الملخص
PURPOSE: In this study, we aimed to review the clinical presentation of preterm infants with gastrointestinal perforations and compare the clinical features of gastric perforation with other intestinal perforations. METHODS: The medical records of preterm neonates with pneumoperitoneum, admitted to the neonatal intensive care unit (NICU) between January 1994 and December 2013, were retrospectively reviewed. RESULTS: Twenty-one preterm infants underwent exploratory laparotomy to investigate the cause of the pneumoperitoneum. The sample consisted of five patients (23.8%) with gastric perforation and 16 patients (76.2%) with intestinal perforation. No statistical differences were found in the birth history and other perinatal factors between the two groups. Underlying necrotizing enterocolitis, bilious vomiting, and paralytic ileus preceding the perforation were statistically more common in the intestinal perforation group. All preterm infants with gastric perforation survived to discharge; however, six preterm infants with intestinal perforation expired during treatment in the NICU. In the gastric perforation group, sudden pneumoperitoneum was the most common finding, and the mean age at diagnosis was 4.4+/-1.7 days of life. The location and size of the perforations varied, and simple closure or partial gastrectomy was performed. CONCLUSION: Patients with gastric perforation did not have a common clinical finding preceding the perforation diagnosis. Although mortality in previous studies was high, all patients survived to discharge in the present study. When a preterm infant aged less than one week presents with sudden abdominal distension and pneumoperitoneum, gastric perforation should first be excluded. Prompt exploratory laparotomy will increase the survival rates of these infants.
الموضوعات
Humans , Infant , Infant, Newborn , Diagnosis , Enterocolitis, Necrotizing , Gastrectomy , Infant, Premature , Intensive Care, Neonatal , Intestinal Perforation , Intestinal Pseudo-Obstruction , Laparotomy , Medical Records , Mortality , Pneumoperitoneum , Reproductive History , Retrospective Studies , Stomach Rupture , Survival Rate , Vomitingالملخص
Malignant salivary gland tumors only represent 0.08% of all childhood tumors and mucoepidermoid carcinoma (MEC) is the most common histologic type. Although there are many reports describing second malignant neoplasm (SMN) in patients treated for childhood cancer, salivary gland tumors rarely appears. In Korea, there has been no report about MEC that developed in children as a SMN. We report a MEC in a 4 years and 8 months old female child that developed after completing treatment for yolk sac tumor of lower abdomen. The primary tumor presented with metastasis at the time of diagnosis, and therefore, the child underwent high-dose chemotherapy with autologous peripheral blood stem cell transplantation along with surgery and radiotherapy. Three years and five months after completing treatment, MEC developed in her submandibular gland. She was treated with surgery and radiotherapy and is in disease free state for 5 months at the time of this writing.
الموضوعات
Child , Female , Humans , Abdomen , Carcinoma, Mucoepidermoid , Diagnosis , Drug Therapy , Endodermal Sinus Tumor , Korea , Neoplasm Metastasis , Peripheral Blood Stem Cell Transplantation , Radiotherapy , Salivary Gland Neoplasms , Salivary Glands , Submandibular Gland , Writingالملخص
A 10-year-old boy with severe aplastic anemia was admitted for allogeneic hematopoietic stem cell transplantation. After conditioning chemotherapy using cyclophosphamide, fludarabine, and antithymocyte immunoglobulin, he presented with fever and abdominal pain on day 0 of stem cell transplantation. After diagnosis of acute appendicitis with minor perforation, appendectomy was performed just after cell infusion. A week after the procedure, he showed two huge liver abscesses in S4 and S6 segments. We used broad spectrum antibiotics along with antifungal agents. Percutaneous drainage was attempted, but no fluid was removed and no microorganisms were isolated. After 7 weeks of antibiotics and antifungal therapy, liver abscesses showed improvement. We report a case of successfully treated appendicitis with liver abscesses in a severely neutropenic patient during allogeneic hematopoietic stem cell transplantation.
