Transperineal ultrasonography for evaluation of the perianal fistula and abscess in pediatric Crohn disease: preliminary study

PURPOSE: To assess the feasibility and effectiveness of transperineal ultrasonography (TPUS) for the evaluation of perianal Crohn disease (PCD) in pediatric patients. METHODS: Between September 2010 and August 2013, 64 TPUS examinations were performed in 43 patients (34 males and 9 females; mean age+/-standard deviation, 13.3+/-2.4 years; age range, 6 to 17 years) to evaluate PCD. The pain severity, location, and activity of perianal fistula, the presence of an abscess, and anal canal hyperemia were retrospectively evaluated. Spearman rank correlation analysis was performed to assess the relationship between the severity of the pain and the fistula activity, the presence of an abscess, and anal canal hyperemia. RESULTS: All examinations were successfully performed. Thirty-nine examinations (60.9%) were performed without any pain experienced by the patient, 19 examinations (29.7%) with mild pain, five examinations (7.8%) with moderate pain, and one examination (1.6%) with severe pain. The pain severity was correlated with the fistula activity (P<0.01). An anterior fistula location was more common than a posterior location. Active fistulas and abscesses were identified during 30 examinations (46.9%) and 12 examinations (18.8%), respectively. Anal canal hyperemia was identified in 31 examinations (48.4%). CONCLUSION: TPUS with a color Doppler study is useful for visualizing a perianal fistula or abscess and for assessing its inflammatory activity in pediatric Crohn patients.

Usefulness and Limitation of 2010 ACR/EULAR Classification Criteria in Korean Patients with Early RA

OBJECTIVE: The 2010 New American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria for rheumatoid arthritis (RA) was raised to identify patients with early RA and replaced the 1987 ACR classification criteria. The aims of this study are to assess the availability of new classification criteria and to evaluate its potential limitation. METHODS: A total of 408 patients with newly diagnosed RA were included from 13 secondary or tertiary hospitals in South Korea. The symptom duration was less than 12 months before the diagnosis of RA. RA was defined as either 1987 ACR classification criteria or new 2010 ACR/EULAR criteria. We compared the full details of both classification criteria. RESULTS: The mean symptom duration was 5.1 months. The majority (76.2%) of the patients were female. Two hundred and seventy three patients (66.9%) fulfilled both of the 2010 and 1987 classification criteria. Forty-seven (14.7%) of the 320 patients fulfilling the 1987 criteria did not fulfill the new classification criteria. On the other hand, eighty-eight (24.4%) of the 361 patients fulfilling the 2010 ACR/EULAR classification criteria did not fulfill the 1987 ACR criteria. Thirty-six (55.4%) of the 65 patient with seronegative RA failed to meet the 2010 classification criteria. In case of seropositive RA (n=343), 85 additional patients (24.8%) could be diagnosed as RA using new classification criteria. CONCLUSION: The new 2010 ACR/EULAR classification criteria enable physicians to diagnose more patients with early RA via the help of serology. However, the sensitivity for the diagnosis of seronegative RA is projected to decrease.

A Case of Campomelic Dysplasia without Sex Reversal

Campomelic dysplasia (CD; OMIM #114290), a rare form of congenital short-limbed dwarfism, is due to mutations in SOX9, a member of the SOX (SRY-related HMG box) gene family. Multiparous mother at 38 weeks' gestation delivered a 3,272 g baby boy with characteristic phenotypes including bowing of the lower limbs, a narrow thoracic cage, 11 pairs of ribs, hypoplastic scapulae, macrocephaly, flattened supraorbital ridges and nasal bridge, cleft palate, and micrognathia. He underwent a tracheostomy at the age of three months for severe laryngomalacia after a number of repeated hospitalizations due to respiratory problems and died at the age of four months from progressive respiratory failure. He was diagnosed as having CD based on a novel frameshift mutation (p.Gln458ArgfsX12) in the SOX9 gene, the mutation which has not yet been reported in Korea.

