RESUMO
PURPOSE: Duplication enucleation (DE) has been described as an alternative to intestinal resection with primary anastomosis (IRA) for intestinal duplications, but no comparative study exists. The aim of this study was to compare both surgical procedures for intestinal duplication. METHODS: A retrospective study was performed, including all children treated for intestinal duplication (2005-2023). Patients that underwent DE were compared to those that underwent IRA. Statistical significance was determined using p < 0.05. Ethical approval was obtained. RESULTS: A total of 51 patients (median age: 5 months) were treated for intestinal duplication, including 27 patients (53%) that underwent DE and 24 IRA (47%). A cystic image was detected prenatally in 19 patients (70%) with DE and 11 patients (46%) with IRA (p = 0.09). Enucleation was performed using laparoscopy in 7 patients (14%). Patients that underwent DE had shorter time to first feed (1 vs 3 days, p = 0.0001) and length of stay (4 vs 6 days, p < 0.0004) compared to IRA. A muscular layer was identified in 68% of intestinal resection specimens. CONCLUSION: Compared to intestinal resection with anastomosis, duplication enucleation is associated with decreased postoperative length of stay and delay to first feeds without increasing post-operative complications. Regarding histological analysis, enucleation seems feasible in most cases.
Assuntos
Anastomose Cirúrgica , Intestinos , Laparoscopia , Humanos , Estudos Retrospectivos , Anastomose Cirúrgica/métodos , Feminino , Masculino , Lactente , Intestinos/cirurgia , Intestinos/anormalidades , Laparoscopia/métodos , Pré-Escolar , Resultado do Tratamento , Tempo de Internação/estatística & dados numéricos , Recém-Nascido , Procedimentos Cirúrgicos do Sistema Digestório/métodos , CriançaRESUMO
INTRODUCTION: Intestinal malrotation is a congenital pathology with potentially catastrophic complications, such as volvulus, whose treatment has barely not changed in nearly 100 years (Ladd's procedure). Dr. Abu-Elmagd recently described a new technique that was applied in one of our patients. CLINICAL CASE: 12-year-old boy who had undergone Ladd's procedure as a result of intestinal volvulus secondary to malrotation when he was 2 days old. He had subocclusion and eventually obstruction, with intestinal volvulus compatible imaging. Intraoperative findings: duodenal subocclusion, volvulus and lymphangiectasias. Kareem's procedure: bowel positioning in normal rotation, duodenopexy (duodenal C posterior to the mesenteric vessels), formation of neo-Treitz, and fixation of the cecum, the ascending colon, and the mesenteric root. The patient was discharged on postoperative day 6 and remains asymptomatic after 1 year of follow-up. DISCUSSION: Kareem's procedure is a safe and effective malrotation repair technique. It can replace Ladd's procedure as it reduces the risk of re-volvulation and improves digestive symptoms.
INTRODUCCION: La malrotación intestinal es una patología congénita con complicaciones potencialmente catastróficas, destacando el vólvulo, cuyo tratamiento no ha variado significativamente en casi 100 años (procedimiento de Ladd). Recientemente el Dr. Abu-Elmagd describió una técnica que hemos aplicado en un paciente. CASO CLINICO: Varón de 12 años, intervenido con 2 días de vida por vólvulo intestinal secundario a malrotación realizándose procedimiento de Ladd. Presenta cuadros suboclusivos y finalmente obstructivo con imágenes compatibles con vólvulo intestinal. Hallazgos intraoperatorios: suboclusión duodenal, vólvulo y linfangiectasias. Procedimiento de Kareem: posicionando intestino en normorotación, duodenopexia (C duodenal posterior a vasos mesentéricos), formación de neoTreitz, fijación de ciego, colon ascendente y raíz mesentérica. Alta hospitalaria al 6º día postoperatorio, asintomático en 1 año de seguimiento. COMENTARIOS: El procedimiento de Kareem es una técnica segura y efectiva que corrige la malrotación, pudiendo reemplazar al procedimiento de Ladd al disminuir el riesgo de revolvulación y mejorar síntomas digestivos.
