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BACKGROUND: In this study, two fibrin sealant products, Fibrin Sealant Grifols (FS Grifols 80 mg/mL fibrinogen; 500 IU/mL thrombin) and Evicel (fibrinogen 55-85 mg/mL; thrombin 800-1200 IU/mL) were studied for efficacy in achieving hemostasis at a targeted bleeding site (TBS) on parenchymous or soft tissue in pediatric surgeries. METHODS: This phase 3, single-blind, active comparator, non-inferiority trial compared the number of patients achieving hemostasis at a TBS at four (T4 - primary endpoint), seven (T7) and 10 (T10) minutes after application, Safety and tolerability were assessed by recording adverse events during and after procedures. Eligible patients were <18 years old undergoing elective, open, non-cardiac thoracic, abdominal or pelvic surgeries. Preterm (<37 weeks gestation) and newborn (0-27 days) infants were eligible. RESULTS: At T4, 98.7% of FS Grifols group (n = 91) and 95.4% of the Evicel group (n = 87) achieved hemostasis. All patients with residual bleeding at T4 were undergoing soft tissue surgery. All patients achieved hemostasis by T7. At T10, all patients achieved hemostasis except one (FS Grifols (no observation recorded)). There were no incidents of persistent bleeding. For FS Grifols, 26.5% of patients had treatment-emergent adverse events (TEAEs) and 18.4% for Evicel. One TEAE (moderate procedural pain - FS Grifols group) was considered possibly related to study treatment. Three patients died for reasons unrelated to the study medications. CONCLUSIONS: FS Grifols was safe and effective at achieving hemostasis in pediatric patients having parenchymous or soft tissue surgeries. The efficacy of FS Grifols was non-inferior to Evicel. LEVEL OF EVIDENCE: I.
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Immature sacrococcygeal teratoma represents a histological form with rapid tumor growth, a risk of premature birth, an enhanced rate of complications, an increased risk of recurrence, and a higher mortality rate than the mature type. Thus, prenatal diagnosis of immature forms would significantly improve the prognosis of these cases. To this end, we performed an extensive literature review on the diagnosis, therapeutic management, and follow-up of immature teratomas. Regarding this medical conduct, we also presented our case. In conclusion, the early identification of immature sacrococcygeal teratomas with or without other associated structural abnormalities and their correct therapeutic approach are basic principles for a favorable evolution of these cases.
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Gastrointestinal hemangiomas (GIH) are unusual vascular tumors found anywhere alongside the GI tract, the small bowel being the most common site. Diagnosis requires good clinical insight and modern imaging. This is a comprehensive review of the literature, starting from a new pediatric case diagnosed through exploratory laparotomy after complex imaging techniques failed. This research was conducted on published articles from the past 25 years. We identified seventeen original papers (two series of cases with three and two patients, respectively, and fifteen case reports). The female/male ratio was 1.5. The youngest patient was a 3-week-old boy, and the was oldest a 17-year-old girl. The most common localization was the jejunum (eight cases), followed by the ileum (four), colon (three), stomach (two), and rectum (one). Seven children had cavernous and four had capillary hemangiomas. Eight patients presented gastrointestinal bleeding, seven had refractory anemia, such as our index patient, three had recurrent abdominal pain, and two had bowel obstruction. Surgical assessment was successful in fifteen cases; three cases experienced great outcomes with oral propranolol, one child was treated successfully with sirolimus, and for one patient, endoscopic treatment was the best choice. The authors present the case of a female patient admitted to the Pediatrics Department of "Grigore Alexandrescu" Emergency Children's Hospital from 25 February to 28 March 2019 for severe anemia, refractory to oral iron treatment, and recurrent blood infusions. No clear bleeding cause had been found. Although very uncommon, intestinal hemangiomas can express puzzling, life-threatening symptoms. We should keep in mind this disorder in cases of unresponsive chronic anemia.
