Gastrointestinal and Hepatic Involvement in Primary Sjögren's Syndrome: Case Report and Literature Review

Sjögren's syndrome is a systemic autoimmune disease characterized by dry eyes and mouth due to the involvement of exocrine glands. However, it can manifest with GI symptoms that cover a broad spectrum from esophageal and intestinal dysmotility, achalasia, hypochlorhydria, and chronic atrophic gastritis to pancreatic enzyme deficiency, biliary dysfunction, and liver cirrhosis, which varies in its clinical manifestations and is often associated with erroneous approaches. This article reviews the GI manifestations of Sjögren's syndrome. It presents the case of a woman in her eighth decade of life with this syndrome. She showed asymptomatic hepatobiliary disease, documented abnormalities in liver profile tests, and a subsequent diagnosis of primary sclerosing cholangitis, for which she received initial treatment with ursodeoxycholic acid. During her condition, the patient has had three episodes of cholangitis, requiring endoscopic retrograde cholangiopancreatography with no findings of stones, with scant biliary sludge and discharge of purulent bile precipitated by her underlying liver disease. The association between Sjögren's syndrome and primary sclerosing cholangitis is rare and calls for special consideration.

El síndrome de Sjögren es una enfermedad autoinmune sistémica que se caracteriza por la sequedad ocular y bucal debido a la afección de glándulas exocrinas; sin embargo, puede manifestarse con síntomas gastrointestinales que abarcan un espectro amplio desde la dismotilidad esofágica e intestinal, acalasia, hipoclorhidria y gastritis crónica atrófica hasta enzimodeficiencia pancreática, disfunción biliar y cirrosis hepática, que tiene variación en sus manifestaciones clínicas y se asocia con abordajes erróneos en muchas ocasiones. En este artículo se hace una revisión acerca de las manifestaciones gastrointestinales de síndrome de Sjögren y se presenta el caso de una mujer en la octava década de la vida con este síndrome, que cursa con enfermedad hepatobiliar asintomática, documentación de alteración en pruebas de perfil hepático y diagnóstico ulterior de colangitis esclerosante primaria, por lo que recibió un tratamiento inicial con ácido ursodesoxicólico. Durante el curso de su enfermedad ha presentado 3 episodios de colangitis, con requerimiento de colangiopancreatografía retrógrada endoscópica sin hallazgos de cálculos, con escaso barro biliar y salida de bilis purulenta, precipitada por su enfermedad hepática de base. La asociación entre el síndrome de Sjögren y la colangitis esclerosante primaria es infrecuente y justifica una consideración especial.

Histoplasmosis in the anal canal: case report and literature review

We herein present the case of a patient with anal condylomatosis concomitant with histoplasmosis, whose diagnosis was only possible through the collection of material and the subsequent evidence of a primary pulmonary focus. Histoplasmosis is a fungal disease whose contamination occurs through the respiratory route, and it can spread to the digestive tract, but the anus is rarely affected. It is important to have a high degree of suspicion to make the diagnosis, especially in immunosuppressed patients.

Malrotación intestinal en el adulto diagnosticado posterior a presentación del síndrome pospolipectomía en ciego: reporte de un caso / Intestinal malrotation in adults diagnosed after presentation of post polypectomy syndrome in the cecum: report of a case

RESUMEN La malrotación intestinal es una anomalía congénita de la rotación y fijación intestinal, diagnosticada de forma infrecuente en la edad adulta. Se presenta un caso de malrotación intestinal en un paciente adulto previamente asintomático con cambios en el hábito intestinal en los últimos 6 meses al que se le realiza una colonoscopia ambulatoria con la evidencia de un pólipo en el ciego, posterior a su resección presenta dolor abdominal agudo en fosa ilíaca izquierda que permite la realización de estudios imagenológicos que confirman el diagnóstico.

ABSTRACT Intestinal malrotation is a congenital anomaly of intestinal rotation and fixation, diagnosed infrequently in adulthood. We report the presence of intestinal malrotation in a previously asymptomatic adult patient with changes in bowel habit in the last 6 months after a colonoscopy is performed with evidence of a polyp in the cecum, after resection presenting acute abdominal pain in the left iliac fossa that allows imaging to confirm the diagnostic.