Assuntos
Procedimentos Cirúrgicos do Sistema Digestório , Volvo Intestinal , Laparoscopia , Masculino , Humanos , Criança , Volvo Intestinal/cirurgia , Volvo Intestinal/complicações , Intestinos/anormalidades , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Laparoscopia/métodosRESUMO
PURPOSE: Heterotaxy syndrome (HS) affects right-left anatomical development in 3% of children with congenital heart disease. Commonly, these patients have intestinal rotation abnormalities (IRA) that differ from typical malrotation. In this prospective study, we examine the development of a management pathway, imaging findings, and clinical course of patients with HS and IRA. METHODS: After literature review, a multispecialty focus group designed a pathway for HS. Participants from a single institution were prospectively enrolled from 2016 to 2019. They underwent an abdominal ultrasound and UGI, however timing was variable based on symptoms. Symptomatic IRA was defined as feeding intolerance, bilious or non-bilious vomiting, bloating, severe reflux, and intermittent pain or abdominal distention. Screening for symptoms occurred every three months for the first two years and then annually. RESULTS: 18 participants were followed for a median of 5.0 years. Three clinical categories were identified: 1) asymptomatic, not requiring intestinal surgery, 72.2%, 2) symptomatic with feeding intolerance or failure to thrive requiring gastrostomy placement and diagnostic laparoscopy with Ladd procedure, 16.7%, and 3) symptomatic requiring urgent exploration for suspicion of volvulus, 11.1%. Need for surgery did not necessarily correlate with US and/or UGI findings. CONCLUSIONS: In children with HS and IRA, postnatal imaging did not inform the need for intestinal surgery. Although rare, volvulus or other forms of proximal obstruction can be anticipated, and symptomatic patients should be offered surgical intervention. A multidisciplinary care pathway for patients with HS and IRA can coordinate care and help counsel families on the likelihood of needing intestinal surgery for IRA. LEVEL OF EVIDENCE: Level II. TYPE OF STUDY: Prospective Cohort Study.
Assuntos
Anormalidades do Sistema Digestório , Síndrome de Heterotaxia , Obstrução Intestinal , Volvo Intestinal , Criança , Humanos , Anormalidades do Sistema Digestório/diagnóstico por imagem , Anormalidades do Sistema Digestório/cirurgia , Gastrostomia , Síndrome de Heterotaxia/diagnóstico por imagem , Síndrome de Heterotaxia/cirurgia , Volvo Intestinal/diagnóstico por imagem , Volvo Intestinal/cirurgia , Intestinos/diagnóstico por imagem , Intestinos/cirurgia , Intestinos/anormalidades , Estudos ProspectivosRESUMO
Abstract The current investigation was used to improve the rate of dissolution of an anti-diarrheal drug i.e., racecadotril (RT) at low pH conditions (i.e., in the stomach) by reducing the water secretion and electrolyte in to the intestine by liquisolid tablets. Different formulations (liquisolid) were prepared using Avicel PH 102 as a carrier. Aerosil 200 as a coating material and sodium starch glycolate used as a disintegrant. Polyethylene glycol 200 was used as a non-volatile vehicle to dissolve the drug. FTIR, DSC, XRD and dissolution studies were conducted to characterise liquisolid tablets. Characterisation studies indicated that no interactions between carrier and drug. Solid state characterization had shown a reduction in crystallinity that further supports increment in solubility and dissolution. The optimised formulation showed a significant increase in dissolution i.e., 99.54±0.62% in 30 min compared to directly compressible tablets (38.47±0.26%). The % dissolution efficiency of racecadotril liquisolid tablets 76.86% compared to marketed tablet (27.56%) and conventional direct compression tablet (17.11%). Significant reduction in mean dissolution time of racecadotril from liquisolid tablets (6.84 min) compared to direct compression tablet (44.57 min), indicating faster release of drug and faster onset of action. Formulation of liquisolid tablets could enhance solubility, dissolution and bioavailability of racecadotril
Assuntos
Dissolução , Antidiarreicos/análise , Estômago/anormalidades , Preparações Farmacêuticas/análise , Celulose/agonistas , Intestinos/anormalidadesRESUMO
Abstract Inflammatory bowel disease (IBD) is a chronic inflammatory disorder of the intestine, demonstrating an increasing incidence every year. TongXieYaoFang (TXYF) has been used widely in China as a complementary therapy to relieve the symptoms of IBD for hundreds of years. In the present research, a network pharmacology-based approach was used to systematically explore the intrinsic mechanisms of TXYF in IBD at the molecular level. Network pharmacology-based methods, which mainly included database mining, screening of bioactive compounds, target prediction, collection of IBD-related targets, gene enrichment analysis, network construction, and molecular docking, were employed in the present study. Network analysis revealed a total of 108 potential targets derived from 22 component compounds of TXYF, among which 34 targets were common with the IBD-related targets. In the protein-protein interaction (PPI) network, 10 key targets were identified. The gene enrichment analysis suggested that anti-inflammatory processes, such as NF-kappa B signaling pathway and Toll-like receptor signaling pathway, could be the core processes involved in the action of TXYF in IBD. Molecular docking results revealed that three compounds present in TXYF exhibited strong binding affinity for PTGS2. The present study provides novel insights into the molecular mechanisms and network approaches of TXYF action in IBD from a systemic perspective. The potential targets and pathways identified in the present study would assist in further research on the clinical application of TXYF in IBD therapy.
