Hinman Syndrome: A Rare Entity With Neurogenic Bladder-Like Symptoms.

Hinman syndrome, as is the case with many other rare conditions, is a disease very commonly under-considered or missed in the diagnosis of patients with the presenting symptoms. Clinical and radiographic manifestations of the condition are easily confused with neurogenic bladder without proper history collection and neurological examination. Patients typically present with symptoms including enuresis, urinary retention, reoccurring urinary tract infections, and encopresis. Imaging often shows hydroureteronephrosis and marked bladder wall thickening. While these signs are characteristic of neurogenic bladder, routine neurologic examinations and MRIs of patients with Hinman syndrome often show normal results, and their condition is currently thought to be an acquired behavioral and psychological disorder, often associated with abnormal family dynamics. We present the case of a 14-year-old boy, who presented to the emergency department with nausea, bilateral flank pain, and urinary retention. The patient had an over seven-year history of recurrent urinary tract infections (UTI) and intermittent encopresis and followed up with different providers. Due to the patient's extensive history and the failure of previous treatments, he was evaluated for causes of neurogenic bladder, but the MRI of the lumbar spine was normal. Fluoroscopic voiding cystourethrogram (VCUG) was ordered and demonstrated abnormal and trabeculated contour of the urinary bladder with bilateral vesicoureteral reflux consistent with the diagnosis of Hinman syndrome.

Splenogonadal fusion presenting as a testicular mass, a case report and literature review.

Splenogonadal fusion is a rare, frequently misdiagnosed, congenital anomaly in which the splenic tissue is abnormally attached to the gonadal or mesonephric remnants. It is commonly found as an incidental finding at autopsy, during orchiopexy or hernia repair. However, it can present as a testicular mass or as an acute scrotal pathology such as testicular torsion or epididymoorchitis. It poses as a diagnostic challenge preoperatively and often leads to unnecessary orchiectomy. We present a case of a 15-year-old male who presented with a long-standing left testicular mass thought to be a testicular tumor. Resection of the lesion along with partial left orchiectomy was done and histopathologic evaluation revealed splenogonadal fusion.

Ultrasound findings of distal umbilical artery calcification: A case report.

Umbilical artery calcification is a rare finding with only a small number of cases reported on radiographs. To date, no cases have been described on ultrasound. Reported cases were limited to the horizontal segment of the medial umbilical ligament. This case report presents a unique case of ultrasound findings of bilateral umbilical artery calcification within the vertical (distal) segment of the medial umbilical ligament.

A case report of an adolescent with ligase-4 deficiency and the potential dangers of ionizing radiation in this rare patient population.

DNA ligase IV deficiency is a rare disorder characterized by mutations in the LIG4 gene. Mutations in this gene cause a wide array of phenotypes, many of which are fatal early in life. We present an adolescent patient with heterozygous LIG4 mutations and the T-B-NK+ DNA ligase IV phenotype. Pelvic ultrasound and magnetic resonance imaging was completed to assess the patient's amenorrhea and delayed puberty, which demonstrated an atrophic cervix, distal vagina, and uterus without direct visualization of the ovaries. Early diagnosis of DNA ligase IV deficiency is important to minimize exposure to ionizing radiation from radiologic studies and preferentially utilize imaging studies that do not require ionizing radiation, such as ultrasonography and magnetic resonance imaging.

Intussusception secondary to signet ring cell adenocarcinoma in adolescent.

Despite the overall decrease in incidence and mortality rates for older adults, colorectal cancer in young adults is increasing. We present a case of a 15-year-old male who presented with 1.5 weeks of intermittent, sharp, severe right-sided abdominal pain. Abdominal radiograph demonstrated an air-fluid level within the right hemiabdomen. Computed tomography demonstrated marked wall thickening and a mass at the junction of the ascending colon and hepatic flexure causing obliteration of the lumen with a fluid-filled, dilated ascending colon. Follow-up ultrasonography demonstrated a 5.9 × 3.9 cm targetoid lesion in the right upper quadrant concerning for intussusception. Contrast enema revealed failure of contrast filling beyond the hepatic flexure due to a lobulated central filling defect surrounded by a claw-like contrast extension. Pathology of the polypoid lesion revealed poorly differentiated signet ring cell adenocarcinoma of the colon at the hepatic flexure. Despite its rarity, this case elucidates the need to consider colorectal carcinoma in adolescent and young adult patients who present with recurrent abdominal signs and symptoms.

