 2.7%; control: 7.6%; p = 0.89). There was no association between Treg frequency/function and clinical parameters. CONCLUSION: Neither the frequency nor function of circulating Tregs was affected in BA patients, suggesting the negative role of circulating Tregs in the pathogenesis of BA. Further investigation of local Treg profiles is warranted.
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Atresia Biliar , Humanos , Atresia Biliar/cirurgia , Linfócitos T Reguladores , Leucócitos Mononucleares , Fígado , Citometria de FluxoRESUMO
PURPOSE: The optimal timing of surgery for congenital diaphragmatic hernia (CDH) is controversial. We aimed to validate our protocol for the timing of CDH repair using the quantified patent ductus arteriosus (PDA) flow pattern. METHODS: This retrospective comparative study analyzed patients with a prenatal diagnosis of isolated CDH between 2007 and 2020. We defined the "LR ratio" as the percentage of velocity-time integral (VTI) of the left-to-right flow of PDA against overall VTI on echocardiography. Since 2010, we followed the decision criterion of performing surgery when LR ratio of > 50% has been achieved in the patients (protocol group). The protocol group (2010-2020) was compared with the historical control group (2007-2009). RESULTS: The average age at surgery was 104.1 ± 175.9 and 37.3 ± 30.6 h in the control and protocol groups, respectively (p = 0.11). Survival rate (88.9% vs. 95.0%, p = 0.53) and the rate of worsening of pulmonary hypertension within 24 h after surgery (22.2% vs. 10.0%, p = 0.57) were not different between the groups. The protocol group had a significantly shorter duration of tracheal intubation (26.9 ± 21.1 vs. 13.3 ± 9.5 days, p = 0.03). CONCLUSION: Our decision criterion might have the advantage of facilitating early and safe surgery for patients with CDH.
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Anormalidades Múltiplas , Velocidade do Fluxo Sanguíneo/fisiologia , Permeabilidade do Canal Arterial/fisiopatologia , Hérnias Diafragmáticas Congênitas/cirurgia , Herniorrafia/métodos , Pré-Escolar , Permeabilidade do Canal Arterial/diagnóstico , Ecocardiografia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Duração da Cirurgia , Estudos RetrospectivosRESUMO
Dendritic cells (DC) play a key role in the initiation of both antitumor immunity and immunological tolerance. It has been demonstrated that exposure to soluble factors produced by tumor cells modulates DC functions and induces tolerogenic DC differentiation. In this study, we investigated the effects of neuroblastoma cell line-derived soluble factors on DC differentiation. Monocytes isolated from healthy volunteers were incubated with interleukin (IL)-4 and granulocyte-macrophage colony-stimulating factor in the presence of culture supernatants from neuroblastoma cell lines. The culture supernatants from neuroblastoma cell lines, such as NLF and GOTO, partially blocked both downregulation of CD14 and upregulation of CD1a, and dramatically decreased IL-12 and tumor necrosis factor (TNF)-α production from mature DC, while no effect of SH-SY5Y cell supernatant was noted. In addition, IL-6 and IL-10 production from monocytes was increased by the supernatants of NLF and GOTO cells at 24 hours after incubation. Furthermore, we evaluated DC functions through stimulation of invariant natural killer T (iNKT) cells. α-Galactosylceramide-pulsed DC co-cultured with supernatants of NLF cells were unable to sufficiently stimulate iNKT cells. The decreased ability of iNKT cells to produce interferon (IFN)-γ after stimulation with neuroblastoma cell line supernatant-cultured DC was reversed by addition of IL-12. CD40 expression and IL-12 production in NLF-sup-treated DC were increased by addition of exogenous IFN-γ. These results indicate that tolerogenic DC are induced in the neuroblastoma tumor microenvironment and attenuate the antitumor effects of iNKT cells. Interactions between iNKT cells and αGalCer-pulsed DC have the potential to restore the immunosuppression of tolerogenic DC through IFN-γ production.