الموضوعات
Child , Humans , Male , Abdominal Pain , Anemia, Aplastic , Anti-Bacterial Agents , Antifungal Agents , Appendectomy , Appendicitis , Cyclophosphamide , Diagnosis , Drainage , Drug Therapy , Fever , Hematopoietic Stem Cell Transplantation , Immunoglobulins , Liver Abscess , Stem Cell Transplantation , Typhlitisالملخص
Malignant salivary gland tumors only represent 0.08% of all childhood tumors and mucoepidermoid carcinoma (MEC) is the most common histologic type. Although there are many reports describing second malignant neoplasm (SMN) in patients treated for childhood cancer, salivary gland tumors rarely appears. In Korea, there has been no report about MEC that developed in children as a SMN. We report a MEC in a 4 years and 8 months old female child that developed after completing treatment for yolk sac tumor of lower abdomen. The primary tumor presented with metastasis at the time of diagnosis, and therefore, the child underwent high-dose chemotherapy with autologous peripheral blood stem cell transplantation along with surgery and radiotherapy. Three years and five months after completing treatment, MEC developed in her submandibular gland. She was treated with surgery and radiotherapy and is in disease free state for 5 months at the time of this writing.
الموضوعات
Child , Female , Humans , Abdomen , Carcinoma, Mucoepidermoid , Diagnosis , Drug Therapy , Endodermal Sinus Tumor , Korea , Neoplasm Metastasis , Peripheral Blood Stem Cell Transplantation , Radiotherapy , Salivary Gland Neoplasms , Salivary Glands , Submandibular Gland , Writingالملخص
A 10-year-old boy with severe aplastic anemia was admitted for allogeneic hematopoietic stem cell transplantation. After conditioning chemotherapy using cyclophosphamide, fludarabine, and antithymocyte immunoglobulin, he presented with fever and abdominal pain on day 0 of stem cell transplantation. After diagnosis of acute appendicitis with minor perforation, appendectomy was performed just after cell infusion. A week after the procedure, he showed two huge liver abscesses in S4 and S6 segments. We used broad spectrum antibiotics along with antifungal agents. Percutaneous drainage was attempted, but no fluid was removed and no microorganisms were isolated. After 7 weeks of antibiotics and antifungal therapy, liver abscesses showed improvement. We report a case of successfully treated appendicitis with liver abscesses in a severely neutropenic patient during allogeneic hematopoietic stem cell transplantation.
الموضوعات
Child , Humans , Male , Abdominal Pain , Anemia, Aplastic , Anti-Bacterial Agents , Antifungal Agents , Appendectomy , Appendicitis , Cyclophosphamide , Diagnosis , Drainage , Drug Therapy , Fever , Hematopoietic Stem Cell Transplantation , Immunoglobulins , Liver Abscess , Stem Cell Transplantation , Typhlitisالملخص
OBJECTIVE: To retrospectively compare the imaging characteristics of retropharyngeal density and associated findings for Kawasaki disease with those for non-Kawasaki disease, and identify the distinguishing features which aid the CT diagnosis of Kawasaki disease with retropharyngeal low density. MATERIALS AND METHODS: Among the enhanced neck CT performed in children less than 8-years old with clinical presentation of fever and cervical lymphadenopathy over a 6-year period, only cases with retropharyngeal low density (RLD) were included in this study. The 56 cases of RLD were divided into two groups; group A included cases diagnosed as Kawasaki disease (n = 34) and group B included cases diagnosed as non-Kawasaki disease (n = 22). We evaluated the CT features including the thickness of RLD and its extent into the deep neck spaces, as well as soft tissue change in the adjacent structure. We also scored the extent of RLD into the deep neck spaces and the soft tissue changes in the adjacent structure. RESULTS: The thickness of RLD was greater in group A than in group B (group A, 6.0 +/- 2.1; group B, 4.6 +/- 1.5, p = 0.01). The score of the RLD extent into the deep neck spaces was significantly greater in group A than in group B (group A, 2.3 +/- 1.3; group B, 0.8 +/- 1.0, p < 0.01). Also, the score of the adjacent soft tissue changes was greater in group A than in group B (group A, 2.0 +/- 1.1; group B, 1.0 +/- 1.0, p < 0.01). CONCLUSION: If children present with fever and cervical lymphadenopathy that display retropharyngeal low density with extension into more deep neck spaces as well as changes in more adjacent soft tissue, the possibility of Kawasaki disease should be considered.
