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PURPOSE: Consensus is lacking regarding the optimal antibiotic treatment for pediatric complicated appendicitis. This study determined the optimal first-line antibiotic treatment for pediatric patients with complicated appendicitis based on peritoneal fluid cultures. METHODS: This retrospective study examined the cases of pediatric patients who underwent appendectomy for complicated appendicitis at our institution between 2013 and 2019. Peritoneal fluid specimens obtained during appendectomy were cultured for the presence of bacteria. RESULTS: Eighty-six pediatric patients were diagnosed with complicated appendicitis. Of them, bacteria were identified in 54 peritoneal fluid samples. The major identified bacteria were Escherichia coli (n=36 [66.7%]), Bacteroides fragilis (n=28 [51.9%]), α-Streptococcus (n=25 [46.3%]), Pseudomonas aeruginosa (n=10 [18.5%]), Enterococcus avium (n=9 [16.7%]), γ-Streptococcus (n=9 [16.7%]), and Klebsiella oxytoca (n=6 [11.1%]). An antibiotic susceptibility analysis showed E. coli was inhibited by sulbactam/ampicillin in 43.8% of cases versus cefmetazole in 100% of cases. Tazobactam/piperacillin and meropenem inhibited the growth of 96.9-100% of the major identified bacteria. E. coli (100% vs. 84.6%) and P. aeruginosa (100% vs. 80.0%) were more susceptible to amikacin than gentamicin. CONCLUSION: Tazobactam/piperacillin or meropenem is a reasonable first-line antibiotic treatment for pediatric complicated appendicitis. In the case of aminoglycoside use, amikacin is recommended.
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PURPOSE: To determine the features which predict torsion and the pre-operative indicators of malignancy in cases of ovarian torsion in ovarian tumors (OTs) in children. METHODS: The medical records of 35 pediatric patients who underwent surgery for OT, except for neonate cases, from 1997 to 2018 at our institution were reviewed retrospectively. RESULTS: The pathological diagnosis was mature teratoma in 17, immature teratoma in 9, yolk sac tumor in 3, and others in 6. The preoperative diagnosis, which was made based on the imaging findings and the serum tumor marker values, matched with the pathological diagnosis in 29/35 (83%). Ovarian torsion occurred in 14/35 (40%). All but one case that presented with torsion had intermittent abdominal pain as the primary symptom. The preoperative white blood cell count was significantly higher in cases where ovary preservation was impossible than where it was possible (p = 0.01) among the cases presenting with torsion. CONCLUSION: Preoperative imaging findings and the serum tumor marker values enabled us to make an accurate preoperative diagnosis. Patients with intermittent abdominal primary symptoms were more likely to have ovarian torsion than those without such symptoms, and leukocytosis may indicate irreversible ischemic changes in the affected ovary.
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Procedimentos Cirúrgicos em Ginecologia/métodos , Neoplasias Ovarianas/diagnóstico , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Neoplasias Ovarianas/cirurgia , Período Pré-Operatório , Estudos Retrospectivos , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND: To assess the actual experiences of long-term follow-up and discuss ways to improve care during the period from childhood to adulthood in newborns who have undergone surgery. METHODS: A total of 306 patients with congenital anomalies requiring newborn surgery who survived to discharge from 1994 to 2013 were eligible for inclusion. Survivors with severe chromosomal and cardiac anomalies were excluded. Patients with myelomenigocele, urogenital anomalies and miscellaneous diagnoses were also excluded. Patients with Hirschsprung's disease were excluded since many of them underwent surgery after the neonatal period. Patients with hypertrophic pyloric stenosis were also excluded since their duration of follow-up was too short for this study. RESULTS: According to the follow-up status, survivors were categorized into 4 groups: under follow-up as an outpatient (UF, n = 67), moved (MV, n = 60), follow-up suspended by doctor (Sus, n = 87), and lost to follow-up (LF, n = 92). The incidence of active medical problems was high, and the duration of follow-up was significantly longer in the survivors with esophageal atresia, congenital diaphragmatic hernia and high-type anorectal malformations than in those with other anomalies. Survivors followed by pediatric surgeons alone, free from active medical problems or free from adverse events during the initial hospitalization were at risk of being LF. CONCLUSIONS: More than 30% of the surgical newborn cases were LF. Disease-specific and standardized multidisciplinary follow-up programs that increase both children's and parents' satisfaction and compliance are needed. (230/250 words).