Assuntos
Diferenciação Celular/imunologia , Células Dendríticas/imunologia , Ativação Linfocitária/imunologia , Neuroblastoma/imunologia , Antígenos CD1/imunologia , Linhagem Celular , Linhagem Celular Tumoral , Fator Estimulador de Colônias de Granulócitos e Macrófagos/imunologia , Humanos , Interferon gama/imunologia , Interleucina-10/imunologia , Interleucina-12/imunologia , Interleucina-4/imunologia , Receptores de Lipopolissacarídeos/imunologia , Monócitos/imunologia , Monócitos/metabolismoRESUMO
PURPOSE: The relationship between reflux index (RI) and bolus exposure parameters in multichannel intraluminal impedance (MII) has not been examined sufficiently among children. The significance of acid and bolus exposure in evaluating pediatric reflux disease (RD) was explored by focusing on mucosal lesions. METHODS: We conducted MII-pH on 28 patients (median age 8 years) with suspected RD. We assessed relationships between RI and bolus exposure indices, and also compared acid and bolus exposures across patients grouped by endoscopic esophageal mucosal lesions. RESULTS: RI correlated significantly with distal acid reflux events (r = 0.60), acid bolus exposure time (BET) (0.55), and bolus clearance time (BCT) (0.48). Significant differences were observed among the control, non-erosive RD (NERD), and erosive RD (ERD) groups in all acid and several bolus exposure indices (distal and proximal frequencies, and BCT), while no significant difference was apparent between NERD and ERD. Acid exposure tended to be more severe in high-grade than in low-grade ERD, while no similar tendency was found in any bolus parameters other than BCT. CONCLUSIONS: MII-pH showed great potential for investigating the pathophysiology of pediatric RD, with RI revealing different correlations with variable bolus exposure indices. However, no specific parameters allowing precise discrimination between RDs or mucosal severities were identified.
Assuntos
Ácido Gástrico/metabolismo , Refluxo Gastroesofágico/diagnóstico , Mucosa Intestinal/patologia , Adolescente , Adulto , Criança , Pré-Escolar , Impedância Elétrica , Monitoramento do pH Esofágico/métodos , Esofagoscopia , Feminino , Refluxo Gastroesofágico/metabolismo , Refluxo Gastroesofágico/fisiopatologia , Humanos , Lactente , Masculino , Índice de Gravidade de Doença , Adulto JovemRESUMO
BACKGROUND: Invariant natural killer T (iNKT) cells play an important role in tumor immunity, enhancing both innate and acquired immunity. We have previously shown the enhancement of antibody-dependent cellular cytotoxicity against neuroblastoma by activated iNKT cells. As a first step towards clinical application, we studied the frequency and proliferative response of circulating iNKT cells in children with and without cancer. METHODS: Blood samples were collected from 10 patients with pediatric malignant solid tumors and 11 patients with non-neoplastic diseases (control). The frequency of circulating iNKT cells was quantified by flow cytometry. Whole peripheral blood mononuclear cells were then stimulated with α-galactosylceramide (α-GalCer) for 7 days, and the expansion rate of the iNKT-cell fraction was assessed. RESULTS: The frequency of iNKT cells in the patients of the cancer and control group did not differ to a statistically significant extent. The iNKT-cell population increased after α-GalCer stimulation in all cases. The iNKT cells of patients who had undergone intensive chemotherapy also had the potential to expand in vitro. CONCLUSIONS: Unlike adult cancer patients, the numbers of circulating iNKT cells were not decreased in pediatric cancer patients. α-GalCer stimulation induced a proliferative response in all of the patients.