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Fetal lingual tumors are very rare, and their early prenatal diagnosis is important for defining the subsequent therapeutic strategy. In this study, we aimed to describe a case of a congenital septate lingual cyst and perform an extensive literature review on two main databases (PubMed, Web of Science), analyzing the clinical manifestations, the imaging appearance, the differential diagnosis, and particularities regarding the treatment of these tumors. The electronic search revealed 17 articles with 18 cases of mixed heterotopic gastrointestinal/respiratory oral epithelial cysts that met the eligibility criteria and were included in this review. The clinical case was diagnosed prenatally during second-trimester screening. On the eighth day of life, the fetus underwent an MRI of the head, which revealed an expansive cystic process on the ventral side of the tongue with the greatest diameter of 21.7 mm, containing a septum of 1 mm inside. On the 13th day of life, surgery was performed under general anesthesia, and the lingual cystic formation was completely excised. The postoperative evolution was favorable. The histopathological examination revealed a heterotopic gastric/respiratory-mixed epithelial cyst with non-keratinized respiratory, gastric squamous, and foveolar epithelium. The lingual cyst diagnosed prenatally is an accidental discovery, the differential diagnosis of which can include several pathologies with different degrees of severity but with a generally good prognosis.
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Klippel-Trenaunay syndrome (KTS) is a very rare vascular malformation syndrome also referred to as a capillary-lymphatic-venous malformation with unknown aetiology. The aim of our paper is to highlight interesting images, regarding a rare case of foetal Klippel-Trenaunay syndrome diagnosed prenatally in our department and confirmed postnatally with a favourable evolution during the gestation and neonatal periods. This case was diagnosed at 26 weeks gestation and characterised through ultrasound by the presence of superficial multiple cystic structures of different sizes spreading over the left leg with hemihypertrophy and reduced mobility. The cystic lesions were spreading to the left buttock and the pelvic area. The right leg and upper limbs had normal appearance with good mobility. There were no signs of hyperdynamic circulation or foetal anaemia, but mild polyhydramnios was associated. The ultrasound findings were confirmed postnatally, the left leg presented multiple cystic lesions and port wine stains, and there was hypertrophy and fixed position, with favourable evolution at 6 months of life, when the size of the lesions began to decrease and the mobility of the leg improved.
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Omphalocele is the most common ventral abdominal wall defect. Omphalocele is associated with other significant anomalies in up to 80% of cases, among which the cardiac ones are the most frequent. The aim of our paper is to highlight, through a review of the literature, the importance and frequency of association between the two malformations and what impact this association has on the management and evolution of patients with these pathologies. We reviewed the titles, the available abstracts, and the full texts of 244 papers from the last 23 years, from three medical databases, to extract data for our review. Due to the frequent association of the two malformations and the unfavorable effect of the major cardiac anomaly on the prognosis of the newborn, the electrocardiogram and echocardiography must be included in the first postnatal investigations. The timing of surgery for abdominal wall defect closure is mostly dictated by the cardiac defect severity, and usually the cardiac defect takes priority. After the cardiac defect is medically stabilized or surgically repaired, the omphalocele reduction and closure of the abdominal defect are performed in a more controlled setting, with improved outcomes. Compared to omphalocele patients without cardiac defects, children with this association are more likely to experience prolonged hospitalizations, neurologic, and cognitive impairments. Major cardiac abnormalities such as structural defects that require surgical treatment or result in developmental delay will significantly increase the death rate of patients with omphalocele. In conclusion, the prenatal diagnosis of omphalocele and early detection of other associated structural or chromosomal anomalies are of overwhelming importance, contributing to the establishment of antenatal and postnatal prognosis.
