An Interdisciplinary Approach to Müllerian Outflow Tract Obstruction Associated with Cloacal Malformation and Cloacal Exstrophy.

People with cloacal malformation and 46,XX cloacal exstrophy are at risk of developing Müllerian outflow tract obstruction (OTO). Management of OTO requires expertise of many medical and surgical specialties. The primary presenting symptom associated with OTO is cyclical and later continuous pain and can be initially quelled with hormonal suppression as a temporizing measure to allow for patient maturation. The decision for timing and method of definitive treatment to establish a patent outflow tract that can also be used for penetrative sexual activity and potential fertility is a complicated one and incredibly variable based on patient age alone. To understand the management approach to OTO, we put forth five phases with associated recommendations: (1) caregiver and patient education and evaluation before obstruction; (2) presentation, diagnosis, and symptom temporization; (3) readiness assessment; (4) peri-procedural management; (5) long-term surveillance. This review will emphasize the importance of interdisciplinary team management of the complex shared medical, surgical, and psychological decision making required to successfully guide developing patients with outflow obstruction secondary to cloacal malformations and cloacal exstrophy through adolescence.

The radiologist's role in assessing differences of sex development.

When infants are identified with a difference of sex development (DSD), a thoughtful approach to imaging is essential to appropriate clinical management. This review provides a comprehensive guide for radiologists who are tasked with performing this critical assignment. We review the embryologic basis of DSDs, with attention to the imaging findings that can indicate specific diagnoses. We also discuss techniques for optimal imaging, including strategies for identifying the gonads by US, tactics for performing genitograms with fluoroscopy and contrast-enhanced US, and the appropriate utilization of MRI. Finally, we review the clinical data and imaging findings that characterize some of the most common DSDs, including congenital adrenal hyperplasia, complete androgen insensitivity syndrome and gonadal dysgenesis.

Distal Vaginal Atresia with Spontaneous Perforation: A Case Report.

BACKGROUND: The definitive treatment of distal vaginal atresia is surgery, but menstrual suppression is often helpful for initial management. CASE: A 13-year-old presented with primary amenorrhea and progressive abdominal pain. She was diagnosed with distal vaginal atresia and started on hormonal suppression. She then re-presented with heavy vaginal bleeding, and follow-up imaging revealed that spontaneous perforation had occurred. There was now evidence of a tract leading from the obstructed vaginal bulge to the introitus. Vaginoplasty was complicated by the tortuosity of the tract. Under ultrasound guidance, a pull-through vaginoplasty was performed. SUMMARY AND CONCLUSION: Spontaneous perforation of an atretic vagina is rare, but in such cases, urgent vaginoplasty is indicated to prevent infection. Despite the presence of a spontaneous tract leading to the obstruction, vaginoplasty can be complex, and intraoperative ultrasound could be beneficial.

Spectrum of Syndromic Disorders Associated with Pediatric Tumors: Evolving Role of Practical Imaging Assessment.

Although pediatric tumors are largely sporadic in cause, continued advancements in science have elucidated a growing body of tumors that are associated with syndromes. Early identification of these syndromic disorders associated with developing tumors can alter the course of disease and potentially save lives. Medical imaging has a pivotal role in screening surveillance, diagnosis, and management of these tumors. Understanding characteristic manifestations of these syndromes is important to optimize image utilization. This article discusses clinically important syndromes associated with pediatric tumors with brief overview of the background, genetics, and clinical features. Diagnosis, imaging, management, and treatment are also reviewed.

Pediatric Musculoskeletal Ultrasound: Practical Imaging Approach.

OBJECTIVE: The purpose of this article is to review some of the common indications for pediatric musculoskeletal ultrasound examination, with emphasis given to imaging technique, normal anatomy, and the spectrum of pathologic findings seen in the pediatric population. CONCLUSION: Ultrasound is an essential first-line tool in pediatric musculoskeletal imaging. It aids in determining which patients may benefit from further imaging, including radiography, CT, and MRI.

Pediatric Abdominal Organ Transplantation: Current Indications, Techniques, and Imaging Findings.

The anatomy, normal postoperative radiological appearance, and imaging features of common postoperative complications of pediatric abdominal transplants are reviewed, including renal, liver, and intestinal transplants. Doppler ultrasound is the mainstay of imaging after transplantation. Computed tomography (CT) and CT angiography, MR imaging and magnetic resonance (MR) angiography, MR cholangiopancreatography, conventional angiography, and nuclear medicine imaging may be used for problem-solving in pediatric transplant patients. Accurate and timely radiological diagnosis of transplant complications facilitates appropriate treatment and minimizes morbidity and mortality.

Radiologic Professionalism in Modern Health Care.