An 18-year experience of tracheoesophageal fistula and esophageal atresia / 소아과

PURPOSE: To determine the clinical manifestations and outcomes of patients with tracheoesophageal fistula (TEF) and esophageal atresia (EA) born at a single neonatal intensive care unit. METHODS: A retrospective analysis was conducted for 97 patients with confirmed TEF and EA who were admitted to the neonatal intensive care unit between 1990 and 2007. RESULTS: The rate of prenatal diagnosis was 12%. The average gestational age and birth weight were 37(+2) weeks and 2.5+/-0.7 kg, respectively. Thirty-one infants were born prematurely (32%). Type C was the most common. The mean gap between the proximal and distal esophagus was 2 cm. Esophago-esophagostomy was performed in 72 patients at a mean age of 4 days after birth; gastrostomy or duodenostomy were performed in 8 patients. Forty patients exhibited vertebral, anorectal, cardiac, tracheoesophageal, renal, limb (VACTERL) association with at least 2 combined anomalies, and cardiac anomaly was the most common. The most common post-operative complications were esophageal stricture followed by gastroesophageal reflux. Balloon dilatation was performed for 1.3 times in 26 patients at a mean age of 3 months. The mortality and morbidity rates were 24% and 67%, respectively, and the most common cause of death was sepsis. The weight of approximately 40% patients was below the 10th percentile at 2 years of age. CONCLUSION: Mortality and morbidity rates of patients with TEF and EA are high as compared to those of infants with other neonatal surgical diseases. Further efforts must be taken to reduce mortality and morbidity and improve growth retardation.

Ultrasonographic Assessment in Rheumatoid Arthritis / 대한류마티스학회지

The administration of disease-modifying antirheumatic drugs (DMARDs) in the early period of rheumatoid arthritis (RA) is critical for protecting against joint damage and inducing remission. Physicians need to identify patients at risk of progression to RA at the early stages of arthritis. Musculoskeletal ultrasonography (MSUS) allows the direct visualization of synovitis and bone erosion in the early phase, and may be useful for differentiating early rheumatoid arthritis from other inflammatory arthritis. Power Doppler sonography is a promising tool for assessing the disease activity and monitoring the effects of DMARDs. This article reviews the current status and recent advances in MSUS imaging in RA.

A Case of Scalp AV Malformation Cured by Embolization

Congenital arteriovenous malformation of the scalp is a relatively rare disease, and its treatment remains challenging because of the unpredictable behavior and high recurrence rate. We report herein a case of an 8 days old male referred for a pulsating scalp mass in the right parietal area with congestive heart failure which was found to be due to AV malformation of the scalp. Embolization of two feeding arteries arising from the right superficial temporal artery on day of life 11 resulted in a complete cure without recurrences.

Comparison of the Clinical Manifestations, Brain MRI and Prognosis between NeuroBehcet's Disease and Neuropsychiatric Lupus

BACKGROUND: Neuropsychiatric systemic lupus erythematosus (NPSLE) shows some similarities to neuroBehcet's disease (NBD) in that both conditions have some analogous clinical features and they are both pathologically associated cerebral vasculopathy. This study compared the clinical manifestations, brain MRI findings and prognosis of NPSLE and NBD patients. METHODS: Forty three patients with NPSLE (n = 25) or NBD (n = 18), who were monitored at a single center, were enrolled in this study. We retrospectively analyzed the clinical and brain MRI data. The neuropsychiatric manifestations were classified in both groups according to the new American College of Rheumatology nomenclature for NPSLE. RESULTS: The diffuse symptoms that included mood disorders, psychosis, confusion, cognitive dysfunctions, generalized seizures and headaches other than migraine or cluster headaches were more commonly observed in the NPSLE patients, while the frequency of focal diseases such as cranial neuropathy tended to be higher in the NBD patients. The brain MRI revealed that the NBD patients had more abnormalities in the brain stem than did the NPSLE patients. Most of the patients improved, at least partially, after being treated with glucocorticoid and/or immune suppressants. However, the disease course differed significantly between the two groups. There were more episodic cases in the NPSLE group of patients, while there were more remittent cases in the NBD group of patients. CONCLUSION: NPSLE had a tendency to cause diffuse neuropsychiatric manifestations, and it has a different predilection of brain lesions compared with NBD. The NBD patients showed a poorer outcome than did the NPSLE patients, suggesting that different therapeutic strategies for the two diseases need to be considered.