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Background and Objectives: To assess the current practice pattern in the management of pediatric acute appendicitis in Romania. Materials and Methods: A questionnaire was emailed to all the members of the Romanian Society of Pediatric Surgery between June-July 2022. Results: 118 answers were received, 79.7% responses being from permanent staff members. In the diagnosis of appendicitis, complete blood count, C-reactive protein and abdominal ultrasound are the most commonly used diagnostic tools, while appendicitis scores are not widely used (25% of surgeons). In the case of simple appendicitis, 49.2% of surgeons prefer the conservative approach-oral/intravenous antibiotics. Those who choose the operative approach begin preoperative antibiotics in 56.7% of patients. In case of a stable patient, only 16.7% of surgeons will operate during the night. Laparoscopic approach is chosen by 51.7% of surgeons. In the case of a complicated appendicitis, 92.4% of surgeons will perform the appendectomy, prescribing preoperative antibiotics in 94% of the cases and continuing the therapy postoperatively in 98.2%. Laparoscopic approach is used by 28.8% of surgeons in case of complicated appendicitis. In presence of appendicular mass, 80% prefer a conservative approach with a delayed appendectomy within 6 months. Appendicular abscesses are managed operatively in 82.2% of the cases. The appendix is sent for histological analysis by 95.8% of surgeons. If the peritoneal cavity is contaminated, 95% of the respondents will take a sample for microbiological analysis, 71% will always place a drainage and 44% will always irrigate (71.9%-saline). Conclusions: Clearly, there seems to be a lack of consensus regarding several aspects of the management of acute appendicitis in children. In addition, minimally invasive surgery is not as widely used as reported, despite literature support.
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Apendicitis , Laparoscopía , Cirujanos , Humanos , Niño , Apendicitis/cirugía , Apendicitis/complicaciones , Rumanía , Apendicectomía , Encuestas y Cuestionarios , Enfermedad Aguda , Antibacterianos/uso terapéuticoRESUMEN
BACKGROUND: Wilms' tumour or nephroblastoma is the most common renal malignancy encountered in the paediatric population. Imaging findings are of great importance to the surgeon, the oncologist and the radiologist in the diagnosis and the staging and surveillance of this tumour. MATERIAL AND METHODS: This study was carried out as a 10-year retrospective study of patients who were diagnosed with Wilms' tumour. RESULTS: The study included 12 boys and 11 girls. Ultrasound and computed tomography were performed in all cases. Ultrasonography was found to be superior to the CT examination when approximating the antero-posterior and transverse diameters; the computer-tomographic examination is cited as superior for estimating the invasion of nephroblastomas. CONCLUSIONS: Ultrasound has been shown to be effective in detecting the rupture of the renal capsule, tumour calcifications and invasion of the renal vein, pelvis and ureter. However, ultrasound cannot replace CT in the detection of lymphadenopathy and the invasion of adjacent organs.
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Background and Objectives: Choledocal cyst is a rare congenital disease of the biliary tree defined by dilatation of the extrahepatic and/or intrahepatic biliary ducts. Untreated, it leads to complications such as cholangitis, stone formation and malignant degeneration. The standard treatment for choledocal cyst is complete excision and subsequent biliary reconstruction via hepaticojejunostomy or hepatiocoduodenostomy. Materials and Methods: We report our experience with 16 pediatric cases of choledocal cyst over a 10-year period. Results: The predominant symptoms were nausea and jaundice, both at 62.5% (n = 10), followed by abdominal pain at 56.3% (n = 9). Ultrasonography was the diagnostic method used in all patients. Computed tomography was used in 75% (n = 12) and magnetic resonance imaging in 25% (n = 4) of cases. Age at the time of intervention ranged from 2 months to 17 years with a mean of 4 years and 5 months. The open approach was used in nine patients and the laparoscopic approach was used in seven patients, with one conversion to open surgery. Complete excision of the choledocal cyst was performed in 15 cases (93.7%), and partial excision with mucosectomy was performed in one case (6.2%). Eight patients (50%) underwent hepaticoduodenostomy and eight (50%) underwent hepaticojejunostomy, out of which one was attempted laparoscopically but was converted. We had a postoperative complication rate of 12.5% (n = 2) represented by anastomotic leak and pancreatitis. Conclusions: From our experience with these cases, we concluded that a wide hepaticoduodenostomy constitutes a favorable choice over the traditional hepaticojejunostomy, being more physiological and less time consuming.