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Anormalidades Congênitas/cirurgia , Recém-Nascido , Sobreviventes , Anormalidades Congênitas/mortalidade , Anormalidades Congênitas/psicologia , Feminino , Seguimentos , Humanos , Perda de Seguimento , Masculino , Pais/psicologia , Cooperação do Paciente , Satisfação do Paciente , Estudos Retrospectivos , Fatores de TempoRESUMO
BACKGROUND: Ovarian immature teratomas (ITs) are relatively rare among all pediatric ovarian tumors. The histological grading for ovarian ITs, which ranges from 1 to 3, is based on the proportion of immature neuroepithelial component. Higher-grade ITs in adults are treated as malignant neoplasms and require adjuvant chemotherapy. However, there is no consensus on the therapeutic management of pediatric ovarian ITs. The aim of our study was to analyze the histological grades and clinical characteristics of ovarian ITs in pediatric patients. METHODS: This retrospective chart review consisted of seven patients, including one, three, and three patients with histological grade 1, 2, and 3 pediatric ovarian ITs, respectively, who were treated at our institute between 2000 and 2016. Collected data comprised age, alpha-fetoprotein (AFP) level, clinical stage, tumor size, treatment, and prognosis. RESULTS: The median age and AFP levels of patients with grade 1, 2, and 3 ovarian ITs were 8, 7, and 10 years and 37, 112, and 221 ng/ml, respectively. All cases were Children Oncology Group (COG) stage I and International Federation of Gynecology and Obstetrics (FIGO) stage IA. All patients had unilateral tumors in the right ovary. The median tumor sizes of the grade 1, 2, and 3 IT patients were 104, 160, and 100 cm2, respectively. All patients underwent primary open surgery alone. Two patients, including one patient each with grade 2 and 3 ITs, underwent tumor enucleation as ovary-sparing surgery, whereas the remaining five patients underwent unilateral salpingo-oophorectomy. The median follow-up was seven years, and all cases achieved event-free survival. CONCLUSIONS: Clinical characteristics of patients with grade 3 ovarian ITs were relatively older and had higher AFP levels than those with lower-grade ITs. According to our patient's clinical course and prognosis, COG stage I pediatric ITs should be treated by surgery alone and that postoperative chemotherapy is unnecessary even for those with grade 3 ITs as well as patients with rather low AFP levels. LEVEL OF EVIDENCE: IV.
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Neoplasias Ovarianas/patologia , Neoplasias Ovarianas/cirurgia , Teratoma/patologia , Teratoma/cirurgia , Adolescente , Criança , Intervalo Livre de Doença , Feminino , Preservação da Fertilidade , Seguimentos , Humanos , Gradação de Tumores , Estadiamento de Neoplasias , Tratamentos com Preservação do Órgão , Neoplasias Ovarianas/sangue , Ovariectomia , Gravidez , Estudos Retrospectivos , Salpingectomia , Teratoma/sangue , Carga Tumoral , alfa-Fetoproteínas/metabolismoRESUMO
BACKGROUND: Nonoperative management (NOM) has been established as the standard treatment for isolated blunt organ injury in hemodynamically stable pediatric patients. Although delayed splenic rupture or bleeding is a rare complication in NOM, it is an issue that many pediatric surgeons are greatly concerned about. We herein report a rare pediatric case concerning the mechanisms involved in delayed splenic rupture after NOM. CASE PRESENTATION: A 9-year-old boy with severe abdominal pain was transferred to our hospital. Twenty-one hours before the admission, he had been kicked in the region of his left lateral abdomen. Contrast-enhanced abdominal computed tomography revealed a severe intra-parenchymal hematoma and multiple lacerations of the spleen with a large amount of hemoperitoneum without active bleeding. His condition was diagnosed as a grade III injury on the AAST splenic injury scale. After fluid resuscitation, his vital signs became stable. The patient was treated with NOM in our intensive care unit. However, suddenly after defecation (72 h after the injury), he started complaining of severe abdominal pain and left shoulder pain. His blood pressure dropped to 70/35 mmHg, and he started to lose consciousness. Abdominal ultrasonography (US) revealed increased ascites. Fluid resuscitation and blood transfusion were performed. His symptoms and abdominal US findings suggested that splenic re-bleeding had caused delayed splenic rupture to occur. Emergency splenectomy was performed. The resected spleen was enlarged with a large parenchymal hematoma. The posterior-lateral side of the splenic capsule was ruptured. CONCLUSIONS: The mechanism of delayed splenic rupture in our case was considered to be the result of a tear in the subcapsular hematoma caused by stretching the splenocolic ligament related to a bowel movement during defecation. Although delayed splenic rupture or bleeding is unpredictable, it is very important to understand the mechanisms and to educate the family of the children with splenic injuries of the warning signs of delayed rupture or bleeding.