Pediatric renal pseudotumour.

We present a case of 6-year-old female with history of respiratory distress who went into respiratory failure requiring intubation. Patient was subsequently found to be in hypertensive crisis with hyponatremic hypochloremic metabolic acidosis and acute kidney injury. Renal ultrasound was performed to find the cause of hypertension. The ultrasound study demonstrated lobulated isoechoic to hyper echoic mass-like lesion in the middle and lower pole of the right kidney with increased vascularity on Color Doppler examination. The renal mass was finally diagnosed as a pseudotumour, representing hypertrophied portion of the spared normal renal parenchyma in otherwise atrophic right kidney. Diagnosis was made using a combination of US, MRI, DMSA and CT angiography thus avoiding unnecessary surgical intervention.

Determining the impact of body mass index on ultrasound accuracy for diagnosing appendicitis: Is it less useful in obese children?

BACKGROUND: Ultrasonography (US) is the preferred imaging for suspected pediatric appendicitis. We hypothesize that children with elevated Body-Mass-Index-for-age percentile (BMIP) may be more likely to have an inaccurate or equivocal (IE) US. METHODS: After IRB approval, a four-year review was performed on pediatric patients evaluated for appendicitis by US. The CDC BMIP Calculator was used. IE subgroups were analyzed together for comparison against the accurate group. RESULTS: 1059 patients were included: median age 11.3 years (IQR: 8.2, 14.6), 506 (47.8%) males. Median BMIP was 65.9 (IQR: 33.9, 89.6). US accurately diagnosed 857 (80.9%), incorrectly diagnosed 76 (7.2%), 126 (11.9%) were equivocal. Overall sensitivity was 0.85, specificity 0.96, PPV 0.93 and NPV 0.91. Obese children (BMIP ≥95%), had higher odds of IE US (OR: 1.86, 95% CI: 1.28, 2.70; p = 0.001). When analyzed by sex, risk increased in obese males (OR: 2.55, 95% CI:1.53, 4.24; p = 0.0003) but normalized in obese females (OR: 1.30, 95% CI:0.74, 2.28; p = 0.35). CONCLUSIONS: An elevated BMIP may increase difficulty in visualizing the appendix, resulting in inaccurate or equivocal findings. This risk is seen specifically in obese males. If US findings do not correlate with clinical assessment in obese children with abdominal pain, further evaluation may be warranted.

Corrigendum to "Lack of restricted diffusion in MR imaging as a potential differentiating tool of reactive lymph node in cat scratch disease from lymphoma or abscess" [Radiology Case Reports 15 (2020) 1180-1183].

[This corrects the article DOI: 10.1016/j.radcr.2020.05.017.].

Lack of restricted diffusion in MR imaging as a potential differentiating tool of reactive lymph node in cat scratch disease from lymphoma or abscess.

Cat scratch disease is an infection caused by Bartonella Henselae with characteristic presentation of lymphadenopathy. Despite self-limited nature of the disease in most cases, it accounts for many lymph node biopsies performed since its imaging features can mimic lymphoma in appearance, thus requiring additional invasive procedures. Lack of restricted diffusion may be helpful in distinguishing imaging feature of reactive lymph nodes with caseating necrosis seen in cat scratch disease from lymphoma or abscess. We present a case of 12-year-old female, who presented with an arm swelling, MRI of which demonstrated absence of restricted diffusion in the subcutaneous enlarged lymph nodes. She then underwent excisional biopsy with final diagnosis of reactive lymph node and cat scratch disease based on biopsy results and immunoglobulin titer.

Incidental finding of renal pseudoaneurysm in child with tuberous sclerosis presenting with atypical clinical symptoms.