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Quiste del Colédoco , Anastomosis Quirúrgica , Conductos Biliares Intrahepáticos/cirugía , Niño , Quiste del Colédoco/cirugía , Humanos , Lactante , Hígado , Complicaciones PosoperatoriasRESUMEN
Abdominal tumor masses are a very rare disease in the fetus. The authors present the first reported case of neonatal multicystic adenomatoid hamartoma of the pancreas associated with well-differentiated fetal epithelial subtype hepatoblastoma and reveal clinical, histologic, and imagistic aspects. Case presentation: A 36-week-old female newborn in whom a 25-week ultrasound showed a relatively homogeneous pancreatic echogenic mass (34 × 30 × 55 mm) with compression of the inferior vena cava and retrograde dilation. Postnatal CT showed a giant pancreatic tumor mass (113 × 70 × 60 mm), with areas enhancing contrast and cystic/necrotic areas and a hypodense, hypocaptive nodule of 8 × 6 mm located at segment IV of the liver; thrombosis of the subhepatic segment of the inferior vena cava and both renal veins. Histopathological and immunohistochemical studies confirmed the diagnosis of multicystic pancreatic adenomatoid hamartoma and well-differentiated fetal epithelial subtype hepatoblastoma. Conclusions: Pancreatic hamartoma can be difficult to diagnose (especially prenatal), with or without nonspecific symptoms. The synchronous presence of hepatoblastoma complicated the therapeutic conduct and prognosis of this case, with the diagnosis being confirmed histopathologically and immunohistochemically after liver biopsy.
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Hypertriglyceridemia induced acute pancreatitis is a rare cause of pancreatitis in children. Hepatic lipase deficiency is an extremely rare cause of hypertriglyceridemia, reported in only a few families to date. Hepatic lipase is the enzyme involved in the hydrolysis of triglycerides and phospholipids in remnants of triglyceride-rich lipoproteins that have a role in the conversion of very low density lipoprotein remnants to low density lipoproteins. Hepatic lipase deficiency is inherited in an autosomal recessive pattern. Detection of heterozygous carriers of hepatic lipase mutations remains accidental at the population level, as affected persons with a heterozygous state of hepatic lipase mutation do not display specific lipoprotein abnormalities and also patients with complete hepatic lipase deficiency have inconstant phenotype. The proximal promoter of the LIPC gene consists of four polymorphic sites in complete linkage disequilibrium. Five missense mutations in encoding exons have been described and proved to be responsible for hepatic lipase deficiency to date: S267F, T383M, L334F, A174T, and R186H, affecting the activity and secretion of hepatic lipase. We identified a primary disorder of the lipid metabolism as the cause of the acute episode of pancreatitis in a four years old patient, consisting of hepatic lipase deficiency caused by a novel genetic variant of the LIPC gene, a gross deletion of the genomic region encompassing exon 1. This variant was not previously described in the literature in persons with LIPC-related disorders and its significance is currently uncertain, but in the presented clinical and paraclinical context, it has the characteristics of a pathological variant inducing a hepatic lipase deficiency phenotype.
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Background and Objecitves: Appendicitis is one of the most frequent surgical emergencies in pediatric surgery. Complicated appendicitis can evolve with appendicular peritonitis characterized by the diffusion of the pathological process to the peritoneal cavity, thus producing generalized or localized inflammation of the peritoneum. The capacity to anticipate the possibility of perforation in acute appendicitis can direct prompt management and lower morbidity. There is no specific symptom that could be used to anticipate complicated appendicitis, and diagnostic clues include a longer period of symptoms, diffuse peritoneal signs, high fever, elevated leukocytosis and CRP, hyponatremia, and high ESR. Imagistic methods, particularly US and CT, are useful but not sufficient. There are no traditional inflammation biomarkers able to predict the evolution of uncomplicated to complicated appendicitis alone, but the predictive capacity of novel biomarkers is being investigated. Materials and Methods: The present study represents a retrospective evaluation of children hospitalized between January 2021 and July 2022 in the Grigore Alexandrescu Clinical Emergency Hospital for Children with a diagnosis of acute appendicitis settled based on clinical characteristics, traditional and novel biomarkers, and ultrasonographic features. The children were subsequently grouped into two groups based on the existence of appendicular peritonitis on intraoperative inspection of the abdominal cavity. The aim of this report is to establish the predictors that may aid physicians in timely identifying pediatric patients diagnosed with acute appendicitis at risk for developing complicated appendicitis with evolution to appendicular peritonitis. Results: The neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte radio (PLR) are representative severity markers in infections. This report analyzes the benefit of these markers for distinguishing uncomplicated appendicitis from complicated appendicitis in pediatric patients. Conclusions: Our study suggests that a value of neutrophil-to-lymphocyte ratio greater than 8.39 is a reliable parameter to predict the evolution to appendicular peritonitis.