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BACKGROUND: The aim of this study was to evaluate the outcome of an early discharge protocol for pediatric acute appendicitis. METHODS: The present new early discharge protocol for appendicitis consisted of both postoperative early feeding and reduced-port laparoscopic surgery, to reduce surgical stress. The outcome was studied in patients with acute appendicitis treated at the present institution from 2012 to 2013. RESULTS: Data on 36 acute appendicitis patients (mean age, 10.3 years) were collected. Operation time was 95 ± 27 min. Preoperatively, mean white blood cell (WBC) count was 13 850 ± 3644/µL; mean C-reactive protein (CRP), 2.7 ± 2.9 mg/dL; and mean procalcitonin, 0.25 ± 0.37 ng/mL. After surgery there was a significant decrease in WBC count, which fell to within the normal range; CRP peaked at 4.9 ± 3.2 mg/dL on postoperative day (POD) 1. On POD 7, all of the hematological markers were within the normal range. There were no postoperative complications. Mean hospital stay was 2.1 ± 1.1 days. Mean frequency of oral painkiller use was 3.2 ± 3.3 times per person. CONCLUSIONS: The present early discharge protocol is safe and effective for the management of acute non-perforated appendicitis.
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Apendicectomia , Apendicite/cirurgia , Alta do Paciente , Cuidados Pós-Operatórios/métodos , Doença Aguda , Apendicectomia/métodos , Criança , Protocolos Clínicos , Nutrição Enteral , Feminino , Seguimentos , Humanos , Laparoscopia , Masculino , Complicações Pós-Operatórias/prevenção & controle , Resultado do TratamentoRESUMO
OBJECTIVE: To establish better management practices to reduce morbidities in survivors with congenital diaphragmatic hernia (CDH). METHODS: Of 60 patients treated for CDH at our institution between 1991 and 2011, 49 patients without severe anomalies were retrospectively reviewed. RESULTS: Since 2004, gentle ventilation (GV) has been the main treatment for CDH. Patients were divided into the following two groups: the non-GV group (n = 29) who were treated before GV treatment was implemented, and the GV group (n = 20). The overall survival rate was 62.1% (18/29) and 95% (19/20) in the non-GV and GV groups, respectively (p = 0.016). Despite the high survival rate, the incidence of long-term complications in survivors was still high (14/19, 73.7%) in the GV group. In the GV group, liver-up (p = 0.106) and the need for patch repair (p = 0.257) tended to be associated with the development of long-term complications, but did not reach statistical significance. The presence of perioperative complications was associated with the development of long-term complications (p = 0.045) in the GV group. CONCLUSION: Patients who developed short-term complications seemed to be at risk of long-term complications. Therefore, to minimize long-term morbidities in CDH survivors, the prevention of short-term complications might be important.
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Hérnias Diafragmáticas Congênitas/complicações , Adolescente , Criança , Pré-Escolar , Terapia Combinada , Feminino , Seguimentos , Hérnias Diafragmáticas Congênitas/mortalidade , Hérnias Diafragmáticas Congênitas/terapia , Herniorrafia , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Respiração Artificial/métodos , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida , Resultado do TratamentoRESUMO
Pulmonary interstitial emphysema (PIE) primarily affects premature infants on positive pressure ventilation. PIE is rarely reported in infants and children in the absence of mechanical ventilation and/or associated respiratory infection. We report a case of PIE in a 22-month-old girl who had severe respiratory distress due to respiratory syncytial virus infection. Chest computed tomography showed cystic lung lesions mimicking congenital cystic adenomatoid malformation. The cystic lesions spontaneously resolved after conservative treatment. Based on the clinical course and the chronological changes on imaging, the cystic lung lesions were diagnosed as localized persistent PIE.