Modern radiology is at the forefront of technological progress in medicine, a position that often places unique challenges on its professional character. This article uses "Medical Professionalism in the New Millennium: A Physician Charter," a document published in 2002 and endorsed by several major radiology organizations, as a lens for exploring professional challenges in modern radiology. The three main tenets of the Charter emphasize patient welfare, patient autonomy, and the reduction of disparities in health care distribution. This article reviews the ways in which modern technology and financial structures potentially create stressors on professionalism in radiology, while highlighting the opportunities they provide for radiologists seeking to fulfill the professional goals articulated in the Charter. Picture archiving and communication systems (PACS) and voice recognition systems have transformed the speed of radiology and enhanced the ability of radiologists to improve patient care but also have brought new tensions to the workplace. Although teleradiology may improve global access to radiologists, it may also promote the commoditization of radiology, which diminishes the professional stature of radiologists. Social media and patient portals provide radiologists with new forums for interacting with the public and patients, potentially promoting patient welfare. However, patient privacy and autonomy are important considerations. Finally, modern financial structures provide radiologists with both entrepreneurial opportunities as well as the temptation for unprofessional conduct. Each of these advances carries the potential for professional growth while testing the professional stature of radiology. By considering the risks and benefits of emerging technologies in the modern radiology world, radiologists can chart an ethical and professional future path.

Imaging findings in children with proliferative disorders following multivisceral transplantation.

BACKGROUND: Multivisceral transplantation represents an important treatment option for children with intestinal failure. The attendant immunosuppression can lead to a spectrum of cellular proliferations including benign and malignant smooth muscle tumors and lymphoproliferative disorders, many related to cellular dysregulation from Epstein-Barr virus infection. OBJECTIVE: The purpose of this study is to investigate the rates of post-transplantation proliferative disorders among children with multivisceral transplantation and to characterize the imaging and pathological features of these disorders. MATERIALS AND METHODS: We identified all consecutive children who underwent multivisceral transplant from August 2004 to October 2011 with at least 27 months of clinical and imaging follow-up. We reviewed medical records to determine the underlying causes of the multivisceral transplant, age at transplantation, onset of neoplasm development, and outcome. Two pediatric radiologists reviewed all imaging studies independently and diagnosis of disease was made by consensus interpretation. Pathological specimens were reviewed for histopathological findings of post-transplantation neoplasm in this pediatric patient population. RESULTS: The study population consisted of 14 consecutive pediatric patients (7 boys and 7 girls; mean age 26 months, range 4-113 months). Of these 14 children, 4 (29%) developed histologically confirmed post-transplant neoplasms at a mean time of 2.4 years after multivisceral transplantation. Types of neoplasms included post-transplant lymphoproliferative disorder (PTLD) in three (21%) and Epstein-Barr-virus-associated smooth muscle tumor in two (14%). (One child developed both neoplasms following transplantation). Both children with smooth muscle tumor associated with Epstein-Barr virus presented with characteristic hypointense solid masses with peripheral rim enhancement on cross-sectional imaging studies. The mortality rate of children who developed post-transplant neoplasms was higher than that of those who did not develop post-transplant neoplasm (50% vs. 10%, P = 0.17), suggesting a possible risk factor for death. CONCLUSION: Post-transplant neoplasm in children with multivisceral transplantation occurs with high frequency, often presents as Epstein-Barr-virus-associated smooth muscle tumor showing characteristic peripheral rim enhancement on cross-sectional imaging studies.

Pediatric abdominal pain: use of imaging in the emergency department in the United States from 1999 to 2007.

PURPOSE: To evaluate use of imaging in children with acute abdominal pain who present to U.S. emergency departments (EDs). MATERIALS AND METHODS: This study received expedited review by the institutional review board. The National Hospital Ambulatory Medical Care Survey is a government-administered yearly survey of EDs that is used to estimate ED care throughout the United States. This retrospective cohort study interrogated the database for the period from 1999 to 2007. Univariate regression analysis was performed, and a multivariate regression model was developed. RESULTS: From 1999 to 2007, 16 900 000 pediatric ED visits were made for acute abdominal pain. Odds of undergoing computed tomography (CT) in this population increased during each year of the study period. No significant changes occurred in use of ultrasonography, number of patients admitted to the hospital, or number of patients with acute appendicitis. A multivariate model for CT use revealed increased odds of CT use in teens, white patients, the Midwest region, urban settings, patients with private insurance, and patients who were admitted or transferred. Odds of undergoing CT were significantly lower among patients who presented to a pediatric-focused emergency department (adjusted odds ratio, 0.72; 95% confidence interval: 0.58, 0.90). CONCLUSION: The main findings of this study are that the rate of CT use in the evaluation of abdominal pain in children increased every year between 1999 and 2007 and that the use of CT was greater among children seen in adult-focused EDs. Factors affecting CT use include sex, race, age, insurance status, and geographic region.

Prevalence of malignancy in thyroid nodules with an initial nondiagnostic result after ultrasound guided fine needle aspiration.