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Apendicitis , Peritonitis , Humanos , Niño , Apendicitis/complicaciones , Apendicitis/diagnóstico , Apendicitis/cirugía , Estudios Retrospectivos , Peritonitis/diagnóstico , Peritonitis/etiología , Peritonitis/cirugía , Biomarcadores , Inflamación/complicacionesRESUMEN
BACKGROUND: N-Pep-12 is a dietary supplement with neuroprotective and pro-cognitive effects, as shown in experimental models and clinical studies on patients after ischemic stroke. We tested the hypothesis that N-Pep-12 influences quantitative electroencephalography (QEEG) parameters in patients with subacute to chronic supratentorial ischemic lesions. METHODS: We performed secondary data analysis on an exploratory clinical trial (ISRCTN10702895), assessing the efficacy and safety of 90 days of once-daily treatment with 90 mg N-Pep-12 on neurocognitive function and neurorecovery outcome in patients with post-stroke cognitive impairment against a control group. All participants performed two 32-channel QEEG in resting and active states at baseline (30-120 days after stroke) and 90 days later. Power spectral density on the alpha, beta, theta, delta frequency bands, delta/alpha power ratio (DAR), and (delta+theta)/(alpha+beta) ratio (DTABR) were computed and compared across study groups using means comparison and descriptive methods. Secondarily, associations between QEEG parameters and available neuropsychological tests were explored. RESULTS: Our analysis showed a statistically significant main effect of EEG segments (p<0.001) in alpha, beta, delta, theta, DA, and DTAB power spectral density. An interaction effect between EEG segments and time was noticed in the alpha power. There was a significant difference in theta spectral power between patients with N-Pep-12 supplementation versus placebo at 0.05 alpha level (p=0.023), independent of time points. CONCLUSION: A 90-day, 90 mg daily administration of N-Pep-12 had significant impact on some QEEG indicators in patients after supratentorial ischemic stroke, confirming possible enhancement of post-stroke neurorecovery. Further research is needed to consolidate our findings.
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Isquemia Encefálica , Accidente Cerebrovascular Isquémico , Accidente Cerebrovascular , Isquemia Encefálica/complicaciones , Isquemia Encefálica/tratamiento farmacológico , Suplementos Dietéticos , Electroencefalografía , Humanos , Accidente Cerebrovascular/tratamiento farmacológicoRESUMEN
Background: Surgery for severe liver trauma remains challenging even for HPB surgeons, mainly due the hemodynamical instability, involvement of major vascular and biliary elements, impaired background liver and frequent anatomical variants. In this setting, despite conservative policy, major liver resection is still required in selected cases. Also salvage liver transplantation may be needed. Our study aims to analyze the results after definitive surgery for hepatic injury (HI) in a tertiary HPB center. Methods: Sixty-six patients with HI were admitted and treated in our center between June 2000 and June 2021. The median age was 29 years (mean 35, range 10-76). The male/female ratio was 50/16. According to the American Association for the Surgery of Trauma (AAST) system, HIs were grade II in one patient (1.5%), grade III in 11 pts (16.7%), grade IV in 25 pts (37.9%), and grade V in 29 pts (43.9%); no patient had grade I or VI HI. Results: Fifty-two pts (78.8%) benefitted from surgery and 14 pts (21.2%) from non-operative treatment (NOT). Perihepatic packing was previously performed in 38 pts (73.1%). Surgery consisted in hepatic resections (HR) in 51 pts (77.3%) and liver transplantation in one patient (1.5%). The rate of major HR was 51.9% (27 HRs). The overall major morbidity and mortality rates were 33.3% (20 pts) and 13.6% (9 pts), respectively. For surgery, the major complication rate was 35.3% (18 pts), while for major and minor HR were 40.7% (11 pts) and 29.2% (7 pts), respectively; the mortality rate was 15.7% (8 pts). After NOT, the major morbidity and mortality rates were 14.3% (2 pts) and 7.1% (1 pt), respectively. Conclusions: Hepatic resections, especially major ones and/or involving vascular and biliary reconstructions, as well as non-operative treatment for severe hepatic injuries, are to be carried out in tertiary HPB centers, thus minimizing the morbidity and mortality rates, while having the liver transplantation as salvage option.