Ultrasound in Diagnosis of Congenital Hypertrophic Pyloric Stenosis / 中国医学影像学杂志

Purpose To explore the value of Doppler ultrasound in the diagnosis of congenital hypertrophic pyloric stenosis (CHPS) by observing pylorus. Materials and Methods Sixyt-five children with CHPS diagnosed by ultrasound in Neonatology Department of Guangzhou First People's Hospital from 2006 to 2015 were retrospectively analyzed. CHPS group and 50 infants in control group were examined by Doppler ultrasound to observe the features of blood flow distribution and blood flow grading, and Doppler spectrum analysis was performed to observe contrast agent passing through pyloric canal. Results The difference of the thickness of muscular layer and mucous layer, and that of the long diameter and inner diameter of pyloric canal in CHPS group and control group all suggested marked statistical significance (P<0.01); the blood flow grading in muscular layer and mucous layer between the two groups showed statistical significance (t=13.33 and 18.77, all P<0.01). The blood flow velocity in muscular layer of CHPS group was (16.96±0.91) cm/s, resistance index (RI) 0.68±0.33, inner diameter of pyloric canal (1.98±0.33) mm. Conclusion Doppler ultrasound enables us to grasp the distribution and grading of blood flow of pylorus as well as the condition of contrast agent passing through pyloric canal, in which way the degree of pyloric stenosis can be evaluated by combining indexes such as Vmax and RI, and an objective basis for clinical choice of treatment can be provided, possessing high application value.

Validation of questionnaires to assess quality of life related to fecal incontinence in children with anorectal malformations and hirschsprung's disease / Validação de questionários para avaliação da qualidade de vida relacionada à continência fecal em crianças com malformações anorretais e doença de hirschsprung

Surgical treatment of anorectal malformations (ARMs) and Hirschsprung's disease (HD) leads to alterations in bowel habits and fecal incontinence, with consequent quality of life impairment. The objectives were to create and validate a Questionnaire for the Fecal Incontinence Index (FII) based on the Holschneider score, as well as a Questionnaire for the Assessment of Quality of Life Related to Fecal Incontinence in Children and Adolescents (QQVCFCA), based on the Fecal Incontinence Quality of Life. Methods: The questionnaires were applied to 71 children submitted to surgical procedure, in two stages. Validity was tested by comparing the QQVCFCA and a generic quality of life questionnaire (SF-36), and between QQVCFCA and the FII. A group of 59 normal children was used as control. Results: At two stages, 45.0% (32/71) and 42.8% (21/49) of the patients had fecal incontinence. It was observed that the QQVCFCA showed a significant correlation with the SF-36 and FII (Pearson's correlation 0.57), showing that the quality of life is directly proportional to improvement in fecal incontinence. Quality of life in patients with fecal incontinence is still globally impaired, when compared with control subjects (p<0.05, Student's t-test). There were also significant differences between the results of children with ARMs and children with HD. Conclusions: QQVCFCA and FII are useful tools to assess the quality of life and fecal incontinence in these groups of children. Children with ARMs submitted to surgical procedure and HD have similar quality of life impairment.