Assuntos
Células T Matadoras Naturais/patologia , Neuroblastoma/sangue , Adulto , Animais , Proliferação de Células , Criança , Pré-Escolar , Citometria de Fluxo , Humanos , Lactente , Contagem de Linfócitos , Masculino , Neuroblastoma/patologia , Células Tumorais CultivadasRESUMO
BACKGROUND: Tumor immunity has been suggested to play a key role in clinical and biological behavior of neuroblastomas. Given that CD1-restricted invariant natural killer T (iNKT) cells enhance both innate and acquired tumor immunity, we investigated the expression of the iNKT-cell-specific T-cell receptor Vα24-Jα18 in neuroblastoma tissues and its correlation with clinical and biological characteristics. METHODS: Using real- time quantitative PCR, we quantified the expression of Vα24-Jα18 in untreated tumor samples from 107 neuroblastoma cases followed in our institution and analyzed the correlation between the presence of infiltrated iNKT cells and clinical characteristics or patients' outcome. RESULTS: Vα24-Jα18 receptor was detected in 62 untreated cases (57.9%). The expression was significantly higher in stages 1, 2, 3, or 4S (P = 0.0099), in tumors with low or intermediate risk (P = 0.0050), with high TrkA expression (P = 0.0229), with favorable histology (P = 0.0026), with aneuploidy (P = 0.0348), and in younger patients (P = 0.0036). The overall survival rate was significantly higher in patients with iNKT-cell infiltration (log-rank; P = 0.0089). CONCLUSIONS: Since tumor-infiltrating iNKT cells were predominantly observed in neuroblastomas undergoing spontaneous differentiation and/or regression, we suggest that iNKT cells might play a key role in these processes.
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DNA de Neoplasias/genética , Regulação Neoplásica da Expressão Gênica , Proteína Proto-Oncogênica N-Myc/genética , Células T Matadoras Naturais/patologia , Neuroblastoma/genética , Receptores de Antígenos de Linfócitos T/genética , Southern Blotting , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Mucosa Intestinal , Masculino , Proteína Proto-Oncogênica N-Myc/biossíntese , Células T Matadoras Naturais/metabolismo , Neuroblastoma/metabolismo , Neuroblastoma/patologia , Reação em Cadeia da Polimerase em Tempo Real , Receptores de Antígenos de Linfócitos T/biossínteseRESUMO
Anti-ganglioside GD2 antibodies mainly work through antibody-dependent cellular cytotoxicity (ADCC) and have demonstrated clinical benefit for children with neuroblastoma. However, high-risk neuroblastoma still has a high recurrence rate. For further improvement in patient outcomes, ways to maximize the cytotoxic effects of anti-GD2 therapies with minimal toxicity are required. Activated invariant natural killer T (iNKT) cells enhance both innate and type I acquired anti-tumor immunity by producing several kinds of cytokines. In this report, we investigated the feasibility of combination therapy using iNKT cells and an anti-GD2 antibody. Although some of the expanded iNKT cells expressed natural killer (NK) cell markers, including FcγR, iNKT cells were not directly associated with ADCC. When co-cultured with activated iNKT cells, granzyme A, granzyme B and interferon gamma (IFNγ) production from NK cells were upregulated, and the cytotoxicity of NK cells treated with anti-GD2 antibodies was increased. Not only cytokines produced by activated iNKT cells, but also NK-NKT cell contact or NK cell-dendritic cell contact contributed to the increase in NK cell cytotoxicity and further IFNγ production by iNKT cells and NK cells. In conclusion, iNKT cell-based immunotherapy could be an appropriate candidate for anti-GD2 antibody therapy for neuroblastoma.
Assuntos
Citotoxicidade Celular Dependente de Anticorpos , Células T Matadoras Naturais/imunologia , Neuroblastoma/terapia , Animais , Antígenos CD1d/metabolismo , Linhagem Celular Tumoral , Citocinas , Humanos , Ativação Linfocitária , Camundongos , Neuroblastoma/imunologiaRESUMO
Minimally invasive surgery has become widely recognized and is commonly used for the diagnosis and treatment of neuroblastoma. However, in post-chemotherapy status or during reoperations, it is occasionally difficult to precisely locate small neuroblastoma lesions, and this becomes prominent in endoscopic surgeries, in which tactile sense is essentially lost. Herein, we report our preliminary experience in two abdominal neuroblastoma cases undergoing laparoscopic tumor resection with aid of intraoperative (123)I- metaiodobenzylguanidine (MIBG) radioguidance using a specifically designed gamma-probe. The procedure enables easier localization of viable neuroblastoma tissue, provided that the tumor shows moderate to high MIBG uptake.