1 year follow-up results of prenatally diagnosed unilateral hydronephrosis according to renal sonographic findings at 1 month of age / 소아과

PURPOSE: The natural courses of prenatally diagnosed hydronephrosis(HN) are diverse. Our purpose was to determine if the findings of renal ultrasonography(USG) in patients with prenatal HN at 1 month of age can predict the 1 year follow-up results and determine the guideline of follow-up study. METHODS: Among 462 hydronephrotic patients registered between 1996 and 2004, 153 unilateral hydronephrotic renal units were enrolled in this study, bilateral HN, vesicoureteral reflux and other associated anomaly were excluded. These were classified into four groups respectively, according to anterior posterior pelvic diameter(APPD) or Society for Fetal Urology(SFU) grading by USG findings at 1 month after birth. Renal USG and Tc(99m)-mercaptoacetyl triglycerine(MAG3) scan were done according to a set protocol. RESULTS: Most cases improved or remained stationary. No one underwent an operation SFU grade 1,2 groups and only one case of SFU grade 3 group was operated. Thirty two cases(64 percent) were operated on among the 50 cases of SFU grade 4 group. 0/2(0 percent), 5/11(45.5 percent), 11/17(64.7 percent) and 16/20(80 percent) were operated on in each group with APPD 30 mm, and the operation risk is higher as the APPD is increased. CONCLUSION: In group with SFU grade below 3 and APPD below 10 mm, we can delay the follow-up study beyond existing set protocol. Operations are recommended immediately if diuretic renogram show the obstructive pattern or decreased renal function in SFU grade 4 group with APPD over 10 mm.

A Case of Haddad Syndrome / 대한소아소화기영양학회지

The combination of Congenital central hypoventilation syndrome with Hirschsprung's disease, also known as Haddad syndrome, belongs to the family of diseases now designated as Neurocristopathies. We have experienced a case of Haddad syndrome in a male infant who presented with repetitive abdominal distension, bilious vomiting, and sleep apnea. Following colon study and rectal biopsy disclosed the absence of the ganglion cell. And the infant could not be weaned from mechanical ventilation since birth because of the absence of effective, spontaneous respiration during sleep. As he was diagnosed as Haddad syndrome, tracheostomy and ileostomy were performed consecutively. At the age of 4 months, he was relatively healthy but remained ventilator-dependent. We report the first Korean case of Haddad syndrome with a brief review of the related literature.

Candida albicans Brain Abscesses in Extremely Low Birth Weight Infant Treated with Amphotericin B, Flucytosine, and Fluconazole

Systemic candidiasis in premature infants often involves the central nervous system (CNS) but only a few reported cases of candidiasis involving the brain parenchyma were found in a recent search of the literature and there is no uniformly accepted approach for treatment of CNS Candida infection in neonates. We report a case of extremely low birth weight infant who had candiduria and Candida meningitis with multiple abscesses formation in brain parenchyma who was successfully treated with long term amphotericin B, flucytosine, and fluconazole therapy without surgical drainage.

A Case of Azathioprine Induced Recurrent Acute Pancreatitis in a Patient with Behcet's Disease / 대한류마티스학회지

Acute pancreatitis is a serious disease with a fatality rate of up to 15%. Drugs are considered a rare cause of acute pancreatitis and azathioprine has been linked to subsequent acute pancreatitis in several case reports. We present a 48-year-old female who developed abdominal pain and elevation of serum amylase and lipase levels following azathioprine treatment for Behcet's disease. Abdominal CT scan reveals diffuse enlarged pancreas and large amount of fluid collection at peritoneal cavity. She was recovered with conventional therapy for acute pancreatitis and withdrawal of azathioprine. When the patient erroneously took the drug once again, acute pancreatitis was more rapidly developed than initial attack.

Diffuse Esophageal Leiomyomatosis in a Child with Alport Syndrome: Case Report

Diffuse esophageal leiomyomatosis is an exceedingly rare, benign, neoplastic condition occurring predominantly in children and young adults. This condition may occur as an isolated finding, or it may be associated with Alport syndrome. We report a case of diffuse esophageal leiomyomatosis with Alport syndrome in a 5-year-old girl who had presented with recurrent pneumonia, and present a review of the literature. We suspected Alport syndrome in the patient because she had a clinical history of congenital cataracts and hematuria, as well as imaging findings of diffuse esophageal leiomyomatosis. Alport syndrome was subsequently confirmed by electron microscopy of the kidney.