Assuntos
Doenças Inflamatórias Intestinais/patologia , Intestinos/anormalidades , Medicina Tradicional Chinesa/métodos , NF-kappa B , Receptores Toll-Like , Farmacologia em Rede/instrumentaçãoRESUMO
La malrotación intestinal es un espectro de malformaciones, que incluye una gran variedad de alteraciones en el proceso de rotación y fijación del intestino. La ausencia completa de rotación intestinal, uno de los tipos de malrotación intestinal más frecuentes, puede presentarse como hallazgo asintomático o manifestarse clínicamente por un vólvulo del intestino medio. Sin embargo, incluso entre aquellos que se presentan con vólvulo del intestino medio, la clínica puede ser muy diferente, según el grado de isquemia y eventual necrosis intestinal. Se presentan dos casos clínicos de malrotación intestinal con vólvulo del intestino medio con presentaciones, imágenes, tratamientos y evoluciones muy disímiles. Se analizan los mismos a la luz de una revisión bibliográfica relevante al tema tratado, se sacan aprendizajes del manejo realizado y la evolución que presentaron, y se enfatizan los elementos de mayor jerarquía para optimizar el manejo de estos pacientes.
Intestinal malrotation is a spectrum of malformations that includes a great variety of alterations in the rotation and fixation process of the intestines. The total absence of intestinal rotation, one of the most frequents types of intestinal malrotation could present as an asymptomatic find or appear clinically as a midgut volvulus. However, even those that appear as midgut volvulus, could show quite different signs and symptoms according to the degree of ischemic insult and possible intestinal necrosis. Two clinical cases of intestinal malrotation with midgut volvulus with quite different presentations, images, treatments, and evolutions, are informed. The two cases are analyzed under a relevant bibliographic revision, knowledge is derived from the carried-out management and evolution, and elements for the future optimization of management are underlined.
A má rotação intestinal é um espectro de malformações, que inclui uma grande variedade de alterações no processo de rotação e fixação do intestino. A ausência completa de rotação intestinal, um dos tipos mais comuns de má rotação intestinal, pode se apresentar como um achado assintomático ou manifestar-se clinicamente como um volvo de intestino médio. Porém, mesmo entre aqueles que apresentam volvo de intestino médio, os sintomas podem ser muito diferentes, dependendo do grau de isquemia e eventual necrose intestinal. Dois casos clínicos de má rotação intestinal com volvo de intestino médio são apresentados com apresentações, imagens, tratamentos e evoluções muito diferentes. São analisados à luz de uma revisão bibliográfica pertinente ao tema em questão, lições aprendidas com o manejo realizado e a evolução que apresentaram, e os elementos de maior hierarquia são enfatizados para otimizar o manejo desses pacientes.
Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Lactente , Volvo Intestinal/cirurgia , Volvo Intestinal/diagnóstico por imagem , Resultado do Tratamento , Intestinos/anormalidadesRESUMO
RATIONALE: Gastrointestinal tract duplication is a rare congenial anomaly which can be found anywhere along the gastrointestinal tract. While many patients are incidentally diagnosed during operation, in some cases it can present with severe gastrointestinal symptoms. In this case report, the patient presented with signs of toxic megacolon leading to rapid aggravation of inflammatory shock. PATIENT CONCERNS: A 49-day old male infant presented with fever, poor feeding, and severe abdominal distension. DIAGNOSIS: Abdominal ultrasonography was done. During the examination, a foley catheter was inserted through the anus to evaluate bowel patency and enable rectal decompression. The tip of the foley catheter was located in a separate narrower tubular lumen adjacent to the distended rectum. These findings suggested possibility of a tubular duplication cyst of the rectum as the culprit for the bowel obstruction. INTERVENTIONS: The patient underwent emergency laparotomy. Findings showed multiple tubular intestinal duplications involving the ileum, appendix, cecum, descending colon, sigmoid colon and rectum. The true lumen of the rectosigmoid colon was completely collapsed while the adjacent tubular cyst remained severely distended and stool passage was not possible. Decompression of the sigmoid colon was done with loop colostomy with both the wall of the true bowel and enteric cyst forming the colostomy orifice. OUTCOMES: After 40âdays of postoperative care, the patient was discharged with no immediate complications. Four months after the initial operation, colostomy take-down and transanal rectal common wall division was done. No complications were observed. LESSONS: To our knowledge, this is the first case to be reported where a rare presentation of intestinal duplication resulted in an acute presentation toxic megacolon. Such emergency cases can be effectively treated with emergency surgical bowel decompression and elective common wall division.