Assuntos
3-Iodobenzilguanidina , Radioisótopos do Iodo , Laparoscopia , Neuroblastoma/diagnóstico por imagem , Neuroblastoma/cirurgia , Compostos Radiofarmacêuticos , Cirurgia Assistida por Computador , Pré-Escolar , Feminino , Humanos , Lactente , Monitorização Intraoperatória , Neuroblastoma/patologia , Prognóstico , CintilografiaRESUMO
BACKGROUND: The significance of pediatric endoscopic retrograde cholangiopancreatography (ERCP) according to age or disease variation is inconclusive. This study aimed to evaluate the usefulness of pediatric ERCP in diagnosing or treating small children with pancreaticobiliary disorders, including choledochal cyst (CC) and biliary atresia (BA). METHODS: From 1980 to 2011, 235 ERCPs were performed in 220 pediatric patients (median age, 2 years) at our institution. Underlying pathology was CC in 92 patients (3 years), BA in 62 patients (55 days), and others in 66 patients. Success and complication rates, and ERCP findings were retrospectively analyzed. RESULTS: The overall success rate was 96%. Rates for individual pathologies were 99% for CC, 92% for BA, and 96% for others. The success rate was 100% among children >3 years, but 92% when limited to infants. Post-ERCP hyperamylasemia and duodenal perforation occurred in 9% and 0.4% of cases, respectively. Regarding preoperative evaluation of the pancreaticobiliary system in CC, ERCP clearly delineated pancreaticobiliary maljunction (79%) and the pancreatic duct (94%), whereas it visualized the common bile duct and intrahepatic bile duct at relatively low rates (77% and 33%, respectively). ERCP successfully identified 16 cases (18%) with non-BA of 90 patients with suspected BA. Moreover, ERCP demonstrated only pancreatic duct in 70% of all BA. CONCLUSIONS: ERCP among children including infants with CC and BA can be performed with fairly satisfactory results. Although pediatric ERCP can also help describe the pancreaticobiliary system in detail, its indication should be deliberately considered when anatomical information from less-invasive imaging modalities is insufficient.
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Atresia Biliar/diagnóstico por imagem , Colangiopancreatografia Retrógrada Endoscópica/métodos , Cisto do Colédoco/diagnóstico por imagem , Colestase/diagnóstico por imagem , Ducto Colédoco/diagnóstico por imagem , Pancreatopatias/diagnóstico por imagem , Ductos Pancreáticos/diagnóstico por imagem , Adolescente , Adulto , Ductos Biliares Intra-Hepáticos/diagnóstico por imagem , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Adulto JovemRESUMO
PURPOSE: In the recent years in Japan, an increasing number of patients with neuroblastoma (NB) are being treated by the "delayed local treatment (DL)" policy, undergoing surgery after the completion of high-dose chemotherapy with hematopoietic stem cell rescue (HDC). We reviewed the histopathological findings of second-look operations, including those of patients treated with DL. PATIENTS: From 1998 to 2013, 26 patients with high-risk NB underwent radical operation following chemotherapy. Surgery was performed after induction chemotherapy in 17 cases (standard; STD), whereas 9 cases completed induction chemotherapy and HDC before undergoing tumor resection (DL). The amount of necrosis and the degree of differentiation within the post-treatment tumor were assessed. RESULTS: Eighty-eight percent of the tumors showed necrosis in more than 1/3 of the specimen. Two DL cases showed complete disappearance of viable tumor cells. Amount of necrosis did not affect the prognosis of the patient. Tumors with immature, poorly differentiated phenotypes showed an extremely aggressive thereafter. Though not statistically proven, (123)I-MIBG (metaiodobenzylguanidine) uptake may be correlated with the amount of viable cells remaining within the tumor, but not with the degree of differentiation. CONCLUSIONS: Our results support the previous reports advocating that tumors that sustain unfavorable histology after chemotherapy behave aggressively thereafter.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Quimioterapia de Indução/métodos , Neuroblastoma/diagnóstico por imagem , Neuroblastoma/tratamento farmacológico , 3-Iodobenzilguanidina , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Japão , Masculino , Neuroblastoma/cirurgia , Cintilografia , Compostos Radiofarmacêuticos , Estudos Retrospectivos , Cirurgia de Second-Look/métodos , Análise de Sobrevida , Resultado do TratamentoRESUMO