الموضوعات
Child , Child, Preschool , Female , Humans , Male , Lymphatic Diseases/complications , Mucocutaneous Lymph Node Syndrome/complications , Neck/diagnostic imaging , Pharyngeal Diseases/complications , Pharynx/diagnostic imaging , Tomography, X-Ray Computedالملخص
A gastric duplication cyst is a rare congenital anomaly. Among gastrointestinal duplication cysts, gastric duplication cyst account for only 3.8%. They tend to be symptomatic in early childhood, but asymptomatic during adulthood. So its diagnosis is incidental. Abdominal pain is the most common complaint in adults, and most cases are discovered incidentally by radiological examination or endoscopic gastroduodenoscopy. Preoperative diagnosis of gastric duplication cyst is difficult, and definitive diagnosis requires pathological examination of the lesion. So far, about 8 cases of gastric duplication cyst have been reported in adults in the Korean literature. We report here a case of multiple gastric duplication cysts presenting with melena in a 14-year-old man, which were detected by endoscopic gastroduodenoscopy, abdominal computed tomography and endoscopic ultrasonography. They were treated by complete excision of the multiple gastric duplication cysts by laparoscopic wedge resection.
الموضوعات
Adolescent , Adult , Humans , Abdominal Pain , Endosonography , Melenaالملخص
PURPOSE: Hand-foot-mouth disease (HFMD) is a common viral illness in children, which is usually mild and self-limiting. However, in recent epidemics of HFMD in Asia, enterovirus 71 (EV71) has been recognized as a causative agent with severe neurological symptoms with or without cardiopulmonary involvement. HFMD was epidemic in Korea in the spring of 2009. Severe cases with complications including death have been reported. The clinical characteristics in children with neurologic manifestations of EV71 were studied in Ewha Womans University Mokdong Hospital. METHODS: Examinations for EV71 were performed from the stools, respiratory secretion or CSF of children who presented neurologic symptoms associated with HFMD by realtime PCR. Clinical and radiologic data of the patients were collected and analyzed. RESULTS: EV71 was isolated from the stool of 16 patients but not from respiratory secretion or CSF. Among the 16 patients, meningitis (n=10) was the most common manifestation, followed by Guillain-Barre syndrome (n=3), meningoencephalitis (n=2), poliomyelitis-like paralytic disease (n=1), and myoclonus (n=1). Gene analysis showed that most of them were caused by EV71 subgenotype C4a, which was prevalent in China in 2008. CONCLUSION: Because EV71 causes severe complications and death in children, a surveillance system to predict upcoming outbreaks should be established and maintained and adequate public health measures are needed to control disease.
الموضوعات
Child , Female , Humans , Asia , China , Disease Outbreaks , Enterovirus , Enterovirus A, Human , Foot , Guillain-Barre Syndrome , Hand , Korea , Meningitis , Meningoencephalitis , Mouth , Mouth Diseases , Myoclonus , Neurologic Manifestations , Polymerase Chain Reaction , Public Healthالملخص
PURPOSE: Renal length offers important information to detect or follow-up various renal diseases. The purpose of this study was to determine the kidney length of normal Korean children in relation to age, height, weight, body surface area (BSA), and body mass index (BMI). MATERIALS AND METHODS: Children between 1 month and 15 years of age without urological abnormality were recruited. Children below 3rd percentile and over 97th percentile for height or weight were excluded. Both renal lengths were measured in the prone position three times and then averaged by experienced radiologists. The mean length and standard deviation for each age group was obtained, and regression equation was calculated between renal length and age, weight, height, BSA, and BMI, respectively. RESULTS: Renal length was measured in 550 children. Renal length grows rapidly until 24 month, while the growth rate is reduced thereafter. The regression equation for age is: renal length (mm) = 45.953 + 1.064 x age (month, 24 months) (R2 = 0.711). The regression equation for height is: renal length (mm) = 24.494 + 0.457 x height (cm) (R2 = 0.894). The regression equation for weight is: renal length (mm) = 38.342 + 2.117 x weight (kg, 18 kg) (R2 = 0.651). The regression equation for BSA is: renal length (mm) = 31.622 + 61.363 xBSA (m2, 0.7) (R2 = 0.715). The regression equation for BMI is: renal length (mm) = 44.474 + 1.163 x BMI (R2 = 0.079). CONCLUSION: This study provides data on the normal renal length and its association with age, weight, height, BSA and BMI. The results of this study will guide the detection and follow-up of renal diseases in Korean children.
