The influence of orbital architecture on strabismus in craniosynostosis.

PURPOSE: To better characterize the correlation of bony orbital dysmorphology with strabismus in craniosynostosis. METHODS: The medical records of patients with craniosynostosis with and without strabismus seen at Rady Children's Hospital (San Diego, CA) from March 2020 to January 2022 were reviewed retrospectively in this masked, case-control study. Computed tomography scans of the orbits were analyzed to obtain dimensions of the orbital entrance and orbital cone. Primary outcome was correlation of strabismus with orbital measurements. RESULTS: A total of 30 orbits from 15 patients with strabismus and 15 controls were included. Craniofacial disorders included in the study were nonsyndromic craniosynostosis (63%), Crouzon syndrome (13%), Apert syndrome (13%), and Pfeiffer syndrome (10%). Orbital index (height:width ratio) (P = 0.01) and medial orbital wall angle (P = 0.04) were found to differ significantly between the strabismus and control groups. CONCLUSIONS: In our small cohort, bony orbital dimensions, including the ratio of orbital height to width and bowing of the medial orbital wall, were associated with strabismus in craniosynostosis.

Acute Pancreatitis Associated With Cytarabine During the Treatment of Pediatric Acute Myeloid Leukemia.

Pancreatitis is a common complication of many pediatric oncology drugs - most commonly asparaginase, followed by pentamidine, mercaptopurine, corticosteroids, and trimethoprim-sulfamethoxazole. Cytarabine-associated pancreatitis is not often mentioned in the pediatric oncology literature. We report the case of a 10-year-old female with acute myeloid leukemia who developed cytarabine-associated pancreatitis.

Classification of pediatric urinary tract dilation: the new language.

The multidisciplinary consensus on the classification of prenatal and postnatal urinary tract dilation (UTD classification system) was created to unify the language used to describe urinary tract dilation on antenatal and postnatal ultrasound examinations and thereby facilitate communication among providers and improve outcomes research. The background and new classification system are described in this review, with imaging examples.

Cyanotic Congenital Heart Disease: Essential Primer for the Practicing Radiologist.

The cyanotic congenital heart diseases are a rare and heterogeneous group of disorders, often requiring urgent neonatal management. Although echocardiography is the mainstay for imaging, continued technological advances have expanded the role for computed tomography and magnetic resonance imaging, helping to limit invasive cardiac catheterization. In this article, the authors review the broad spectrum of cyanotic congenital heart disease, focusing on the utility of advanced noninvasive imaging modalities while highlighting key clinical features and management considerations.

Skeletal and Brain Abnormalities in Fucosidosis, a Rare Lysosomal Storage Disorder.

Fucosidosis is a rare genetic lysosomal storage disorder caused by a deficiency in alpha- L-fucosidase. We present a case of a 4-year, 11-month-old girl with developmental delay, as well as skeletal and brain abnormalities as shown on X-ray and MRI. Her spinal X- rays demonstrated lumbar kyphosis and anterior beaking of lumbar vertebral bodies. Lower iliac segment constriction, increased angulation of the acetabular roof, and widening of the ribs were apparent on abdominal X-ray. Her brain MRI illustrated symmetric T1 hyperintensity and T2 hypointensity of the bilateral globi pallidi. The case report highlights clinical and imaging findings of this rare disease.

Carotid artery pseudoaneurysm: A rare complication following tonsillectomy.

Tonsillectomy is one of the most common surgical procedures performed in children. The most frequent complications are dehydration and bleeding. We present the case of a 6 year old child who developed an internal carotid artery pseudoaneurysm following elective tonsillectomy, necessitating urgent coil embolization and stenting. This is the first reported case in the pediatric population of a vascular injury that manifested in a delayed fashion (6 months) after routine tonsillectomy, and is also one of the youngest reported cases. It is imperative for the otolaryngologist to be aware of this rare complication.

Breast Imaging-Reporting and Data System (BI-RADS) classification in 51 excised palpable pediatric breast masses.