BACKGROUND: The serum level of amylase (sAm) is commonly used as a biochemical marker for diagnosis and management of pancreatic disorders. However, the use of the urine level of amylase (uAm) is limited in practice, because the diagnostic ability of uAm is inferior to that of sAm. In the present study, the possible concordance of uAm-rerated parameters with sAm was investigated, and evaluate the usefulness of uAm for management of hyperamylasemia. METHODS: From June 1995 to October 2009, 804 samples of both urine and blood were collected from 128 patients in order to measure the serum level of amylase (sAm) and the urine level of amylase (uAm) and creatinine (uCr). Concordance of parameters using uAm compared to sAm was assessed. Parameters used were uAm, amylase creatinine clearance ratio (ACCR), and the ratio of uAm to uCr (uAm/uCr). RESULTS: uAm/uCr had the best correlation with sAm (r = 0.779, p < 0.001) compared to uAm (r = 0.620, p < 0.001) and to ACCR (r = 0.374, p < 0.001), when sAm was over the standard level. The area under the receiver operating characteristic curve of uAm/uCr (0.884) was significantly higher than that of uAm (0.766) and of ACCR (0.666) (p < 0.001 for each). The cutoff value of uAm/uCr was 569.8, with a sensitivity of 81.0% and a specificity of 83.1%. CONCLUSIONS: The uAm/uCr ratio correlated with sAm, and may be an alternative to sAm for prediction of hyperamylasemia. Use of urine samples results in a decreased need for blood sampling, which is especially beneficial in pediatric patients.
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Amilases/urina , Creatinina/urina , Hiperamilassemia/urina , Adolescente , Adulto , Envelhecimento/urina , Amilases/sangue , Biomarcadores/urina , Criança , Pré-Escolar , Cisto do Colédoco/complicações , Cisto do Colédoco/urina , Grupos Diagnósticos Relacionados , Feminino , Humanos , Hiperamilassemia/etiologia , Hiperamilassemia/terapia , Lactente , Masculino , Pancreatite/complicações , Pancreatite/urina , Estudos Retrospectivos , Viés de Seleção , Centro Cirúrgico Hospitalar/estatística & dados numéricos , Centros de Atenção Terciária/estatística & dados numéricos , Adulto JovemRESUMO
BACKGROUND: Soft tissue sarcomas (STS) of pelvic origin in boys often involve the urogenital organs. The optimal extensiveness of radical surgery has long been an issue of discussion, since exenterative surgeries result in severe urogenital adverse effects. We conducted a retrospective review of patients with pelvic STS treated in two regional center hospitals and assessed the radicality of surgery and the functional outcome of the bladder. PATIENTS: Medical records and surgical reports of nine cases (embryonal rhabdomyosarcoma 6, malignant triton tumor 2, suspected rhabdomyosarcoma 1) treated within 1997-2012 were reviewed. Site of origin was prostate in seven, retroperitoneal in two. Average follow-up period was 96 months. TREATMENT AND OUTCOME: All cases were subjected to neoadjuvant chemotherapy. Response was PR in four, SD in two, and PD in two. Radical surgery resulted in gross total resection in eight, and partial resection in one. Three underwent cystoprostatectomy, two cases underwent prostatectomy, and bladder-preserving tumor resection was carried out in four cases. At the last follow-up, three retained a functional bladder. Two required augmentation cystoplasty with intestinal conduits. CONCLUSIONS: The majority of the on-going clinical trials recommend conservative surgery for bladder/prostate rhabdomyosarcoma, and to preserve the bladder function particularly in chemosensitive tumors. Some other groups, however, advocate the importance of radical surgery to prevent local relapse. These reports include heterogenous group of patients in the cohort, and therefore it is difficult to draw a conclusion of which approach truly contributes to the survival of the patients better. Future studies should evaluate bladder and sexual function objectively to establish reliable evidence regarding the benefit and adverse effects of different surgical approaches. These data would be informative to optimize the treatment balance for children with pelvic rhabdomyosarcomas.