الموضوعات
Child , Humans , Body Mass Index , Body Weight , Follow-Up Studies , Kidney , Prone Positionالملخص
The authors report two cases with distension of the obturator internus bursa identified on MR images, and describe the location and characteristic features of obturator internus bursitis; the "boomerang"-shaped fluid distension between the obturator internus tendon and the posterior grooved surface of the ischium.
الموضوعات
Adult , Humans , Male , Bursitis/diagnosis , Hip Joint , Magnetic Resonance Imagingالملخص
Intestinal pseudo-obstruction (IPO) is a rare and poorly understood manifestation of systemic lupus erythematosus (SLE), especially in children. The characteristic clinical feature of IPO is obstruction without an identifiable obstructive lesion. The authors a 13-year-old girl whose first symptom of SLE was IPO. The patient presented with a 3-day history of nausea, bilious vomiting, abdominal distention, and no bowel movement. Simple abdominal radiographs revealed mild dilatation with partial air-fluid levels in the small intestine. Abdominal CT and methylcellulose small bowel studies showed massive ascites, engorgement of the small mesenteric vessels, pleural effusion, and diffuse bowel wall thickening of the gastric antrum, duodenum. and jejunum. The delayed passage of contrast for 15 days after the methylcellulose small bowel studies was suggestive of decreased bowel motility. Laboratory findings were positive for ANA, anti-double-stranded DNA, anti-Smith and lymphopenia. After 10-day treatment with high-dose corticosteroids, the symptoms improved. IPO associated with SLE should be considered in the differential diagnosis for patients presenting with symptoms of intestinal obstruction. Early recognition of IPO in SLE and appropriate therapy are important for prevention of complications and unnecessary surgery. This case raises awareness among pediatricians that although rare, IPO can be the presenting symptom of SLE in children.
الموضوعات
Adolescent , Child , Humans , Adrenal Cortex Hormones , Ascites , Diagnosis, Differential , Dilatation , DNA , Duodenum , Intestinal Obstruction , Intestinal Pseudo-Obstruction , Intestine, Small , Jejunum , Lupus Erythematosus, Systemic , Lymphopenia , Methylcellulose , Nausea , Pleural Effusion , Pyloric Antrum , Unnecessary Procedures , Vomitingالملخص
BACKGROUNDS: Due to the low sensitivity of plain radiography, the diagnosis of early stage ankylosing spondylitis (AS) is often difficult since many patients do not meet the radiographic criteria. The objective of our study was to investigate the diagnostic value of performing multidetector computed tomography (MDCT) of the sacroiliac (SI) joint in the evaluation of AS patients. METHODS: Thirty seven patients with definite or probable AS were evaluated. Plain radiography and MDCT imaging of the pelvis were performed for evaluating the SI joints. Two radiologists analyzed the images, and they graded the sacroiliitis on a scale of 0-4 according to the modified NY criteria. The clinical variables we analyzed included the disease duration, the treatment duration, the prescribed drugs, peripheral joint involvement, enthesopathy, the functional limitations and the BASDAI.. RESULTS: MDCT detected more bilateral sacroiliitis as compared to the plain radiography (86.5% vs. 75.7%, respectively), and MDCT yielded a higher grade of disease in 32.4% (right SI joint) and 24.3% (left SI joint) of the patients. More patients satisfied the modified NY criteria with using MDCT as compared with that when using the plain radiography (81.1% vs. 54.1%, respectively, p=0.002). CONCLUSIONS: Visualization of the sacroiliac joint by MDCT provided a better diagnosis of AS, and especially during the early stage of the disease.