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Doenças do Prematuro/diagnóstico , Recém-Nascido Prematuro , Enfisema Pulmonar/etiologia , Infecções por Vírus Respiratório Sincicial/complicações , Vírus Sinciciais Respiratórios , Feminino , Humanos , Lactente , Enfisema Pulmonar/diagnóstico , Infecções por Vírus Respiratório Sincicial/diagnóstico , Infecções por Vírus Respiratório Sincicial/virologia , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND: Hypergastrinemia and the resultant peptic ulcer related to an enteric duplication has been quite rarely reported in the literature. CASE PRESENTATION: We herein report the case of a 4-year-old girl who presented with hypergastrinemia and a duodenal ulcer at 2 years of age. She had been followed up with a proton pump inhibitor, which resulted in resolution of the ulcer; however, unexplained hypergastrinemia had continued. A cystic lesion at the antrum was discovered at 4 years of age, which we suspected to be a gastric duplication. After we resected the lesion, the hypergastrinemia resolved without recurrence of the duodenal ulcer. The histology was compatible with a gastric duplication, and the lumen was lined with antral mucosa that strongly stained positive for gastrin. We presumed that the antral mucosa inside the duplication in our case had no hydrogen ion feedback inhibition of gastrin release from gastrin cells and increased release of gastrin from the mucosa inside the duplication led to the duodenal ulcer. Only two cases have been reported in the literature that had hypergastrinemia related to enteric duplication. CONCLUSION: Gastric duplication should be included in the differential diagnosis of sustained hypergastrinemia in children.
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An 8-month-old boy with a left-sided incarcerated inguinal hernia involving the appendix, cecum, and terminal ileum was successfully managed via an inguinal approach during an emergency operation. A mobile cecum seemed to have contributed to the left-sided incarceration. Only 13 similar cases with the left-sided Amyand's hernia have been reported in the literature.
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BACKGROUND: Rhabdomyosarcoma (RMS) is one of the most common soft tissue sarcomas among children. Patients who developed genitourinary/pelvic rhabdomyosarcoma (GU/P-RMS) have a higher complication ratio and relatively poorer event free survival, with local therapy being very important. While proton beam therapy (PBT) is expected to reduce co-morbidity, especially for children, this lacks firm evidence and analysis. We analyzed GU/P-RMS children who had undergone multimodal therapy combined with PBT at a single institution. METHOD: We retrospectively reviewed charts of children with GU/P-RMS treated from January 2007 to May 2013 at the University of Tsukuba Hospital who had undergone multimodal therapy with PBT. RESULTS: There were 5 children and their median age at diagnosis was 2.8 years (0.6-4.4 years). Primary sites were the bladder (2) and the prostate (3). All received neo-adjuvant chemotherapy and 3 underwent chemotherapy during PBT (Group Cx). All patients of Group Cx developed leukocytopenia (WBC <1000/µL). The median dose of PBT was 47.7 GyE (41.4-50.4 GyE). All patients survived by their last hospital visit (median, 36 months). CONCLUSIONS: We analyzed multimodal treatment combined with PBT applied for GU/P-RMS. PBT was well tolerated and could be a plausible choice instead of photon therapy for this population.
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PURPOSE: Persistent pulmonary hypertension remains a major cause of mortality and morbidity in cases of congenital diaphragmatic hernia (CDH). Recently, RhoA/Rho-kinase-mediated vasoconstriction has been reported to be important in the pathogenesis of pulmonary hypertension (PH). Several recent reports have described that fasudil, a potent Rho-kinase inhibitor and vasodilator, could represent a potential therapeutic option for PH. We designed this study to investigate the hypothesis that the expression level of RhoA is increased in the nitrofen-induced CDH rat model. The expression level of Wnt11, an activator of RhoA, was also evaluated. METHODS: Pregnant rats were treated with or without nitrofen on gestational day 9 (D9). Fetuses were sacrificed on D17, D19 and D21 and were divided into control and CDH groups. Quantitative real-time polymerase chain reaction was performed to determine the pulmonary gene expression levels of both Wnt11 and RhoA. An immunofluorescence study was also performed to evaluate the expression and localization of RhoA. RESULTS: The relative mRNA expression levels of pulmonary Wnt11 and RhoA on D21 were significantly increased in the CDH group compared with the control group (p=0.016 and p=0.008, respectively). The immunofluorescence study confirmed the overexpression of RhoA in the pulmonary vessels of CDH rats on D21. CONCLUSIONS: Our results provide evidence that the RhoA/Rho-kinase-mediated pathway is involved in the pathogenesis of PH in the nitrofen-induced CDH rat model. Our data also suggest that the fasudil, a Rho-kinase inhibitor, could represent a therapeutic option for the treatment of PH in CDH.