Assuntos
Neoplasias Pélvicas/cirurgia , Rabdomiossarcoma/cirurgia , Sarcoma/cirurgia , Micção/fisiologia , Neoplasias Urogenitais/cirurgia , Procedimentos Cirúrgicos Urogenitais/métodos , Pré-Escolar , Seguimentos , Humanos , Lactente , Masculino , Neoplasias Pélvicas/diagnóstico , Neoplasias Pélvicas/fisiopatologia , Estudos Retrospectivos , Rabdomiossarcoma/diagnóstico , Rabdomiossarcoma/fisiopatologia , Sarcoma/diagnóstico , Sarcoma/fisiopatologia , Resultado do Tratamento , Neoplasias Urogenitais/diagnóstico , Neoplasias Urogenitais/fisiopatologiaRESUMO
BACKGROUND: Laryngotracheoesophageal cleft (LTEC) is a rare disease in which the larynx and trachea communicate posteriorly to the esophagus. It is often associated with other congenital malformations, particularly gastrointestinal anomalies. Herein, we report a case of LTEC associated with a gastric polypoid lesion in bronchial tissue. CASE PRESENTATION: A gastric mass was detected in a male fetus since week 21 of gestation using fetal ultrasonography. Esophagogastroduodenoscopy performed after birth revealed a pedunculated polypoid lesion of the gastric fornix. The patient experienced frequent vomiting and aspiration pneumonia, which persisted after nasoduodenal tube feeding. Communication between the airway and esophagus was suspected. Laryngoscopy performed 30 days later revealed an LTEC (type III). Partial gastrectomy was performed when the patient was 93 days of age. Histopathological examination revealed tumor consisting of cartilage tissue covered with a layer of respiratory epithelium. CONCLUSION: The gastric tumor associated with LTEC exhibited structures mimicking bronchial tissue. LTEC occurs because of foregut maldevelopment, and the tumorous respiratory tissue in the stomach may have been formed from the same abnormal foregut development event as LTEC.
RESUMO
PURPOSE: Gastric perforation (GP) of the newborn is a rare, serious, and life-threatening problem, and its etiology remains unclear. Although historically GP has often been described as "spontaneous'', some cases are non-spontaneous. The aim of the present study was to review cases of GP and to discuss its etiology in a single prefecture in Japan over a period of 20 years. METHODS: Eleven cases with GP that underwent surgery in 4 institutions in the Chiba Prefecture from 1991 to 2010 were reviewed and divided into 2 groups: the early (1991-2000, n = 7) and late (2001-2010, n = 4) groups. RESULTS: No factors were observed that could have caused GP other than malformations associated with distal obstruction (3 midgut volvulus, 1 jejunal stenosis, 1 diaphragm eventration). Distal obstruction was present in 1 case in the early group and all 4 cases in the late group (p = 0.015). While the incidence of GP did not change over the 20-year period reviewed, the incidence of GP without distal obstruction significantly decreased in the late group. CONCLUSION: The proportion of patients with GP and distal obstruction increased and true "spontaneous" cases of GP decreased over time. The possible presence of distal obstruction should be evaluated during surgery for GP.