Assuntos
Anormalidades do Sistema Digestório/complicações , Tratamento de Emergência/métodos , Obstrução Intestinal/diagnóstico , Intestinos/anormalidades , Megacolo Tóxico/diagnóstico , Colostomia , Descompressão Cirúrgica/métodos , Anormalidades do Sistema Digestório/diagnóstico , Anormalidades do Sistema Digestório/cirurgia , Humanos , Lactente , Obstrução Intestinal/etiologia , Obstrução Intestinal/cirurgia , Intestinos/diagnóstico por imagem , Intestinos/cirurgia , Laparotomia , Masculino , Megacolo Tóxico/etiologia , Megacolo Tóxico/cirurgia , Resultado do Tratamento , UltrassonografiaRESUMO
Background: Malrotation is a common congenital anomaly that can lead to bowel obstruction and ischemia if not corrected with a Ladd procedure. Controversy exists between open and laparoscopic approaches. We sought to compare postoperative outcomes and determine risk factors for conversion to an open procedure. Methods: The National Surgical Quality Improvement Program (NSQIP)-Pediatric was used to identify patients undergoing Ladd procedures from 2013 to 2018. Propensity score matching was used to account for differences in patient characteristics between open and laparoscopically treated cohorts. Chi-square tests and adjusted logistic regression analysis were used to determine patient outcomes differences between treatment groups and factors associated with conversion. Results: A total of 2437 patients were identified, 1889 (77.5%) open, 548 (22.5%) laparoscopic, and 193 (35.2%) laparoscopic converted to open. Patients undergoing laparoscopic compared with open procedures had shorter length of stay (5 versus 7 days, P < .001) and lower overall complication rates (13.1% versus 18.1%, P = .025), despite longer operative times (108.9 versus 93.7 minutes, P < .001). Patients requiring conversion were more likely to be younger, have an urgent/emergent case, sepsis/septic shock, and nutritional support requirement. Conclusions: After risk adjustment, laparoscopic Ladd procedure is associated with decreased complications and minimal operative time increases compared with an open approach. Risk factors associated with conversion should be considered during operative planning.
Assuntos
Conversão para Cirurgia Aberta , Anormalidades do Sistema Digestório/cirurgia , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Intestinos/anormalidades , Laparoscopia , Fatores Etários , Criança , Pré-Escolar , Procedimentos Cirúrgicos do Sistema Digestório/efeitos adversos , Emergências , Feminino , Humanos , Lactente , Recém-Nascido , Laparoscopia/efeitos adversos , Tempo de Internação , Masculino , Apoio Nutricional , Duração da Cirurgia , Complicações Pós-Operatórias/etiologia , Pontuação de Propensão , Estudos Retrospectivos , Fatores de Risco , Choque Séptico/complicaçõesRESUMO
There are some incidental findings during bariatric surgery. Some of these findings may make the operation more challenging than routine surgery. Intestinal malrotation/non-rotation are rare congenital anomalies that may remain asymptomatic until bariatric surgery. Here we report a 30-year-old female patient with unknown intestinal congenital anomaly which was diagnosed during surgery and she underwent Roux-en-Y gastric bypass, which can be a challenging procedure in intestinal malrotation/non-rotation and conversion may be needed.
Assuntos
Anormalidades do Sistema Digestório/cirurgia , Derivação Gástrica/métodos , Intestinos/cirurgia , Obesidade/cirurgia , Adulto , Cirurgia Bariátrica , Anormalidades do Sistema Digestório/complicações , Feminino , Humanos , Intestinos/anormalidades , Laparoscopia , Obesidade/complicaçõesRESUMO
BACKGROUND: Malrotation is a congenital anomaly most often affecting the pediatric population. The Ladd procedure is the standard treatment for this pathology. Well-studied in the pediatric population, large studies of the demographics and outcomes of patients who reach adulthood are lacking. METHODS: An analysis of the American College of Surgeons National Surgical Quality Improvement Program (ACS NSQIP) database (2015-2018) was performed, capturing patients with a post-operative diagnosis of malrotation and who underwent surgical correction with or without appendectomy, excluding those who underwent other major procedures such as colectomy. Baseline demographics and outcomes were compared. The primary outcome was mortality. Secondary outcomes such as length of stay and discharge destination were included. RESULTS: Two hundred twenty patients undergoing surgical correction of malrotation were captured, all of which were performed by a general surgeon under general anesthesia. One hundered and nine (49.55%) of these patients also underwent an appendectomy. Most of these patients were female (68.18%). Comorbidities and perioperative variables were clinically similar. Operative time was similar between the two groups (112 ± 86 vs. 98 ± 49 min, p = 0.1385). Thirty-day mortality (1.36%), length of stay (4.79 ± 6.21 days), readmission rate (13.64%), wound infection (2.27%) and discharge destination (95.00% to home) were statistically similar between groups. CONCLUSIONS: The data describes demographics and outcomes in adults undergoing Ladd procedures with and without appendectomy. Immediate outcomes may be equivalent regardless of incidental appendectomy. Further work is necessary to describe the population of adults with malrotation reaching adulthood.