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Hérnias Diafragmáticas Congênitas/genética , Pulmão/metabolismo , Prenhez , RNA Mensageiro/genética , Proteína rhoA de Ligação ao GTP/genética , Animais , Modelos Animais de Doenças , Feminino , Hérnias Diafragmáticas Congênitas/induzido quimicamente , Hérnias Diafragmáticas Congênitas/metabolismo , Éteres Fenílicos/toxicidade , Gravidez , Ratos , Ratos Sprague-Dawley , Reação em Cadeia da Polimerase em Tempo Real , Proteína rhoA de Ligação ao GTP/biossínteseRESUMO
Nevoid basal cell carcinoma syndrome (NBCCS) is characterized by basal cell carcinoma, skeletal abnormalities, benign tumors including ovarian fibroma, and various other phenotypic expressions. Ovarian fibromas in NBCCS before puberty are very rare. We report a 6-year-old prepubescent girl with NBCCS showing skeletal abnormalities, medulloblastoma, and ovarian fibromas. The patient was referred to our hospital owing to abdominal distension. On admission, a huge elastic hard tumor was palpable and computed tomography showed a huge tumor of the left ovary. We performed a left salpingo-oophorectomy and diagnosed the tumor as a benign fibroma. Further examination of the computed tomography images showed skeletal abnormalities. In addition, the patient had a history of medulloblastoma at the age of 4 years. Therefore, we diagnosed NBCCS. A genetic examination indicated a novel 1 bp deletion in exon 18 (c.3055delG). Sequence analysis of exon 18 using DNA from the ovarian tumor revealed a mutant allele (c.3055delG) dominant to the wild-type allele, thus suggesting loss of heterozygosity in the PTCH1 gene, which is known to be associated with NBCCS. Conclusion On the basis of our experience, physicians treating pediatric ovarian tumors should be aware that such huge benign ovarian tumors may be a phenotype of NBCCS, as shown in our patient. In addition, genetic examination focusing on the PTCH1 gene might be important for diagnosis of NBCCS in pediatric patients.
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Síndrome do Nevo Basocelular/diagnóstico , Fibroma/diagnóstico , Neoplasias Ovarianas/diagnóstico , Criança , Diagnóstico Diferencial , Diagnóstico por Imagem , Feminino , Fibroma/cirurgia , Humanos , Japão , Neoplasias Ovarianas/cirurgiaRESUMO
BACKGROUND/PURPOSE: The aim of this study is to clarify the clinical features and risk factors of pre- and postoperative pancreatic complications in pediatric choledochal cysts. METHODS: A retrospective chart review was carried out on pediatric patients with choledochal cysts who underwent radical operation at our department. RESULTS: Twenty-one, 24, and 24 patients were classified into the Todani Ia, Ic, and IV-A choledochal cyst, respectively. Preoperative acute pancreatitis and protein plugs were observed in 31 (43.7%) and 11 (15.5%) patients, respectively. Patients with preoperative pancreatitis were more likely to have fusiform dilatation of choledochal cysts (79.3% vs. 35.0%) and a dilated common channel (53.9% vs. 23.1%) compared to those without preoperative pancreatitis. Compared to patients without preoperative protein plugs, those with protein plugs were more likely to have fusiform dilatation (90.9% vs. 46.5%) and pancreatic divisum with communicating ducts and a dilated ductal system (60.0% vs. 2.5%). Postoperatively, three patients (4.2%) experienced acute pancreatitis. One of these and all 3 had protein plugs and preoperative pancreatitis, respectively. CONCLUSIONS: Fusiform-type choledochal cyst is a significant risk factor for preoperative pancreatic complications in choledochal cysts. While postoperative pancreatic complications were relatively rare, preoperative pancreatic complications might be risk factors for postoperative pancreatitis.