O tratamento cirúrgico das malformações anorretais (MAR) e da doença de Hirschsprung (DH) leva a alterações do hábito intestinal e incontinência fecal com prejuízo da qualidade de vida. Os objetivos foram criar e validar o Questionário para o Índice de Continência Fecal (ICF), baseado no Holschneider Criteria, bem como o Questionário para Avaliar a Qualidade de Vida Relativa à Continência Fecal em Crianças e Adolescentes (QQVCFCA), baseado no Fecal Incontinence Quality of Life. Métodos: Os questionários foram aplicados em 71 crianças operadas, em duas etapas. A validade foi testada por meio da comparação do QQVCFCA e um questionário genérico de qualidade de vida (SF-36) e entre o QQVCFCA e o ICF. Um grupo de 59 crianças normais foi usado como controle. Resultados: Nas duas etapas, 45,0% (32/71) e 42,8% (21/49) dos pacientes apresentaram incontinência fecal. Verificou-se que o QQVCFCA apresentou correlação significativa com o SF-36 e o ICF (correlação de Pearson 0,57) e mostrou que a qualidade de vida é diretamente proporcional à melhoria da continência fecal. A qualidade de vida no paciente com incontinência fecal está ainda comprometida globalmente, em comparação com os indivíduos controles (p<0,05; teste t de Student). Não houve ainda diferença significativa entre os resultados de crianças com MAR e crianças com DH. Conclusões: O QQVCFCA e o ICF são instrumentos úteis para a avaliação da qualidade de vida e da incontinência fecal nesses grupos de crianças. Crianças operadas de MAR e DH apresentam comprometimentos semelhantes da qualidade de vida.

Analysis of influence factors on congenital digestive tract malformation / 重庆医学

Objective To investigate the prevalence of congenital digestive tract malformation in Foshan city ,and to investi‐gate the influential factors on congenital digestive tract malformation .Methods A total of 17 hospitals in Foshan city were moni‐tored from 2011 to 2014 ,the data of digestive tract malformation defects of neonates were analyzed .Results A total of 308 530 newborn infants were monitored ,among them ,189 cases were diagnosed with digestive tract malformation ,the incidence rate was 0 .61‰ .The high risk factors for digestive tract malformation were as follows :infant‐mother aged or more than 30 years old ,smok‐ing or passive smoking ,adverse pregnancy history of infant‐mother ,male gender of neonate and infant born at full term . Conclusion The age of infant‐mother ,smoking or passive smoking ,adverse pregnancy history of infant‐mother ,gender of neonate and gestational age could influence the incidence of gastrointestinal malformations .

Imaging manifestations of 92 cases of congenital intestinal malrotation / 中华围产医学杂志

Objective To investigate the imaging manifestations of congenital intestinal malrotation. Methods The clinical data of 92 infants with congenital intestinal malrotation admitted from January 1993 to December 2012 were studied retrospectively. All the 92 cases enrolled in this study were diagnosed based on imaging examinations and confirmed surgically. The imaging features of four examinations including plain abdominal radiography, upper gastrointestinal radiography, lower gastrointestinal angiography and ultrasound examination were analyzed. Results The percentage of the infants who presented with the initial clinical symptoms of vomit, blood stool and abdominal dilatation was 87.0%(80/92), 3.3%(3/92) and 1.1%(1/92), respectively, and eight cases [8.7%(8/92)] received prenatal ultrasound, which showed intestinal malrotation. Clinical examination showed no abdominal abnormalities in 68 (73.9%) cases. Seventy-seven cases underwent plain abdominal radiography, revealing intestinal obstruction in 31 cases. Thirty-six cases underwent upper gastrointestinal radiography, which revealed malrotation in 14 cases, and duodenal complete/incomplete obstruction or stenosis in 19 cases. Twenty-two cases underwent lower gastrointestinal angiography, and all of them were diagnosed as malrotation. Seventy-nine cases were examined with abdominal ultrasonography, and 58 cases were diagnosed as malrotation. Conclusions Rational selection of imaging examinations can improve the preoperative diagnosis. When congenital intestinal malrotation is suspected, abdominal ultrasound should be done as the first optional examination. At the same time, plain abdominal radiography should be selected accordingly. When intestinal malrotation with or without midgut volvulus is suggested, surgery should be performed as early as possible. When the diagnosis is not clear, upper or lower gastrointestinal radiography should be done.

Idiopathic meconium peritonitis as a surgical emergency: A case report in a newborn / Peritonitis meconial idiopática como urgencia quirúrgica. Reporte de caso en una recién nacida

Meconium peritonitis is a rarely described condition that is typically found in fetal and perinatal patients. It manifests in different ways and requires an early diagnosis. In this case report, a clinical suspicion of the disease was made at the antenatal period through an obstetric ultrasound scan showing an abdominal mass in both the bottom and top right quadrants. The patient initial symptoms were those of acute intestinal obstruction. Nevertheless, these symptoms improved after surgical approach was timely performed. Based on this outcome, it can be concluded that a favorable outcome to this pathology depends on its early diagnosis, since an early diagnosis decreases morbidity and mortality chances.