The objective of this study was to determine the rate of malignancy in thyroid nodules with an initial nondiagnostic fine needle aspiration. From October 2001 to April 2007, biopsies were performed on 1344 thyroid nodules in our practice. Biopsies were performed on nodules using 25-27 gauge needles, ultrasound guidance and multiple passes using both suction and capillary action. We retrospectively reviewed the results of these biopsies as well as any further management of nodules that received nondiagnostic results (IRB HUM00006459). Following initial biopsy, 295/1344 (21.9%) of nodules received nondiagnostic pathologic results. Of this population, 39 nodules (13.1%) were lost to follow-up. Of the remaining 256 nodules that received a repeat FNA, surgical excision, or greater than 24 months of clinical and imaging follow-up, only five cancers were detected, representing only 2% of the population that received an initial nondiagnostic biopsy result. All of these cancers were papillary neoplasms. When rigorous, ultrasound-guided, fine needle aspiration of thyroid nodules is performed, a nondiagnostic histopathologic result should not be interpreted as suspicious for thyroid cancer. Given the low rate of malignancy in this population (2%), we suggest that clinical and imaging follow-up of these nodules, opposed to repeat sampling, is warranted.

Anaplastic large cell lymphoma of the esophagus in a pediatric patient.

We report a 3-year-old boy who initially presented with abdominal pain and was subsequently found to have an esophageal perforation. The child did not respond to conservative management, and subsequent lymphadenopathy led to a lymph node biopsy demonstrating an anaplastic lymphoma kinase (ALK)+ anaplastic large cell lymphoma. Esophageal perforation and thickening is most commonly seen in children with a history of esophageal intervention or foreign body/caustic ingestion. Esophageal involvement in children with non-Hodgkin lymphoma (NHL) has not, to our knowledge, been reported in the literature. This case illustrates an unusual presentation of pediatric NHL.

Accuracy of CT-guided percutaneous core needle biopsy for assessment of pediatric musculoskeletal lesions.

BACKGROUND: CT-guided percutaneous core needle biopsy has been shown in adults to be an effective diagnostic tool for a large number of musculoskeletal malignancies. OBJECTIVE: To characterize our experience with CT-guided percutaneous core needle biopsy of pediatric bone lesions and determine its utility in diagnosing pediatric osseous lesions, in a population where such lesions are commonly benign. MATERIALS AND METHODS: From 2000 to 2009, 61 children underwent 63 CT-guided percutaneous biopsies. Radiological, pathological and clinical records were reviewed. RESULTS: Fourteen biopsies (22%) were performed on malignant lesions, while 49 biopsies (78%) were performed on benign lesions. Forty-nine of the 63 biopsies (78%) were adequate; these children underwent no further tissue sampling. Fourteen of the 63 biopsies (22%) were inadequate or non-conclusive. Of these patients, 12 underwent open biopsy. Retrospective analysis of percutaneous biopsies in these patients demonstrates that 9/12 provided clinically relevant information, and 4/12 patients received final diagnoses that confirmed initial core biopsy findings. No malignancies were diagnosed as benign on percutaneous biopsy. Overall, percutaneous core needle biopsy provided accurate diagnostic information in 84% (53/63) of biopsies. CONCLUSION: Our results demonstrate that CT-guided percutaneous biopsy is safe and beneficial in children. This study supports the use of CT-guided percutaneous core needle biopsy for primary diagnosis of pediatric bone lesions.

Validation of US as a first-line diagnostic test for assessment of pediatric ileocolic intussusception.

BACKGROUND: From the early 1980s onward, US has been considered a possible primary modality to assess patients for ileocolic intussusception. Since 2001, our institution has routinely used US to assess patients for ileocolic intussusception. OBJECTIVE: We analyzed 7 years of institutional experience to assess the value of US as a primary diagnostic test for intussusception. MATERIALS AND METHODS: This study was IRB-approved. From 1 January 2001 through 16 December 2007 814 US examinations for intussusception were performed in children aged 10 years and younger. Clinical records and radiological reports were reviewed for each patient, and cases were classified as true-positive, true-negative, false-positive, or false-negative. RESULTS: Of the 814 US examinations, 112 (13.8%) were interpreted as positive for intussusception, and of these, 96 were confirmed by enema, 1 was confirmed by surgery, and 15 (13.4%) were false-positive. Of the 814 examinations, 700 (85.9%) were interpreted as negative for intussusception, and of these, 698 (99.7%) were true-negative, and 2 were false-negative. Less than 1% of studies were nondiagnostic. The overall sensitivity of US for detecting intussusception was 97.9% and specificity was 97.8%. The positive predictive value of the test was 86.6% and the negative predictive value was 99.7%. CONCLUSION: US is a sensitive and specific test for detecting ileocolic intussusception and should be utilized as a first-line examination for assessment of possible pediatric intussusception.