Assuntos
Doenças do Recém-Nascido/etiologia , Ruptura Gástrica/etiologia , Adulto , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Feminino , Seguimentos , Humanos , Incidência , Recém-Nascido , Doenças do Recém-Nascido/diagnóstico , Doenças do Recém-Nascido/epidemiologia , Japão , Masculino , Estudos Retrospectivos , Ruptura Espontânea , Ruptura Gástrica/diagnóstico , Ruptura Gástrica/epidemiologia , Taxa de Sobrevida/tendênciasRESUMO
The clinical features and risk factors of acute appendicitis in infants are unclear. Our aim was to evaluate the association between anthropometrics and the occurrence of infantile appendicitis. This was a retrospective study of infants (<6 years of age) and school-age children (6-10 years of age) of Asian ethnicity who required hospitalization for appendicitis at our two participating institutions between 2004 and 2018. The Z-score for height, body weight, and body mass index (BMI) was compared between the two groups, as well as between patients presenting with perforated and non-perforated appendicitis. The analysis included data from 73 infants and 362 school-age children. Z-scores were greater in infants than in school-age children for height (0.37 versus -0.03, p = 0.003) and body weight (0.12 versus -0.36, p = 0.023), with no between-group difference for the Z-score of BMI. There was no difference in Z-scores for height, weight, and BMI between the perforated and non-perforated appendicitis infant groups. Infants presenting with acute appendicitis were characterized by a larger physique but with normal proportion. This trend was not observed in school-age children. Therefore, larger infants presenting with abdominal pain should be screened for appendicitis.
RESUMO
Omphalomesenteric cysts are an exceedingly rare type of embryologic remnant of the omphalomesenteric duct. Owing to its rarity and unspecific imaging findings, it is occasionally difficult to diagnose preoperatively. Herein, we report the case of a 15-month-old female with an omphalomesenteric cyst that presented as a painful abdominal mass. Imaging showed a 4 cm cystic lesion just beneath the umbilicus, which also contained a 1 cm enhanced lesion. On the immediate right side of this cyst, a 7 cm hematoma was found within the abdominal wall. Aspiration revealed bloody fluid with an amylase level of 38,250 U/L. She was then diagnosed with an omphalomesenteric cyst, with aberrant pancreas and associated hematoma of the abdominal wall. These findings were confirmed with laparotomy and subsequent pathological examinations. The high level of amylase in the cyst led us to speculate the existence of ectopic pancreatic tissue. Thus, amylase measurement may be considered for the diagnosis of an omphalomesenteric cyst.
RESUMO
It is essential to accurately and safely resect all tumors during surgery for multiple lung metastases. Here, we report a case of hepatoblastoma (HB) with multiple pulmonary nodules that ultimately underwent complete resection using combined three-dimensional image reconstruction and indocyanine green (ICG) fluorescence guidance. A 1-year-old boy was diagnosed with HB and multiple lung metastases. After intensive chemotherapy, complete resection with subsegmentectomy (S5 + 6) and partial resection (S3, S8) were performed. More than 100 pulmonary nodules, which remained visible on computed tomography (CT) despite additional postoperative chemotherapy, were subjected to pulmonary resection. We used the SYNAPSE VINCENT software (Fujifilm Medical, Tokyo, Japan) to obtain three-dimensional images of the nodules. We numbered each nodule, and 33 lesions of the right lung were resected by multiple wedge resections through a right thoracotomy, with the aid of palpation and ICG fluorescence guidance. One month after the right metastasectomy, resection of 64 lesions in the left lung was performed via left thoracotomy. Postoperative CT showed complete clearance of the lung lesions, and the patient remained disease-free for 15 months after the treatment. This case study confirms that the combination of three-dimensional localization and ICG fluorescence guidance allows for accurate and safe resection of nearly 100 lung metastases.