Assuntos
Procedimentos Cirúrgicos do Sistema Digestório , Intestinos/anormalidades , Adulto , Idoso , Apendicectomia , Bases de Dados Factuais , Procedimentos Cirúrgicos do Sistema Digestório/efeitos adversos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias , Melhoria de Qualidade , Estudos Retrospectivos , Rotação , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND: Laparoscopic approach for malrotation has become more popular for neonates and in cases with volvulus, but its safety and efficacy remains controversial. This study reviewed laparoscopy outcomes in neonate/infant malrotation. METHODS: Medline/PubMed and Lilacs databases were reviewed. Data from studies published in English/Spanish between 1995 and 2019 were collected. Results are presented as percentages and means/medians; logistic regression was used to study possible associations. RESULTS: Nineteen papers offered 99 neonates/infants with median age and weight of 10.5 days and 3.5 kg, respectively. Ladd's procedure was performed in 95 (96%) patients and bands' division in 4 (4%); appendectomy was not included in 16 (16.2%) patients, and cecopexy was not performed in all cases. Volvulus was reported in 39 (39.4%) patients. There were 11 conversions (11.1%) and 10 recurrences of symptoms (10.1%) that required reintervention. An association was found between volvulus and recurrence (P = .05) and the need for conversion (P < .01). There were 10 (10.1%) minor complications and no mortality. The median follow-up was 10 months. DISCUSSION: Laparoscopic approach to malrotation is feasible and safe in hemodynamically stable neonates/infants without intestinal necrosis and is associated with 11% conversion rate and 10% reinterventions. The presence of volvulus is associated with recurrence and conversion. Laparoscopic Ladd's procedure with appendectomy and without cecopexy is the commonly practiced approach that is associated with minor complications.
Assuntos
Intestinos/anormalidades , Laparoscopia/métodos , Conversão para Cirurgia Aberta/estatística & dados numéricos , Humanos , Lactente , Recém-Nascido , Intestinos/cirurgia , Reoperação/estatística & dados numéricos , Resultado do TratamentoRESUMO
Malrotation occurs in approximately 1 in 500 live births. However, the true incidence of malrotation is unknown since many asymptomatic patients fail to present. Approximately 90% of patients with malrotation are diagnosed within the 1st year of life. Eighty per cent of them are diagnosed within the 1st month of life. Nevertheless, there are recent reports of manifestations later in life both as emergency conditions and more chronic gastrointestinal symptoms. The relationship between malrotation and horseshoe kidney has not been fully understood, but few case reports have highlighted their occurrence in the same patient. The mode of presentation of this case and its association with a horseshoe kidney is the reason for this report. This was a case of malrotation associated with horseshoe kidney. He had exploratory laparotomy and Ladd's procedure. Malrotation is associated with horseshoe kidney which presented as gastric outlet obstruction. He responded well to treatment after Ladd's procedure.
Assuntos
Anormalidades Múltiplas , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Rim Fundido/diagnóstico , Obstrução da Saída Gástrica/etiologia , Intestinos/anormalidades , Laparotomia/métodos , Adolescente , Rim Fundido/complicações , Obstrução da Saída Gástrica/diagnóstico , Obstrução da Saída Gástrica/cirurgia , Humanos , MasculinoRESUMO
Congenital bands are rare causes of intestinal obstruction and often leads to diagnostic challenges. Diagnostic delays in cases of mechanical obstruction might lead to irreversible bowel ischemia and perforation. Presently described is a case of an 18 month young child with severe vomiting developed for one day. The child was initially thought to have acute viral enteritis and treated accordingly. Due to the severity, an X-Ray and computed tomography scan were sent which pointed towards the possibility of having congenital bands. He was treated operatively. The child was kept under observation for eleven days and was discharged. Although rare, intestinal obstruction due to congenital bands must be considered when treating a child with severe vomiting. Keywords: case reports, congenital abnormalities, intestinal obstruction, vomiting.