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Traumatismos Abdominales , Traumatismos Abdominales/cirugía , Adulto , Femenino , Hepatectomía , Humanos , Hígado/cirugía , Masculino , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Dysplasia epiphysealis hemimelica (DEH), also known as Trevor's disease, is a rare nonhereditary skeletal disorder affecting one side of the epiphyses or the epiphyses-equivalents. It is often misdiagnosed for traumatic injuries, infections, or other tumors because of the nonspecific clinical features. The diagnosis is mostly based on radiographic involvement of one half of the epiphysis displaying an overgrowth; it is hard to distinguish between DEH and osteochondroma on the gross hystopathological exam. There are few immunohistochemical markers, as well as genetic tests, for EXT1 and EXT2 gene expression that can reveal a more accurate diagnosis. No evidence of malignant changes has been reported and no hereditary transmission or environmental factor has been incriminated as an etiological factor. The natural history of the disease is continuous growth of the lesions until skeletal maturity. Without treatment, the joint might suffer degenerative modification, and the patient can develop early onset osteoarthritis. In the present paper, we report two new cases of DEH of the ankle. The aim of this paper is to consider Trevor's disease when encountering tumoral masses in the epiphyses of pediatric patients and to present our treatment approach and results.
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BACKGROUND: Nephroblastoma is the most common renal malignancy in children kidney. They are highly heterogeneous tumors with challenging imagistic and histopathological (HP) differential diagnosis. Imaging is critical for understanding local anatomy, staging and for planning surgical approach. PURPOSE: To determine whether HP staging can be successfully predicted by the imagistic staging using computed tomography. Also, we find it important to make a brief review of the imagistic, HP and immunohistochemical differential diagnosis of nephroblastoma, considering that a correct diagnosis is essential for an appropriate therapeutic strategy in all stages. PATIENTS, MATERIALS AND METHODS: We present a retrospective study of the medical dossiers of 22 patients that underwent surgery at our Center between 2014 and 2020. We provided descriptive data and compared imagistic and HP staging using a Mann-Whitney U-test. An up-to-date literature review was also done. RESULTS: We found that imagistic staging tends to under- or over-stage at similar rates and that the difference between the two staging systems is statistically significant. Immunohistochemistry is necessary for establishing the correct diagnosis, especially in cases with one predominant HP pattern. CONCLUSIONS: HP and imagistic staging are not yet sufficiently similar for successfully predict the former via imagistic means.
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Neoplasias Renales , Tumor de Wilms , Niño , Diagnóstico Diferencial , Humanos , Neoplasias Renales/diagnóstico por imagen , Neoplasias Renales/patología , Estadificación de Neoplasias , Estudios Retrospectivos , Tumor de Wilms/diagnóstico por imagen , Tumor de Wilms/patologíaRESUMEN
BACKGROUND: Esophageal atresia (EA) is the most frequent and severe congenital anomaly of the esophagus, occurring in 1:2500-1:4500 live births. Five types of EA have been described, EA with tracheoesophageal fistula (TEF) being the most frequent. AIM: The aim of this paper is to evaluate epidemiological aspects, malformative associations, and prenatal diagnosis in an unusual case series of EA with distal TEF. CASE PRESENTATIONS: The authors are analyzing a series of seven cases of EA with distal TEF. The seven cases of EA with TEF presented occurred during a period of two years, resulting in an unusually increased rate - 1.1:1000 live births. Except a late suspicion of EA (one day before delivery), EA was not diagnosed during prenatal scans despite association with polyhydramnios in two cases and single umbilical artery in four cases. None of the two cases of unilateral renal agenesis or anorectal malformations were diagnosed on prenatal ultrasound scans. In two of the cases, EA was part of VACTERL (vertebral defects, anorectal malformations, heart defects, EA with or without TEF, renal anomalies/dysplasia, and limb defects) association. Despite lack of prenatal diagnosis, postnatal diagnosis of EA was suspected at birth in four cases, at two hours in one case. CONCLUSIONS: An increased index of suspicion for congenital structural defects, particularly for EA, should be maintained in the presence of a single umbilical artery and÷or polyhydramnios on prenatal ultrasound scan. Prenatal diagnosis of EA offers the chance for parental counseling, planned birth and transfer for corrective surgery and decreases the risk for postnatal aspiration pneumonia associated with early feedings.