الموضوعات
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Sacroiliac Joint/diagnostic imaging , Sacrum/diagnostic imaging , Sensitivity and Specificity , Severity of Illness Index , Spondylitis, Ankylosing/diagnostic imaging , Tomography, X-Ray Computed/methodsالملخص
BACKGROUND: Rotavirus is the most common cause of childhood gastroenteritis during winter season. Rapid, accurate diagnosis is essential for preventing severe complications of rotaviral gastroenteritis. The sensitivity and specificity of five detection test kits for rotavirus including latex agglutination (LAT), enzyme immunoassay (EIA) and three immunochromatographic methods (ICG) were evaluated in this study. METHODS: A total of 95 stool samples collected from patients with acute gastroenteritis were studied. The test kits were as follows: LAT (Slidex latex, bioMerieux Vitek, France); three kinds of ICG (Dipstick ROTA, Eiken, Japan; SAS Rota Test, SA Scientific, Inc., USA; and ASAN Easy Test Rota strip, ASAN Pharmaceutical., Korea); and EIA (VIDAS Rotavirus, bioMerieux Vitek). The samples showing discordant results were reevaluated by reverse-transcription (RT) PCR and clinical manifestations. RESULTS: Of a total of 95 cases, 56 (58.9%) were positive and 39 (41.1%) were negative. Thirteen cases showed discordant results. Sensitivity and specificity were, respectively, 85.7% and 100% for LAT, 100% and 95% for both of Dipstick ROTA and SAS Rota, 86.7% and 87.5% for ASAN Rota strip and 98.1% and 97.3% for EIA. CONCLUSIONS: LAT was rapid and easy to perform and showed the lowest sensitivity among the five test kits. ICG showed a good agreement with EIA and RT-PCR. EIA was the best in respect of sensitivity and specificity, but difficulty in interpretations of equivocal results and time-consuming procedures were limitations. In conclusion, ICG, which is easy to perform at a low cost, may be an optimal method in place of LAT for the detection of rotavirus.
الموضوعات
Humans , Antigens, Viral/analysis , Chromatography/methods , Enzyme-Linked Immunosorbent Assay/methods , Gastroenteritis/virology , Latex Fixation Tests/methods , Reagent Kits, Diagnostic , Reverse Transcriptase Polymerase Chain Reaction , Rotavirus/immunology , Rotavirus Infections/diagnosis , Sensitivity and Specificityالملخص
Group A rotaviruses are the most common causes of gastroenteritis among infants and young children. The outer capsid layer of the virus is composed of two structural proteins, VP4 and VP7, and they play important roles in protection by eliciting neutralization antibodies. Group A rotaviruses are subdivided into distinct G and P serotypes according to the antigenic differences of the VP7 and VP4, respectively. Rotavirus G9 serotype was thought to be the fifth most common serotype circulating among the population worldwide. In this study, G9 human rotaviruses (HRV) were isolated from fecal samples using MA104 cells and characterized. Characteristic cytopathic effects of rotavirus were observed and rotaviral antigens were confirmed by indirect immunofluorescence antibody test in MA104 cells inoculated with isolated HRV strains. The nucleotide sequences of the VP7 gene of Korean G9 HRV isolated in this study were determined and compared with those of other recent and prototype G9 rotavirus strains from other parts of the world. Also, the nucleotide sequences of VP4 and NSP4 gene of Korean G9 HRV were determined and compared with those of other rotavirus strains from other countries. The results showed that the Korean HRV isolates belong to a G9, P[8] and NSP4 B genotype. The Korean G9 HRV isolates and their nucleotide sequence data would be usefully applied for the vaccine development of HRV in the near future.