La peritonitis meconial es una entidad poco descrita, propia de la edad fetal y perinatal, que se presenta de diversas formas y requiere un diagnóstico temprano. En el presente caso se realiza sospecha clínica desde el periodo antenatal, por medio de una ecografía obstétrica que muestra una masa abdominal ocupando el cuadrante derecho inferior y superior. La paciente inicia con síntomas agudos de obstrucción intestinal que mejoran luego de un abordaje quirúrgico oportuno. De acuerdo a lo anterior, se concluye que un desenlace favorable en esta patología depende de un diagnóstico temprano, pues a través del mismo se disminuyen las probabilidades de morbilidad y mortalidad

Clinical analysis of 29 cases of fetal digestive tract malformation diagnosed prenatally by ultrasound / 天津医药

Objective To investigate the clinical significance of prenatal ultrasound examination in the diagnosis of fe?tal digestive tract development. Methods Twenty-nine cases of congenital digestive tract malformation were examined in according to the different characteristics of their different fetal ultrasound images. Results There were 11 cases with non-magenblase or less magenblase (37.93%), 4 cases with combination of multiple malformations, and 9 cases with combination of amniotic fluid in the 29 cases. There were 7 cases (24.14%) with dilatation of intestine and intestinal vesicles, in which 3 with multiple malformations and 3 with polyhydramnios. There were 8 cases (27.58%) with double bubbles, in which 1 case with multiple malformations and 7 cases with amniotic fluid. Conclusion The prenatal ultrasound examination in 30 to 32 weeks of pregnancy is very valuable in diagnosis of fetal digestive tract development, which is worthy of clinical application.

Subacute combined spinal cord degeneration and pancytopenia secondary to severe vitamin B12 deficiency / Degenarión combinada subaguda medular y pancitopenia secundaria a déficit severo de vitamina B12

CONTEXT: Decreased vitamin B12 concentration does not usually result in clinical or hematological abnormalities. Subacute combined spinal cord degeneration and pancytopenia are two serious and rarely displayed consequences that appear in severe deficits. CASE REPORT: We present the case of a patient with subacute combined spinal cord degeneration and pancytopenia secondary to severe and sustained vitamin B12 deficiency. Such cases are rare nowadays and have potentially fatal consequences. CONCLUSIONS: Vitamin B12 deficiency should be taken into consideration in the differential diagnosis in cases of blood disorders or severe neurological symptoms. Early diagnosis and treatment can avoid irreversible consequences.

CONTEXTO: El descenso de la concentración de vitamina B12 no suele derivar en alteraciones clínicas o hematológicas. La degeneración combinada subaguda medular y la pancitopenia son dos consecuencias graves y poco frecuentes que aparecen en los déficit severos. CASO CLÍNICO: Presentamos el caso de un paciente con una degeneración combinada subaguda medular y pancitopenia secundarios a un déficit severo y mantenido de vitamina B12. Un caso poco común en nuestros días y con consecuencias potencialmente fatales. CONCLUSIONES: Debemos considerar el déficit de vitamina B12 dentro del diagnóstico diferencial en caso de alteraciones hematológicas o síntomas neurológicos graves. Su diagnóstico y tratamento precoz pueden evitar secuelas irreversibles.

A Case Report of Bile Duct Duplication Arising from the Hepatic Hilum / 대한내과학회지

Anatomic variation in the hepatobiliary tract is relatively common, although bile duct duplication is rare. Detection of biliary tract variation is important because it is closely related to complications such as cholangitis, cholecystitis, pancreatitis, stone formation, and gastrointestinal tract malignancy. Therefore, preoperative imaging and a high index of suspicion are required to make an accurate diagnosis of a biliary tract anomaly. A 44-year-old woman presented with a 2-year history of epigastric pain after eating a meal and gallbladder sludge with chronic cholecystitis. The presumed diagnosis was gallbladder duplication. However, during surgery it was observed that the tubular structure was parallel with the fissure for the ligamentum venosum and ended at the lesser curvature of the stomach. The pathologic diagnosis was bile duct duplication. We report a case of a unique variant of bile duct duplication arising from the hepatic hilum.