Assuntos
Anormalidades do Sistema Digestório , Obstrução Intestinal , Intestinos , Procedimentos Cirúrgicos Operatórios/métodos , Tomografia Computadorizada por Raios X/métodos , Diagnóstico Diferencial , Anormalidades do Sistema Digestório/diagnóstico , Anormalidades do Sistema Digestório/fisiopatologia , Anormalidades do Sistema Digestório/cirurgia , Humanos , Lactente , Obstrução Intestinal/congênito , Obstrução Intestinal/diagnóstico , Obstrução Intestinal/fisiopatologia , Obstrução Intestinal/cirurgia , Intestinos/anormalidades , Intestinos/diagnóstico por imagem , Intestinos/cirurgia , Masculino , Radiografia Abdominal/métodos , Resultado do Tratamento , Vômito/diagnóstico , Vômito/etiologiaRESUMO
OBJECTIVE: To determine the key sonographic features for the diagnosis of sirenomelia in the first trimester of pregnancy. METHODS: Cases of sirenomelia from several prenatal diagnosis centers were retrospectively identified and reviewed. The diagnosis was established through the detection of fused lower limbs. Additional sonographic findings were also noted. RESULTS: A total of 12 cases were collected. The most striking sonographic finding was the detection of malformed lower limbs, which appeared to be fused and in an atypical position. Nuchal translucency thickness was mildly increased in three cases (25%). An abdominal cyst, representing the dilated blind-ending bowel, was noted in seven cases (58%). Color flow imaging detected a single umbilical artery in six cases (50%) and the associated intra-abdominal vascular anomalies in three cases (25%). No cases of aneuploidy were detected. The pregnancy was terminated in nine cases (75%) and intrauterine demise occurred in the remaining three cases (25%). CONCLUSIONS: The sonographic detection of abnormal lower limbs or an intra-abdominal cyst located laterally during the first-trimester scan may be warning signs of sirenomelia. This should prompt a detailed examination of the fetal lower body and intra-abdominal anatomy, including the main abdominal vessels, in order to look for additional confirmatory findings.
Assuntos
Anormalidades do Sistema Digestório/diagnóstico por imagem , Ectromelia/diagnóstico por imagem , Deformidades Congênitas das Extremidades Inferiores/diagnóstico por imagem , Artéria Umbilical Única/diagnóstico por imagem , Malformações Vasculares/diagnóstico por imagem , Aborto Induzido , Adulto , Feminino , Morte Fetal , Humanos , Intestinos/anormalidades , Intestinos/diagnóstico por imagem , Medição da Translucência Nucal , Gravidez , Primeiro Trimestre da Gravidez , Ultrassonografia Doppler em Cores , Ultrassonografia Pré-NatalRESUMO
Medulloblastoma in a Patient with Curry-Jones Syndrome with a mosaic variant, c.1234C > T (p.Leu412Phe), in SMO.
Assuntos
Anormalidades Craniofaciais/diagnóstico , Predisposição Genética para Doença , Intestinos/anormalidades , Meduloblastoma/diagnóstico , Anormalidades da Pele/diagnóstico , Receptor Smoothened/genética , Sindactilia/diagnóstico , Anormalidades Craniofaciais/genética , Anormalidades Craniofaciais/patologia , Humanos , Lactente , Recém-Nascido , Intestinos/patologia , Masculino , Meduloblastoma/genética , Meduloblastoma/patologia , Mutação , Anormalidades da Pele/genética , Anormalidades da Pele/patologia , Sindactilia/genética , Sindactilia/patologiaRESUMO
Total colonic aganglionosis occurring together with malrotation is a rare occurrence and may pose diagnostic and management dilemmas for the pediatric surgeon. We report the case of a new born, who was operated at the age of three days for malrotation with volvulus, treated by Ladd procedure. Postoperatively, we noticed persistent abdominal distension and emission of a small amount of meconium every 4 to 5 days. The barium enema showed a non-functional microcolon. Surgical exploration on the 24th day found an ileo-ileal transition zone located 60âcm distal to the ligament of Treitz. Extemporaneous biopsies from the colon and mid-ileum confirmed the absence of ganglion cells. We performed an ileostomy at 50âcm from duodeno-jejunal flexure. Unfortunately, the patient succumbed to nosocomial infection at 33 days of age.This case was a challenging scenario for us where a diagnosis of complicated malrotation had obscured the Hirschsprung's disease.