Sonography-guided Gastrografin Enema for Meconium Plug Syndrome in Premature Newborns: Preliminary Results

PURPOSE: To evaluate the usefulness of sonography-guided Gastrografin enema for the diagnosis and treatment of meconium plug syndrome in premature newborns in a neonatal intensive care unit (NICU). MATERIALS AND METHODS: Fifteen premature newborns underwent 23 sonography-guided Gastrografin enemas on the 8th day of life on average (range: 3 days-21 days). The gestational age and birth weight (mean+/-standard deviation) of the patients were 29+/-2 weeks and 999+/-148 g, respectively. The diagnosis of meconium plug syndrome was based on relevant clinical and radiological findings. Diluted Gastrografin (1:3, 17-45 ml) was administered through a rectal tube under the guidance of sonography until it reached to the cecum. Thereafter, immediate post-procedure and delayed portable abdominal radiographs were taken. All the procedures were done within the NICU. We reviewed the dates of meconium evacuation and the first feeding after the last enema from the clinical data. Radiologically, the timing of the radiographic improvement after the last enema was checked. In cases of distended distal ileum with meconium on pre-enema sonography, follow-up sonography was performed to determine the interval change after gastrografin enema. RESULTS: None of the sonography-guided Gastrografin enemas performed within the NICU caused procedure-related complications. Meconium was evacuated within one day in all patients. In 14 patients, on average, feeding was started on the 7th day and radiographic improvement was observed on the 3rd day. Four of the five patients showing a distended distal ileum with meconium revealed a decrease in ileal distension on follow-up sonography. On the other hand, the other patient, who did not show such a decrease on follow-up sonography, was found to have ileal stenosis during subsequent surgery. CONCLUSION: Sonography-guided Gastrografin enema is a safe and effective bedside procedure, when performed in the NICU for the diagnosis and treatment of meconium plug syndrome.

Multi-Slice Spiral CT of Living-Related Liver Transplantation in Children: Pictorial Essay

In pediatric living-related liver transplantation, preoperative evaluation of the recipient is important for surgical planning, while the accurate diagnosis of postoperative complications is essential for graft salvage. Multiplanar and three-dimensional imaging using multi-slice spiral CT can be used for preoperative vascular imaging, as well as for evaluating postoperative complications. In this essay, we describe the usefulness of multi-slice CT, combined with a variety of different reconstruction techniques, for the preoperative evaluation of transplant recipients. In addition, we demonstrate the multi-slice CT findings of postoperative complications, including vascular stenosis or thrombosis, bile duct leak or stricture, and extrahepatic fluid collection.

Unusual Infarction of the Accessory Spleen or Polysplenia in Two Children: Case Report

The usual imaging findings of common splenic infarction are well known, while the findings for splenic infarctions in the accessory spleen or polysplenia are rare; these unusual imaging findings may make the diagnosis difficult. We report here on two patients who have complained of abdominal pain, and they were diagnosed as splenic infarction that developed in either the accessory spleen or as has having polysplenia. We can diagnose splenic infarction that unusually develops in the accessory spleen or polysplenia when we identify a round, hyperechoic, avascular solid mass on US, or when we identify a round, rim-enhancing, hypodense solid mass with adjacent inflammatory changes and a small amount of ascites on CT that is adjacent to the normal spleen or in one of splenules of polysplenia in the clinical settings of acute abdominal pain.

Venous Complications after Pediatric Liver Transplantation / 대한이식학회지

PURPOSE: We performed this study to assess the incidence of venous complications, including portal vein and hepatic vein stenosis, in both split cadaveric and living donor liver transplants and to assess the diagnostic and therapeutic modalities of these lesions. METHODS: Seventy-six liver transplantations were performed in 75 children with split (5) or living donor (71) graft between December 1994 and March 2002. Patients' data were analyzed retrospectively with special emphasis on venous complications. RESULTS: Venous complications occurred in 14 patients (18.6%) including hepatic vein stenosis in 8, portal vein stenosis in 4, portal vein thrombosis in 1, and combined portal vein thrombosis and hepatic artery stenosis in 1 patient. Venous complications were accompanied by abnormality of liver fuction, ascites, progressed splenomegaly, and gastrointestinal bleeding. To diagnose the venous complications, Doppler ultrasonography was performd at first, and those were confirmed by angiography or CT. Hepatic vein stenosis was managed by percutaneous transhepatic angioplasty (6), angioplasty followed by reposition of graft (1), and supportive care only 1 patient. Portal vein complications were managed by angioplasty (4), angioplasty followed by mesocaval shunt (1), and combined revascularization and angioplasty (1). The overall survival rate was 86% (12 of 14 patients). CONCLUSION: Close surveillance of the complication of vascular anastomoses and multidisciplinary approach to treat of venous complication after pediatric liver transplantation have made it possible to reduce the graft loss and mortality. Aggressive and successful radiologic intervention should be considered to eliminate the need for surgical revision, portacaval shunting or retransplantation.