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Cisto do Colédoco/cirurgia , Pancreatite/epidemiologia , Complicações Pós-Operatórias/epidemiologia , Amilases/sangue , Anastomose em-Y de Roux , Bilirrubina/sangue , Criança , Pré-Escolar , Cisto do Colédoco/classificação , Cisto do Colédoco/complicações , Cisto do Colédoco/patologia , Colestase/sangue , Colestase/complicações , Colestase/epidemiologia , Colestase/patologia , Dilatação Patológica/etiologia , Dilatação Patológica/patologia , Feminino , Humanos , Lactente , Jejunostomia , Fígado/cirurgia , Masculino , Pancreatite/sangue , Pancreatite/etiologia , Pancreatite/patologia , Pancreatite Crônica/sangue , Pancreatite Crônica/epidemiologia , Pancreatite Crônica/etiologia , Pancreatite Crônica/patologia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Fatores de Risco , Transaminases/sangue , gama-Glutamiltransferase/sangueRESUMO
We report the case of 2-week-old female infant with cystic lung disease who presented with mild tachypnea and had no history of mechanical ventilation. Chest CT demonstrated multiple air-filled cystic lesions in right upper lobe, and the patient subsequently underwent a right upper lobectomy. Histology revealed cystic lesions located in the pulmonary parenchyma and showed that the lesions were lined by lymphatic endothelium and were communicating with dilated lymphatic vessels in the interstitium. Additionally, multinucleated foreign body giant cells were attached to the lumen of the cyst. On the basis of these findings, we considered this a case of persistent interstitial pulmonary emphysema (PIPE) with massive pneumatic expansion of the lymphatic vessels, resulting in cystic lesions with lymphatic endothelium in the pulmonary parenchyma. While PIPE is extremely rare in term non-ventilated infants, our case demonstrated that this disease should be added to the differential diagnosis of cystic lung diseases with lymphatic endothelium even in infants without mechanical ventilation. When cystic lesions and symptoms persist despite conservative treatment, open or thoracoscopic resection is an appropriate option for diagnosis and treatment.
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Malformação Adenomatoide Cística Congênita do Pulmão/cirurgia , Doenças Pulmonares Intersticiais/cirurgia , Vasos Linfáticos/diagnóstico por imagem , Vasos Linfáticos/patologia , Enfisema Pulmonar/cirurgia , Biópsia por Agulha , Malformação Adenomatoide Cística Congênita do Pulmão/diagnóstico por imagem , Malformação Adenomatoide Cística Congênita do Pulmão/patologia , Feminino , Seguimentos , Humanos , Imuno-Histoquímica , Recém-Nascido , Doenças Pulmonares Intersticiais/congênito , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Pneumonectomia/métodos , Enfisema Pulmonar/congênito , Enfisema Pulmonar/diagnóstico por imagem , Radiografia Torácica/métodos , Doenças Raras , Medição de Risco , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios X/métodos , Resultado do TratamentoRESUMO
Pancreatitis, a late complication of an annular pancreas (AP), results from coexisting pancreaticobiliary malformations including pancreas divisum (PD), and pancreaticobiliary maljunction (PBM). The authors report the case of a 3-year-old boy with an unusual type of AP in which the dorsal anlage encircled the duodenum. The patient developed duodenal obstruction as well as duodenopancreatic reflux with resulting hyperamylasemia and hyperlipasemia. This type of AP associated with duodenopancreatic reflux in AP has not been reported previously. The patient was successfully treated by duodenoduodenostomy, which, by correcting the duodenopancreatic reflux, prevented the later development of pancreatitis.
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Refluxo Duodenogástrico/etiologia , Pancreatopatias/complicações , Pré-Escolar , Colangiopancreatografia Retrógrada Endoscópica/métodos , Obstrução Duodenal/diagnóstico , Obstrução Duodenal/etiologia , Obstrução Duodenal/cirurgia , Refluxo Duodenogástrico/diagnóstico , Refluxo Duodenogástrico/cirurgia , Duodenostomia/métodos , Seguimentos , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Pâncreas/anormalidades , Pâncreas/diagnóstico por imagem , Pâncreas/patologia , Pâncreas/cirurgia , Pancreatopatias/diagnóstico , Pancreatopatias/cirurgiaRESUMO
Cloacal dysgenesis sequence (CDS) is a rare and lethal malformation. We report such a case of long-term survival, currently to 12 years of age. In the fetal period, she received a timely placement of vesico-amniotic shunt for a megabladder due to a severe urethral obstruction. Postnatally, cystostomy and colostomy were created because of no perineal opening of urethra, vagina, and anus. Anorectoplasty, construction of efferent conduit, and colostomy closure were performed at 4 years of age. Ileovaginoplasty and ileovesicostomy which was a Mitrofanoff-type of conduit, and labioplasty were performed at the age of 11 years. To the best of our knowledge, only five survivors with CDS over 1 year of age have been reported.