INTRODUCTION: The American College of Radiology Breast Imaging Reporting and Data System (BI-RADS) classification was developed to risk stratify breast lesions and guide surgical management based on imaging. Previous studies validating BI-RADS for US do not include pediatric patients. Most pediatric breast masses present as palpable lesions and frequently undergo ultrasound, which is often accompanied with a BI-RADS classification. Our study aimed to correlate BI-RADS with pathology findings to assess applicability of the classification system to pediatric patients. METHODS: We performed a retrospective review of all patients who underwent excision of a breast mass at a single center from July 2010 to November 2013. We identified all patients who underwent preoperative ultrasound with BI-RADS classification. Demographic data, imaging results, and surgical pathology were analyzed and correlated. RESULTS: A total of 119 palpable masses were excised from 105 pediatric patients during the study period. Of 119 masses, 81 had preoperative ultrasound, and BI-RADS categories were given to 51 masses. Of these 51, all patients were female and the average age was 15.9 years. BI-RADS 4 was given to 25 of 51 masses (49%), and 100% of these lesions had benign pathology, the most common being fibroadenoma. CONCLUSIONS: Treatment algorithm based on BI-RADS classification may not be valid in pediatric patients. In this study, all patients with a BI-RADS 4 lesion had benign pathology. BI-RADS classification may overstate the risk of malignancy or need for biopsy in this population. Further validation of BI-RADS classification with large scale studies is needed in pediatric and adolescent patients.

Does abdominal pain duration affect the accuracy of first-line MRI for pediatric appendicitis?

PURPOSE: To compare the accuracy of first-line MRI for pediatric appendicitis between children with different durations of abdominal pain. MATERIALS AND METHODS: Between November 2012 and September 2013, 364 pediatric patients underwent MRI for clinically suspected appendicitis. Patients were stratified into one group with pain lasting less than or equal to 1 day and pain longer than 1 day. RESULTS: In patients with early abdominal pain, appendicitis occurred in 83 of 208 patients (39.9%) vs. 49 of 156 patients with pain longer than 1 day (31.4%, p = 0.09). Sensitivity and specificity of MRI in patients with early abdominal pain were 97.6% (95% CI [91.6%-99.3%]) and 94.4% (95% CI [88.9%-97.3%]), respectively, vs. 93.9% (95% CI [83.5%-97.9%]) and 97.2% (95% CI [92.1%-99.4%]), respectively, in patients with pain longer than 1 day in duration (p = 0.36 and p = 0.35 for sensitivity and specificity, respectively). CONCLUSIONS: First-line MRI is accurate for acute appendicitis in children presenting with early abdominal pain, and may be appropriate as the initial examination in children. Further prospective studies are needed to compare MRI vs. ultrasound as the initial examination in children with early abdominal pain to help further delineate a diagnostic imaging algorithm.

Diagnostic performance of contrast-enhanced MR for acute appendicitis and alternative causes of abdominal pain in children.

OBJECTIVE: Unenhanced MRI has emerged as a useful tool for diagnosing pediatric acute appendicitis. The use of contrast-enhanced MRI for diagnosing pediatric appendicitis has not been documented. The purpose of this study is to examine the diagnostic performance of contrast-enhanced MRI for acute appendicitis and alternative entities in the pediatric population presenting with acute abdominal pain. MATERIALS AND METHODS: A retrospective review was conducted of 364 consecutive pediatric patients undergoing contrast-enhanced MRI for the evaluation of possible appendicitis at a single institution between November 2012 and September 2013. RESULTS: There were 132 cases of pathologically confirmed appendicitis out of 364 pediatric patients (36.3%) included in the study. Overall sensitivity and specificity were 96.2% (95% CI [91.4-98.4%]) and 95.7% (95% CI [92.3-97.6%]), respectively. Positive predictive value and negative predictive value were 92.7% (95% CI [86.6-96.3%]) and 97.8% (95% CI [94.7-99.1%]), respectively. The appendix was visualized in 243 cases (66.8%). Imaging confirmed alternative diagnoses in 75 patients, including most commonly colitis, enteritis or terminal ileitis (n = 25, 6.9%), adnexal cysts (n = 25, 6.9%) and mesenteric adenitis (n = 7, 1.9%). CONCLUSION: Contrast-enhanced MRI is capable of accurately diagnosing acute appendicitis while detecting many alternative entities of abdominal pain, and it allows good visualization of the appendix. Further evaluation is needed to determine whether contrast-enhanced MRI provides an advantage over non-enhanced MRI for imaging evaluation of acute abdominal pain in the pediatric population.

A prospective randomized comparison of traditional laparoendoscopic single-site surgery with needlescopic-assisted laparoscopic nephrectomy in the porcine model.