الموضوعات
Child , Humans , Infant , Antibodies , Base Sequence , Capsid , Fluorescent Antibody Technique, Indirect , Gastroenteritis , Genotype , Rotavirusالملخص
PURPOSE: We wanted to evaluate the findings and diagnostic accuracy of MDCT for diagnosing occlusive acute myocardial infarction in rabbits. MATERIALS AND METHODS: Myocardial infarction was induced in 14 rabbits. MDCT was performed in the early and delay phases at 1 minute and 6 minutes, respectively, after intravenous contrast injection. The rabbits were sacrificed after scanning. The cardiac specimens were sliced and then stained with triphenyltetrazolium chloride (TTC). The agreement in the transmural extent of infarction between the MDCT scans and the TTC-stained specimens were analyzed by using kappa values. RESULTS: Acute myocardial infarction was found in 9 of 14 rabbits on the TTC-stained specimens and MDCT. The infarcted myocardium was demonstrated as a low-attenuation area on the early phase and as a central low-attenuation area with rim-like enhancement along the endocardial and pericardial sides of the myocardial wall on the delay phase. There was excellent agreement in the scores of the transmural extent of myocardial infarction between the TTC-stained specimens and the early phase scan (kappa value = 0.882, p = 0.000), and there was fair to good agreement between the TTC-stained specimens and the delay phase scan (kappa value = 0.439, p = 0.000). Microscopic examination of the cardiac specimens revealed necrosis of myocardial cells in the central portion and granulation tissue along the endocardial and pericardial sides of the necrotic myocardium. CONCLUSION: 16 slice MDCT scan was useful for the diagnosis of acute myocardial infarction. The early phase scan was more accurate than the delay phase scan for evaluating the transmural extent of myocardial infarction. Histopathologic examination suggested that the low-attenuation area on the delay phase might correspond to necrotic myocardium and the enhanced area might correspond to granulation tissue.
الموضوعات
Rabbits , Diagnosis , Granulation Tissue , Infarction , Myocardial Infarction , Myocardium , Necrosis , Tomography, Spiral Computedالملخص
PURPOSE: We wanted to evaluate the findings and diagnostic accuracy of MDCT for diagnosing occlusive acute myocardial infarction in rabbits. MATERIALS AND METHODS: Myocardial infarction was induced in 14 rabbits. MDCT was performed in the early and delay phases at 1 minute and 6 minutes, respectively, after intravenous contrast injection. The rabbits were sacrificed after scanning. The cardiac specimens were sliced and then stained with triphenyltetrazolium chloride (TTC). The agreement in the transmural extent of infarction between the MDCT scans and the TTC-stained specimens were analyzed by using kappa values. RESULTS: Acute myocardial infarction was found in 9 of 14 rabbits on the TTC-stained specimens and MDCT. The infarcted myocardium was demonstrated as a low-attenuation area on the early phase and as a central low-attenuation area with rim-like enhancement along the endocardial and pericardial sides of the myocardial wall on the delay phase. There was excellent agreement in the scores of the transmural extent of myocardial infarction between the TTC-stained specimens and the early phase scan (kappa value = 0.882, p = 0.000), and there was fair to good agreement between the TTC-stained specimens and the delay phase scan (kappa value = 0.439, p = 0.000). Microscopic examination of the cardiac specimens revealed necrosis of myocardial cells in the central portion and granulation tissue along the endocardial and pericardial sides of the necrotic myocardium. CONCLUSION: 16 slice MDCT scan was useful for the diagnosis of acute myocardial infarction. The early phase scan was more accurate than the delay phase scan for evaluating the transmural extent of myocardial infarction. Histopathologic examination suggested that the low-attenuation area on the delay phase might correspond to necrotic myocardium and the enhanced area might correspond to granulation tissue.