Gastric duplication cyst associated with dorsal pancreatic agenesis and a cystic pulmonary lesion / 대한내과학회지

Gastric duplication cysts and dorsal pancreatic agenesis are rare congenital anomalies, and little is known of the association between these two anomalies. A 17-year-old woman was admitted with a cystic lung mass detected as part of a health screening program. Chest computed tomography (CT) showed a cystic lung lesion in the right lower lobe and an incidental cystic abdominal mass. She had no specific symptoms or signs. Abdominal CT and endoscopic ultrasonography (EUS) revealed a 9x7x5.5-cm cystic mass and agenesis of the body and tail of the pancreas. The cyst was removed successfully using laparoscopic gastric wedge resection. Microscopically, the muscle coat of the cyst wall was fused with the muscle layer of the stomach. Therefore, the cystic mass was diagnosed as a gastric duplication cyst. We present a patient with a pulmonary cystic lesion associated with two congenital anomalies: a gastric duplication cyst and dorsal pancreatic agenesis.

A Case of Colonic Duplication Cyst Mimicking Gastrointestinal Stromal Tumor

Duplications of the gastrointestinal tract are rare congenital malformations that are usually present during the first decade of life. However, a smaller number of cases may remain occult until adulthood. Overall, the colon is the least common site of congenital gastrointestinal duplications. Colonic duplications can present with symptoms of diverticulitis and can be confused with acquired giant cysts or masses. We present a rare case of a duplication cyst of the colon in a female adult. Although the preoperative evaluations, including an abdominal CT scan and colonoscopy, were suggestive of a gastrointestinal tumor of the colon, the final diagnosis was a colonic duplication cyst based on the histopathologic examination of the resected specimen. Even if intestinal duplication cysts are uncommon, they should be considered in the differential diagnosis of intestinal masses.

Achado incidental de agenesia do lobo hepático esquerdo em paciente com colecistite aguda / Incidental finding of agenesis of the left hepatic lobe in a patient with acute cholecystitis

INTRODUÇÃO: Anomalias da morfologia hepática são entidades raras causadas por desenvolvimento embriológico anormal do fígado. Podem ser classificadas como anomalias devido a desenvolvimento defeituoso do fígado (agenesia, aplasia e hipoplasia) ou devido ao desenvolvimento excessivo (lobos acessórios). RELATO DO CASO: Paciente de 48 anos, do sexo feminino, obesa (IMC=40), que procurou o serviço de emergência com quadro de colecistite aguda. A paciente foi submetida a colecistectomia videolaparoscópica e durante o procedimento cirúrgico foi verificada a agenesia do lobo hepático esquerdo. A vesícula biliar estava em sua topografia habitual e não havia parênquima hepático à esquerda do ligamento falciforme. A colecistectomia foi realizada sem intercorrências e uma banda gástrica foi colocada para o tratamento da obesidade mórbida. Ela recebeu alta após 60 horas.

INTRODUCTION: Anomalies of hepatic morphology are rare entities caused by abnormal embryological development of the liver. They can be classified as anomalies due to defective development (agenesis, aplasia or hypoplasia) or anomalies due to excessive development of the liver (accessory lobes). CASE REPORT: A 48 years old obese female looked for emergency assistance with acute cholecystitis. An incidental finding of left hepatic lobe agenesis was observed when the patient was submitted to laparoscopic cholecystectomy. There was no hepatic parenchyma to the left of falciform ligament and gallbladder was settled on its normal position. Cholecystectomy was performed uneventfully and an adjustable gastric band was placed for the treatment of the morbid obesity. The patient was discharged after 60 hours.