Assuntos
Colo/anormalidades , Doença de Hirschsprung , Ileostomia , Doenças do Recém-Nascido , Obstrução Intestinal , Volvo Intestinal/cirurgia , Intestinos , Enema Opaco/métodos , Biópsia/métodos , Colo/diagnóstico por imagem , Colo/fisiopatologia , Infecção Hospitalar/diagnóstico , Infecção Hospitalar/etiologia , Evolução Fatal , Feminino , Doença de Hirschsprung/diagnóstico , Doença de Hirschsprung/fisiopatologia , Doença de Hirschsprung/cirurgia , Humanos , Ileostomia/efeitos adversos , Ileostomia/métodos , Recém-Nascido , Doenças do Recém-Nascido/diagnóstico , Doenças do Recém-Nascido/fisiopatologia , Doenças do Recém-Nascido/cirurgia , Obstrução Intestinal/diagnóstico por imagem , Obstrução Intestinal/etiologia , Obstrução Intestinal/fisiopatologia , Volvo Intestinal/diagnóstico , Volvo Intestinal/etiologia , Intestinos/anormalidades , Intestinos/inervação , Intestinos/patologia , Intestinos/fisiopatologiaRESUMO
Resumen: La obstrucción duodenal congénita agrupa un amplio espectro de anomalías en el desarrollo del intestino delgado; se clasifica según su etiología tanto en extrínseca como intrínseca. Su presentación es relativamente común, teniendo una incidencia de 1: 2.500-10.000 nacidos vivos y normalmente presenta asociación con aneuploidías (como la trisomía 21) y malformaciones cardíacas. Las imágenes tienen un papel importante en la aproximación diagnóstica y la radiografía de abdomen simple, es el primer escalón en la aproximación de la obstrucción intestinal. El tratamiento involucra una exploración quirúrgica del abdomen con corrección de los defectos encontrados. Este es un reporte de caso de un neonato que presentó obstrucción duodenal congénita de etiología tanto extrínseca (malrotacion intestinal y bandas de Ladd) como obstrucción intrínseca (membranas duodenales).
Abstract: Congenital duodenal obstruction groups together a broad spectrum of abnormalities in the development of the small intestine; it is classified according to its etiology as both extrinsic and intrinsic. Its presentation is relatively common, having an incidence of 1: 2.500-10.000 live births and it is normally associated with aneuploidy (such as trisomy 21) and cardiac malformations. Images play an important role in the diagnostic approach, with simple abdominal radiography being the first step in the approximation of intestinal obstruction. The treatment corresponds to a surgical exploration of the abdomen with resolution of the defects found. This is a case report of a newborn who presented congenital duodenal obstruction of both extrinsic etiology (intestinal malrotation and Ladd bands) and intrinsic obstruction (duodenal membranes).
Assuntos
Humanos , Recém-Nascido , Obstrução Duodenal/congênito , Obstrução Duodenal/diagnóstico por imagem , Radiografia Abdominal , Aderências Teciduais/complicações , Obstrução Duodenal/etiologia , Intestinos/anormalidadesRESUMO
BACKGROUND: Hirschsprung's disease (HSCR) is a common congenital malformation of the enteric nervous system (ENS). During fetal development, ganglion cells of the ENS are derived from neural crest cells that migrate to the bowel. These cells reside principally in two ganglionated plexus: 1) The myenteric plexus, extending from the esophagus to the anus, and 2) submucous plexus, extending from the duodenum to the anus. In large animal species, there is a third plexus called Henle's or Schabadasch's plexus. ENS ganglion cells play a key role in normal gastrointestinal motility, respond to sensory stimuli and regulate blood flow. Both plexus show a high degree of independence from the central nervous system. Alterations in the embryonic development of the ENS can induce multiple pathologies in animal models and humans. CASE PRESENTATION: The present case was a female the fifth born in a litter of 5 puppies. At about 2-3 weeks of age, she suffered from abdominal distension, pain, and constipation. At approximately 8-10 weeks of age, the puppy started to vomit abundantly, and the regurgitated food appeared undigested. Progressive abdominal distention was observed, with quite visible peristaltic movements and more frequent vomiting episodes. The abdominal radiographs, based on AP and side projections, revealed an enlargement of the abdominal diameter and an increased width in the epigastric region. At 12 weeks of age, exploratory surgery revealed a stenotic segment in the jejunum, followed by a small transition zone and then a significantly reduced diameter. Immunohistochemical examinations were performed using antibodies against calretinin, S-100 protein, CD56, neuron specific enolase (NSE) and synaptophysin, which are the biological markers for diagnosing HSCR. CONCLUSION: A reduced number of ganglion cells (1-3 cells per ganglion) were found. There was no specific staining pattern for many of these; while for others, the pattern was compatible with HSCR. Surgical intervention to remove the stenotic section prolonged the life of the puppy for 13 years. Extremely rare pathologies such as that discussed herein should be studied to understand the pathophysiology and be able to diagnose small species in veterinary medicine in a timely fashion. To our knowledge, this is the first report of congenital intestinal stenosis and Hirschprung's disease in a newborn puppy.