الموضوعات
Rabbits , Diagnosis , Granulation Tissue , Infarction , Myocardial Infarction , Myocardium , Necrosis , Tomography, Spiral Computedالملخص
PURPOSE: We wanted to evaluate the usefulness of MRI 3D quantitative analysis for measuring osteonecrosis of the femoral head in comparison with MRI 2D quantitative analysis and quantitative analysis of the specimen. MATERIALS AND METHODS: For 3 months at our hospital, 14 femoral head specimens with osteonecrosis were obtained after total hip arthroplasty. The patients preoperative MRIs were retrospectively reviewed for quantitative analysis of the size of the necrosis. Each necrotic fraction of the femoral head was measured by 2D quantitative analysis with using mid-coronal and mid-sagittal MRIs, and by 3D quantitative analysis with using serial continuous coronal MRIs and 3D reconstruction software. The necrotic fraction of the specimen was physically measured by the fluid displacement method. The necrotic fraction according to MRI 2D or 3D quantitative analysis was compared with that of the specimen by using Spearman's correlation test. RESULTS: On the correlative analysis, the necrotic fraction by MRI 2D quantitative analysis and quantitative analysis of the specimen showed moderate correlation (r = 0.657); on the other hand, the necrotic fraction by MRI 3D quantitative analysis and quantitative analysis of the specimen demonstrated a strong correlation (r = 0.952) (p < 0.05). CONCLUSION: MRI 3D quantitative analysis was more accurate than 2D quantitative analysis using MRI for measuring osteonecrosis of the femoral head. Therefore, it may be useful for predicting the clinical outcome and deciding the proper treatment option.
الموضوعات
Humans , Arthroplasty, Replacement, Hip , Femur , Hand , Head , Magnetic Resonance Imaging , Necrosis , Osteonecrosis , Retrospective Studiesالملخص
The neurofibromatosis type 1 (NF-1) is a rare hereditary disease of autosomal dominant fashion with the overall incidence of one in 3,000. It is characterized by cafe-au-lait spots of skin, multiple cutaneous neurofibromas and a broad spectrum of clinical finding. Plexiform neurofibroma is a frequent complication of NF-1 but symptomatic involvement of the gastrointestinal tract in children with NF-1 is rare. It may present with complications such as obstruction, dysfunction, pain and hemorrhage. We report that a 13-year-old female diagnosed with NF-1 at the age of 10 years had abdominal pain and showed huge plexiform neurofibromas of right mesocolon in abdominal cavity. She also presented with multiple cafe-au-lait spots and axillary freckling. She was performed a right hemicolectomy with complete excision of the mass and had an uneventful course.
الموضوعات
Adolescent , Child , Female , Humans , Abdominal Cavity , Abdominal Pain , Cafe-au-Lait Spots , Colon, Ascending , Gastrointestinal Tract , Genetic Diseases, Inborn , Hemorrhage , Incidence , Mesentery , Mesocolon , Neurofibroma , Neurofibroma, Plexiform , Neurofibromatosis 1 , Skinالملخص
PURPOSE: To evaluate the ultrasonographic findings of BCG lymphadenitis complicated by BCG vaccination in children MATERIALS AND METHODS: Ultrasonography was performed for 22 cases of BCG lymphadenitis in 21 patients who were diagnosed by clinical (n=10) or pathological (n=11) examinations. Their age ranged from 4 months to 3 years (mean age; 14 months). We retrospectively analyzed the ultrasonographic findings for location, multiplicity, size, shape, margin, echogenecity, posterior enhancement, calcifications, inner anechoic portion and Doppler pattern of the BCG lymphadenitis. RESULTS: The BCG lymphadenitis was found at the axillary area in 15 cases (68%) and at the supraclavicular area in 7 cases (32%). There were ten cases (45%) of solitary lesion and 12 cases (55%) of multiple conglomerated lesions. The maximum diameter ranged from about 0.9 cm to 3.2 cm. The BCG lymphadenitis showed as round (82%), well defined (86%), or heterogeneous hypoechoic (68%) lesions with posterior enhancement (78%). Calcifications were found in 6 cases (27%) and 5 cases (83%) had been vaccinated more than 5 months ago. There were eccentric inner anechoic portions in 16 cases (73%), which were pathologically confirmed as having caseating necrosis. There were increased Doppler flow patterns in 15 cases (68%); 4 cases (18%) were of the central type, 6 cases (27%) were of the peripheral type and 5 cases (23%) were of mixed type. CONCLUSION: BCG lymphadenitis is frequently located at the axillary area adjacent to a vaccination site. The ultrasonographic findings of BCG lymphadenitis are well-defined, round, heterogeneously hypoechoic lesions with posterior enhancement, calcifications and inner eccentric anechoic portion.