Annular pancreas in children: a report of 5 cases and literature review / 中国普通外科杂志

Objective To explore clinical characteristics of the digestive tract obstruction due to annular pancreas. Methods We reviewed retrospectively clinical features, operative findings and the autopsy of 5 children with annular pancreas. Results Neonate patients usually present complete upper gastro intestinal obstruction because most of them were complicated with duodenal atresia. Infants present chronic incomplete intestinal obstruction duo to annular pancreas. 35.8% of duodenal constriction was caused by annular pancreas. Conclusions All the symptomatic patients with annular pancreas should undergo exploration to restore the consecution of the digestive tract and to detect if there is a concurrent malformation such as intestinal atresia.

The diagnosis and treatment of Currarino syndrome: report of 7 cases / 中国普通外科杂志

Objective To study the diagnosis and treatment of Currarino triad Methods A retrospective study was made on clinical features, diagnosis; treatment and prognosis of 7 cases with Currarino triad Results In 6 patients definite diagnosis was made only intraoperatively including reoperation in 3 cases All the 7 cases were cured Postoperative urinary retention and infection developed in one each Conclusion Correct preoperative diagnosis of Currarino triad has great influence on the treatment Total excision of presacral mass should be emphasized

Evaluation of the anal sphincter function by vector manometry in congenital anorectal malformation / 中国现代普通外科进展

Objective: To evaluate the anal sphincter function of congenital anorectal malforotation retrospectively.Methods: With continuous pull through technique, the manometry of 22 congenital anorectal malforotation patients and 24 controls were studied and the results were analysed with their clinical scores. Results: Anal resting pressure in the children with neurogenic anorectum induced by myelodysplasia(21.3?3.4) mmHg was lower than that in normal children(66.7?24.0) mmHg.The maximum contractive pressure of anus in the patients(22.4?3.3) mmHg was lower than that in normal children(129.0?18.8) mmHg. The length of high pressure zone in the patients(12.3?4.6 mm) was lower than that in normal children(23.6?4.6 mm). Rectoanal inhibitory reflex was identified in both patients and normal children. Conclusion:Anorectal manometry might be an effective parameter to evaluate the anal sphincter function of congenital anorectal malformation.

Diagnosis and surgical treatment of neuronal intestinal dysplasia type B in childhood / 中国普通外科杂志

Objective To evaluate the diagnosis and treatment of intestinal neuronal dysplasia type B (IND) in childhood. MethodsForty-five patients underwent preoperative barium enema examination, 23 patients underwent electromanometry, and mucosal biopsy and immunohistochemical staining for S100 protein were performed in 17 cases. All 45 patients underwent resection of the invalid segment with coloproctostomy. Whole layer was sampled on several sites of the resected segment and examined by two independent pathologists. All patients were followed up from 3 months to 9 years (mean 4.6 years).ResultsTwenty eight patients were complicated with Hirschsprung′s disease, one patient with hypogangliosis, and isolated IND was diagnosed in the other 16 children. The narrowed distal segment with proximal dilatation was merely noted in 4 children with isolated IND. Internal sphincter relaxations were missing in 6 children with isolated IND. The indicative diagnosis might be merely gained in 7 patients by the mucosa biopsy. The correct diagnosis can be established by whole layer biopsy of the resected segment. Three children with enterocolitis after operation were cured by conservative treatment. One patient suffering from postoperative sluice syndrome underwent second resection. Postoperative continence was achived in all patients. ConclusionThe correct diagnosis of IND can be obtained by biopsy of whole layer, and resection of invalid bowel segment with coloproctostomy is the choice of therapy.

The assessment of anal sphincter function after surgical correction of anorectal malformation by using three dimensional manometric vector volume / 中国普通外科杂志

Objective This study was to assess the anal sphincter function of postoperative patients with anorectal malformation by using vector volume manometry Methods The anal manometric vector volume were assessed in 40 cases by three dimensional PC Polygram HR system Results Results were graded as good, fair and poor group according to the clinical anal function score Anal sphincter pressures were (85?44)mm?Hg, ( 71? 38)mm?Hg and (42?28)mm?Hg respectively Anal sphincter pressure in control group was (161?64)mm?Hg ( P