BACKGROUND AND PURPOSE: Laparoendoscopic single-site (LESS) surgery produces virtually no scar but is technically challenging because of the loss of triangulation. The objective of this study is to compare classic transumbilical LESS nephrectomy with needlescopic-assisted laparoscopy (NAL) surgery. In doing so, we evaluated whether the addition of a single 2-mm subcostal port could restore triangulation while not jeopardizing recovery or cosmetic outcome in the porcine model. MATERIALS AND METHODS: Ten female farm pigs were randomized to laparoscopic nephrectomy with either LESS or NAL. In LESS, a TriPort was placed through a single 2.5-cm umbilical incision. In NAL, 5- and 10-mm ports were placed in the umbilicus and a 2-mm port was placed in the midclavicular line. Preoperative, perioperative, and postoperative parameters were compared. Variables were analyzed with the Wilcoxon signed-rank test and two-tailed Fisher exact test. Cosmesis was evaluated objectively using the Vancouver Scar Scale and subjectively by a blinded dermatologist. A cost analysis was performed. RESULTS: Estimated blood loss was minimal in both groups (28.8 mL in LESS and 9.4 mL in NAL). Operative time was significantly shorter in NAL (103 vs 150 min; P<0.001). There was no difference in complications (2 vs 1; P=0.500), objective cosmesis (3.9 vs 3.8; P>0.2), or subjective cosmesis (2 vs 3; P=0.500). The NAL protocol had significantly lower disposable equipment costs ($363 vs $1696). CONCLUSIONS: The addition of a 2-mm subcostal port and the restoration of triangulation in the NAL protocol enable shorter operative times, increased surgeon comfort, improved technical ease, and lower costs while maintaining the scarless cosmesis of the traditional LESS protocol.

Ocular complications after open and hand-assisted laparoscopic donor nephrectomy.

OBJECTIVES: To compare the incidence of ocular complications (OC) and corneal abrasion (CA) after hand-assisted laparoscopic donor nephrectomy (HALDN) and open donor nephrectomy (ODN). METHODS: A retrospective review was conducted of 241 consecutive patients (141 HALDN and 100 ODN) over a 9-year period. OC were strictly defined as ocular complaints requiring any treatment or ophthalmologic consultation. Chi-square tests were used to compare the incidence of OC and CA by type of surgery. RESULTS: OC were observed in 9 HALDN patients (6.4%) and no ODN patients (0%; P = .01). All OC in HALDN patients involved the dependent eye (P <.001). CA occurred in 2 HALDN patients (1.4%) compared with no ODN patients (0%; P = .23). HALDN patients had significantly higher net fluid intake than the ODN patients (P <.01). CONCLUSIONS: The increased OC and CA seen in HALDN patients may result from the increased fluid intake, flank positioning, and potential increased venous compression resulting from the effects of the pneumoperitoneum. The fact that the dependent eye was involved in all patients suggests conjunctival edema as a potential common pathway. The high frequency of OC suggests the importance of techniques to minimize OC after HALDN.

Effect of low dose radiation computerized tomography protocols on distal ureteral calculus detection.

PURPOSE: Unenhanced multidetector computerized tomography is the imaging modality of choice for urinary calculi but exposes patients to substantial radiation doses with a subsequent risk of radiation induced secondary malignancy. We compared ultra low dose and conventional computerized tomography protocols for detecting distal ureteral calculi in a cadaveric model. MATERIALS AND METHODS: A total of 85 calcium oxalate stones 3 to 7 mm long were prospectively placed in 14 human cadaveric distal ureters in 56 random configurations. The intact kidneys, ureters and bladders were placed in a human cadaveric vehicle and computerized tomography was performed at 140, 100, 60, 30, 15 and 7.5 mA seconds while keeping other imaging parameters constant. Images were independently reviewed in random order by 2 blinded radiologists to determine the sensitivity and specificity of each mA second setting. RESULTS: Overall sensitivity and specificity were 98% and 83%, respectively. Imaging using 140, 100, 60, 30, 15 and 7.5 mA second settings resulted in 98%, 97%, 97%, 96%, 98% and 97% sensitivity, and 83%, 83%, 83%, 86%, 80% and 84% specificity, respectively. Interobserver agreement was excellent (kappa >0.87). There was no significant difference in sensitivity or specificity at any mA second settings. All false-negative results were noted for 3 mm calculi at a similar frequency at each mA second setting. CONCLUSIONS: Ultra low dose computerized tomography protocols detected distal ureteral calculi in a fashion similar to that of conventional computerized tomography protocols in a cadaveric model. These protocols may decrease the radiation dose up to 95%, reducing the risk of secondary malignancies.