Tuberous sclerosis (TS) is a complex medical disorder with multisystemic clinical manifestations. Although the renal manifestations of this disease are well researched, the complexities of clinical diagnosis are raised significantly in patients with cognitive impairments, particularly in the pediatric population. We present a case of a 12-year-old male with intellectual disabilities and renal angiomyolipomas associated with his TS complex presenting with subtle cognitive and behavioral changes leading to the eventual diagnosis of a renal pseudoaneurysm. The purpose of this case report is to highlight the subtleties of diagnosis and management of patients with TS and cognitive impairments and maintaining a high clinical index of suspicion for life threatening complications when presenting symptoms are nonspecific. This case also demonstrates the importance of obtaining a thorough clinical history from parents and caregivers of these children and educating them on significance of recognizing changing behavior patterns. The healthcare responsibility for diagnosis and management must be shared by all levels of personnel that participate in the care to allow for improved morbidity, mortality, and quality of lives for these patients.

Herlyn-Werner-Wunderlich syndrome: A rare cause of abdominal pain and dyspareunia.

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital disease characterized by Mullerian duct anomalies. The characteristic triad of this syndrome includes didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis (hence, also known as Obstructed Hemivagina and Ipsilateral Renal Agenesis or OHVIRA syndrome). The clinical presentation of HWW is nonspecific and patients may present with abdominal/pelvic pain, dyspareunia, or dysmenorrhea. However, the imaging findings of HWW are characteristic and discussed in this case report of a 16-year-old female who presented with abdominal pain and dyspareunia.

Pediatric Ovarian Torsion: Spectrum of Imaging Findings.

The accurate diagnosis of ovarian torsion is imperative, as loss of the ovary can have long-term consequences in terms of fertility. However, a nonspecific clinical presentation in conjunction with a highly variable imaging appearance makes the diagnosis of ovarian torsion challenging. This is complicated even further in the pediatric population, as these patients cannot always articulate their symptoms or provide an adequate medical history. Therefore, imaging has a critical role in the diagnosis of ovarian torsion in pediatric patients. Common imaging findings of ovarian torsion in the prepubescent and adolescent populations include asymmetric enlargement of the ovary, peripheral location of ovarian follicles, and midline location of the ovary. A coexistent mass within the ovary may or may not be present. Antenatal torsion also can occur and may be discovered at routine or specific imaging of the fetus or postnatal imaging of the neonate. Imaging findings in the perinatal population that may suggest torsion include a cystic mass with a fluid-debris level and a complex, multiseptated mass. This article reviews ovarian torsion throughout the pediatric years-from the fetal period through adolescence. It reviews the clinical presentation and imaging findings of this abnormality while describing the relevant anatomy, embryologic features, and pathophysiology. Ovarian torsion may be variable in appearance owing to the age and degree of torsion, which is seen early as a large ovary with peripheral follicles and later, once necrosis has ensued, as a complex cystic mass. ©RSNA, 2017.

"Lying-Down" Adrenal Sign: There Are Exceptions to the Rule Among Fetuses and Neonates.

A linear-shaped or "lying-down" adrenal gland is a sign often seen with the absence of the kidney in the renal fossa due to renal agenesis, renal ectopia, or horseshoe kidney. It is theorized that the presence of the kidney in the normal location within the renal fossa is important for the formation of the normal triangular inverted V or Y adrenal shape. There are exceptions to this rule whereby a kidney is missing from the renal fossa, yet a normal adrenal shape is present. This series looked at 18 cases of an empty renal fossa in fetal, neonatal, and pediatric patients and recorded the shape of the adrenal gland. Nine cases (50%) appropriately showed the linear or lying-down adrenal gland; 6 (33%) showed an exception to the rule, with a normally shaped adrenal gland; and 3 (17%) showed a pseudo exception in which the adrenal gland was linear but blended with the diaphragmatic crus to simulate a triangular adrenal gland. The sonographic characteristics of the crus are different from those of the adrenal gland; thus, this pseudo exception can be avoided by careful inspection. Because the absence of the kidney is often a difficult diagnosis, the lying-down adrenal gland sign can be a helpful secondary sign for confirming that a kidney is absent or ectopic in position and not within the renal bed.