Guideline in the Management of Antenatally Diagnosed Unilateral Hydronephrosis

PURPOSE: Neonatal hydronephrosis has been detected with increasing frequency with the widespread use of prenatal ultrasonography, but the consensus about its postnatal management has not yet been reached, especially about surgical intervention. We attempted to determine the guideline of follow-up study and surgical intervention of hydronephrosis by analyzing clinical outcomes of neonates with hydronephrosis. MATERIALS AND METHODS: Between 1994 and 2000, 128 hydronephrotic kidneys were postnatally confirmed. Cases associated with other urologic anomalies were excluded and 90 unilateral hydronephrotic kidneys with a minimum follow-up of 12 months were enrolled in this study. We classified the patients into 6 groups according to the anterior posterior pelvic diameter(APPD) at initial ultrasonography(USG) within 1 month after birth. Renal USG and Tc99m-mercaptoacetyl triglycerine(MAG3) scan were done according to a set protocol, and pyeloplasty was performed when indicated according to our protocol. RESULTS: Most cases whose APPD were below 10 mm improved or resolved. Only few cases with APPD above 20 mm showed spontaneous improvement and most(88%) had undergone operation. Those with initial APPD within 10-19 mm showed variable outcomes. When the risk factors for irreversible renal functional deterioration were analyzed, the age at pyeloplasty and pre-operative functional deficit were significant. CONCLUSION: We concluded that in infants with initial APPD below 10 mm, consideration of surgery is not needed, and in those with initial APPD above 20 mm, early operation is recommended. Our set protocol based on initial USG is useful, but the cut-off value of relative renal function(RRF) for operation might be increased to 40% to improve post operative RRF.

Endoscopic Retrograde Cholangiopancreatography in Thirty-four Children / 대한소화기내시경학회지

BACKGROUND/AIMS: Endoscopic retrograde cholangiopancreatography (ERCP) is being used with increasing frequency as a diagnostic and therapeutic tool in children with suspected disorders of the pancreaticobiliary tract. We reviewed ERCPs performed in children and investigated clinical indications and usefulness of ERCP. METHODS: A total of 80 ERCPs were performed in 34 patients (age: 17 months~15 yrs) at Asan Medical Center from 1994 to 2001. Adult side-viewing duodenoscope, Olympus JF or TJF, was used for all procedures. General anesthesia was used in 73% of the patients, whereas intravenous sedation was employed in the remainder. RESULTS: Cannulation was successful in 77 attempts (96%). ERCP was commonly indicated for the evaluation of pancreatic disease (18 cases) such as acute pancreatitis (4), recurrent pancreatitis (4), chronic pancreatitis (9), and non-resolving acute pancreatitis (1). Biliary tract diseases (15 cases) were common bile duct stones (4), choledochal cyst (8), and traumatic or nontraumatic common bile duct stricture (3). Therapeutic ERCP (n=20) included sphincterotomy (55.9%), insertion of stents (12%), and removal of common bile duct stones or pancreatic duct stones with balloon or basket (32%). The complications were developed in 12 out of 80 attempts (15%) and resolved with medical management. CONCLUSIONS: ERCP seems to be a useful and relatively safe procedure in the evaluation of pancreaticobiliary disorders in children and also can be used for nonoperative treatment of these diseordrs.

Horseshoe Adrenal Gland in a Newborn with Right Isomerism

Horseshoe adrenal gland is a rare congenital abnormality associated with multiple congenital anomalies that may involve the cardiovascular, central nervous, and genitourinary systems. In this report, we describe the sonographic findings of horseshoe adrenal gland in a newborn with right isomerism a frequently associated cardiovascular anomaly.

Systemic Arterial Supply to the Normal Basal Segments of the Left Lower Lobe: Case Report

Systemic arterial supply to the normal basal segments of the left lower lobe is a rare congenital anomaly which has characteristic radiologic features. The lung supplied by the anomalous systemic artery has a normal bronchial tree. We recently encountered a case in which the diagnosis of systemic arterial supply to the normal basal segments of the left lower lobe was strongly suggested by the chest radiographic, CT, and MRI findings. The diagnosis was subsequently confirmed by left lower lobectomy.