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Atresia Esofágica/complicaciones , Fístula Traqueoesofágica/etiología , Atresia Esofágica/patología , Femenino , Humanos , Recién Nacido , Masculino , Fístula Traqueoesofágica/patologíaRESUMEN
Segmental aganglionosis Hirschsprung's is an extremely rare condition. Described as a segment of normally ganglionated bowel surrounded proximally and distally by aganglionosis, zonal aganglionosis is of interest because it may cause confusion in interpreting surgical margins. Diagnosis of segmental lesions in Hirschsprung's disease may be missed as it is rarely suspected at initial surgery. We report the case of a 2-week-old baby girl admitted to our clinic for abdominal distension and vomiting. Considering the family history (near total colonic aganglionosis in a 2-month-old sister with unfavorable outcome), the suspicion of Hirschsprung's is raised and serial large intestine biopsies are taken. Intraoperatively, a transverse colon stenosis caused by an incomplete web is noticed and segmental colectomy with anastomosis is performed at this level. Histopathological and immunohistochemical results established the diagnosis of segmental transverse colon aganglionosis, with the presence of ganglia cells in the ascending and descending colon. Subtotal colectomy with ascending colon pull-through was performed with favorable postop evolution. In our patient, the association with a transverse colon stenosis raised suspicion concerning the diagnosis of Hirschsprung's disease, but considering the family history, extended biopsies were taken and the correct diagnosis of zonal aganglionosis was established. Although zonal aganglionosis lesions are extremely rare, this case illustrates the point that the presence of ganglia cells at the resection line is not sufficient to guarantee postoperative function. Extended intestinal biopsies should be included in the algorithm for management of long segment Hirschsprung's disease and will enable the surgeon to correctly detect zonal aganglionosis.
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Enfermedad de Hirschsprung/patología , Calbindina 2/metabolismo , Femenino , Humanos , Inmunohistoquímica , Recién Nacido , Proteínas S100/metabolismoRESUMEN
T-cell lymphoblastic lymphoma is an aggressive malignancy that represents 85% of all lymphoblastic lymphomas. It usually occurs in late childhood, adolescence and young adulthood with a 2:1 male preponderance and it presents with pleural effusion and respiratory symptoms and in rare cases vena cava syndrome can be encountered. We present the case of a 13-year-old patient who was referred to our clinic from a local hospital where he was diagnosed with a mediastinal tumor. The patient presented with thoracic pain, fever, coughing and fatigability for a month prior to admission, after having underwent surgery for abdominal pain (appendectomy). On admission to our hospital, a thoracic computed tomography (CT) scan was performed and showed the presence of an anterior mediastinal mass measuring 109/76/140 mm, well defined, which came in close contact with the superior vena cava, the ascending aorta and the pulmonary artery, right pleural effusion and a collapsed lung on the right side. The decision was taken to perform a tumor biopsy and a right pleural drain was placed. The patient's post-operative evolution was favorable with the remission of the respiratory symptoms. The histopathological result showed the presence of T-cell lymphoblastic lymphoma and the patient was then transferred to the oncology ward where he underwent chemotherapeutic treatment, with a favorable outcome. T-cell lymphoblastic lymphoma is an aggressive type of lymphoma and it is usually hard to diagnose considering the fact that the symptoms are often vague. It is essential to establish the diagnosis without delay and start appropriate chemotherapeutic treatment.