Assuntos
Constrição Patológica/veterinária , Doença de Hirschsprung/veterinária , Intestinos/anormalidades , Animais , Animais Recém-Nascidos , Constrição Patológica/cirurgia , Cães , Feminino , Doença de Hirschsprung/diagnóstico , Imuno-Histoquímica/veterinária , Intestinos/cirurgiaRESUMO
BACKGROUND: We examined the association between body mass index (BMI) and development of endoscopic intestinal metaplasia. METHODS: This retrospective cohort study included 142,832 Korean adults free of endoscopic intestinal metaplasia and atrophic gastritis who underwent upper endoscopy at baseline and subsequent visits and were followed for up to 5 years. A parametric proportional hazards model was used to estimate the adjusted HR with 95% confidence interval (CI) for incident intestinal metaplasia. RESULTS: In more than 444,719.1 person-years of follow-up, 2,281 participants developed endoscopic intestinal metaplasia (incidence rate, 5.1 per 1,000 person-years). Increased BMI categories were associated with increased risk of new-onset intestinal metaplasia in a dose-response manner. After adjustment for age, sex, center, year of screening exam, smoking status, alcohol intake, exercise, total calorie intake, history of diabetes and hypertension, and history of Helicobacter pylori infection, the multivariable adjusted HRs (95% CIs) for incident intestinal metaplasia comparing BMIs of <18.5, 23-24.9, 25.0-29.9, and >30 kg/m2 with a BMI of 18.5-22.9 kg/m2 were 0.84 (0.64-1.09), 1.03 (0.93-1.16), 1.07 (0.96-1.20), and 1.48 (1.20-1.83), respectively. These associations did not differ by clinically relevant subgroups. Risk of endoscopic atrophic gastritis also increased as the baseline BMI category increased. CONCLUSIONS: In a large cohort of Korean men and women, obesity was independently associated with increased incidence of endoscopic atrophic gastritis and intestinal metaplasia. IMPACT: Excessive adiposity appears to play a role in development of stomach precursor lesions of stomach cancer, requiring further studies to determine whether strategies to reduce obesity will also help reduce precancerous lesions and, in turn, gastric cancer.
Assuntos
Índice de Massa Corporal , Intestinos/anormalidades , Metaplasia/etiologia , Adulto , Estudos de Coortes , Feminino , Humanos , Masculino , Estudos RetrospectivosRESUMO
BACKGROUND: An incidental finding of intestinal nonrotation at the time of bariatric surgery poses the following 2 dilemmas: (1) which operation to perform, and (2) whether an appendectomy should be performed concurrently. OBJECTIVES: To review the experience of 2 Bariatric Centers of Excellence with laparoscopic sleeve gastrectomy (LSG) in patients with intestinal nonrotation, and to perform a systematic review of the literature on this topic. SETTING: Two Bariatric Centers of Excellence as designated by the Ontario Bariatric Network. METHODS: A chart review of all LSG cases performed in patients with intestinal nonrotation at 2 centers was performed. A systematic review on performing bariatric surgery in patients with intestinal nonrotation/malrotation was conducted using EMBASE and MEDLINE databases. RESULTS: Four patients (.4% of all cases) underwent LSG in the setting of intestinal nonrotation. Two patients underwent a concurrent appendectomy. Three patients developed postoperative gastrointestinal reflux disease and 1 patient required conversion to a laparoscopic Roux-en-Y gastric bypass. A total of 12 retrospective studies with 23 patients were included in the systematic review. Nineteen patients underwent Roux-en-Y gastric bypass, 3 patients underwent a duodenal switch, and 1 patient underwent LSG. Nine patients (41%) underwent a concurrent appendectomy. Reasons cited for not performing an appendectomy include not completely understanding the anatomic defect, being surprised by the discovery of nonrotation, no consent for the procedure, and suboptimal trocar placement for an appendectomy. CONCLUSIONS: LSG is a reasonable alternative to laparoscopic Roux-en-Y gastric bypass in patients with intestinal nonrotation. A concurrent appendectomy may not be necessary in the era of modern cross-sectional imaging for diagnosing acute appendicitis.