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Anormalidades Múltiplas , Canal Anal/anormalidades , Cloaca/anormalidades , Procedimentos de Cirurgia Plástica/métodos , Uretra/anormalidades , Vagina/anormalidades , Adulto , Canal Anal/cirurgia , Cloaca/embriologia , Cloaca/cirurgia , Colostomia/métodos , Diagnóstico Diferencial , Feminino , Seguimentos , Humanos , Recém-Nascido , Gravidez , Resultado da Gravidez , Fatores de Tempo , Ultrassonografia Pré-Natal , Uretra/cirurgia , Vagina/cirurgiaRESUMO
PURPOSE: The aim of the study was to identify the clinical characteristics and outcome of patients with liver fibrosis in choledochal cyst (CC). METHODS: Forty patients with CC who underwent liver biopsy were included. Liver fibrosis was classified as follows: grade 0, no fibrosis; grade 1, mild fibrosis localized in the portal area; grade 2, moderate fibrosis with occasional bridging; and grade 3, severe fibrosis with diffuse bridging. RESULTS: Fourteen patients (35%) had liver fibrosis. Patients in the fibrosis group were significantly younger (1.2 vs 2.7 years) and had higher total bilirubin (5.3 vs 2.6 mg/dL). Severity of liver fibrosis was inversely correlated with age (P = .044). Amylase and lipase in bile were significantly lower in the fibrosis group (amylase, 531 vs 15,000 U/L; lipase, 783 vs 23,100 U/L). Postoperative serum analysis demonstrated no differences between the two groups. Most patients in both groups had normal aspartate aminotransferase, alanine aminotransferase, total bilirubin, and γ-glutamyl transpeptidase regardless of severity of fibrosis. Postoperative biliary complication or cholangiocarcinoma was not found in the fibrosis group. CONCLUSIONS: Our data suggest that liver fibrosis is mainly influenced by obstructive cholangiopathy rather than refluxed pancreatic secretion. Prognosis of patients with CC and liver fibrosis was as good as that of patients without fibrosis.
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Cisto do Colédoco/complicações , Cirrose Hepática/etiologia , Dor Abdominal/etiologia , Alanina Transaminase/sangue , Amilases/análise , Aspartato Aminotransferases/sangue , Bile/enzimologia , Biópsia , Pré-Escolar , Cisto do Colédoco/cirurgia , Feminino , Humanos , Hiperbilirrubinemia/etiologia , Lactente , Lipase/análise , Cirrose Hepática/sangue , Cirrose Hepática/fisiopatologia , Cirrose Hepática/cirurgia , Masculino , Suco Pancreático/enzimologia , Prognóstico , Estudos Retrospectivos , Índice de Gravidade de Doença , Resultado do Tratamento , gama-Glutamiltransferase/sangueRESUMO
PURPOSE: The aim of this study is to investigate the clinical characteristics of cases of duodenal atresia (DA) which present with bowel gas distal to a typical double-bubble sign through an anomalous bile duct conduit. METHODS: Medical records of 57 neonates with duodenal obstruction (atresia or stenosis), presenting with a double-bubble sign and treated at our institute from 1978 to 2010, were retrospectively reviewed. RESULTS: Thirteen (23%) of 57 neonates presented with bowel gas distal to the double-bubble sign. Passage of gas through the duodenal stenosis may have occurred in 3 cases, whereas in 9 cases, gas may have bypassed the atresia through an anomalous bifurcated bile duct termination and through the pancreatic duct from the accessory to the main pancreatic duct in one case. A preoperative upper gastrointestinal series was performed in 9 cases, and an anomalous bifurcated bile duct conduit was demonstrated in 5 cases. Severe and prolonged cholestasis necessitating evaluation for biliary atresia was found in 2 patients with anomalous bile duct anatomy. CONCLUSIONS: Neonatal DA presenting with distal bowel gas via an anomalous bifurcated bile duct conduit is more common than initially thought and occurs more frequently than duodenal stenosis. These patients might be at risk for cholestasis, possibly owing to duodeno-biliary reflux through the ampulla.