Normal Ultrasonography Research Articles

DIAGNOSTIC PERFORMANCE OF ULTRASONOGRAPHY FOR DETECTION OF ABRUPTION AND ITS CLINICAL CORRELATION AND MATERNAL AND FETAL OUTCOME

Placental abruption complicates about 1% of singleton pregnancies and is an important cause of perinatal mortality and morbidity. Though sensitivity and reliability of ultrasound are poor for detecting or excluding placental abruption, because of the advances in ultrasound resolution, imaging and interpretation, sensitivity of ultrasound is better than what was reported previously. To determine the diagnostic performance of Aim: Ultrasonography (USG) for the detection of placental abruption and whether sonographic results correlate with maternal and foetal management and outcome Sixty patients with clinical diagnosis of placental abruption we Materials and Methods: re studied in the Obstetrics and Gynaecology Department of Gujarat Adani Institute of Medical Sciences, Bhuj over a period of 6 months. These patients underwent ultrasonography for conrmation. Obstetric and neonatal outcome and sonographic results were compared and reviewed. Sonographic sensitivity and specicity and positive and negative predictive values were calculated. Incidence of abruption in present study was 3.05% (56 Results: patients out of 1834 total deliveries). Sensitivity of ultrasonography in the diagnosis of abruption was 57% while its specicity was 100% with a positive predictive value of 100% and a 14% negative predictive value. An 87.5% of patients(28 out of 32) with a positive USG nding of abruption had Intrauterine foetal Death (IUD)/still birth while 91.6% of patients (22 out of 24) with negative USG ndings of abruption gave birth to babies who required NICU admission. Sonography has a poor sensitivity for diagnosing placental abrupt Conclusion: ion, even though it has a high specicity and PPV. In a positive sonographic result, maternal morbidity and perinatal mortality are high which needs aggressive obstetric management as compared to the normal sonography. In case of a negative sonographic nding but a strong clinical suspicion of abruption if Obstetric intervention is made in due time, foetal as well as maternal outcome are better

Clinical manifestations, laboratory markers, and renal ultrasonographic examinations in 1-month to 12-year-old Iranian children with pyelonephritis: a six-year cross-sectional retrospective study

BackgroundUpper urinary tract infection (UTI) or pyelonephritis may increase the pathogenesis rate and risk of severe complications in children due to kidney atrophy.ObjectiveA set of clinical symptoms, laboratory markers, and ultrasound findings were assessed to achieve the early diagnosis and prognosis of pyelonephritis in hospitalized pediatrics.MethodsA cross-sectional study with 104 Iranian children (95 girls and 9 boys) aged 1 month to 12 years with acute pyelonephritis during 2012–2018 was conducted. The ultrasound examination of kidneys and urinary tract during hospitalization, the incidence of clinical symptoms, and laboratory markers in blood and urine were monitored to identify the best predictive factors of early diagnosis of this bacterial infection.ResultsThree-fourth of the patients had one of the four clinical symptoms of abdominal pain, constipation, dysuria, and vomiting, while others were asymptomatic. A much frequency of pyuria (88.46%), Escherichia coli in urine (92.31%), leukocytosis (81.73%), and high ESR (> 10 mm/h, 92.30%) and CRP (> 10 mg/L, 82.82%) was observed. The kidney and urinary tract ultrasonography only in 32.7% of children revealed findings in favor of pyelonephritis (cystitis, ureteral stones, and hydronephrosis).ConclusionThere was a high frequency of clinical signs and laboratory markers associated with pyelonephritis. Ultrasound alone was not an efficient tool to track febrile UTI as most patients presented normal sonography.

Outcome of nonurinary surgical malformations predicted by fetal abdominal signs on prenatal ultrasound

Aim: The aim of our study was to study the outcome of “nonurinary” surgical malformations predicted by fetal abdominal signs on prenatal ultrasound (US). Methods: This prospective observational study was done over a 3-year period. Results: Out of 66 cases, 15 different malformations were detected. The accuracy of prenatal US was 83.3%. There were four still births, two medical termination of pregnancy, and sixty live births (91%). Postnatal surgery was necessary in 35 neonates (62.5%) with postoperative survival of 71.4%. Sixteen neonates (26.7%), 7 with normal postnatal US and 9 asymptomatic lesions, were managed conservatively. Overall 1-year survival rate of fetuses was 62.1%. The most common malformations confirmed postnatally were duodenal atresia (n = 14, 23.3%), followed by gastroschisis (n = 9; 15%), esophageal atresia (n = 8; 13.3%), meconium peritonitis (n = 4; 6.7%), and ovarian cyst (n = 4; 6.7%). Prematurity (n = 34; 56.7%) and low birth weight (n = 44; 66.7%) had no significant effect on survival due to in utero transfer to tertiary care. Salient factors adversely affecting the survival were: (1) type of anomaly-omphalocele major and fetal ascites (FA) had no survivors, (2) postoperative complications, and (3) associated cardiac anomalies. It was also notable that, among fetal signs of meconium peritonitis, isolated intra-abdominal calcification had a good prognosis with 75% survival, whereas FA had no survivors. Conclusions: Results of this study will be helpful in realistic prognostication and postnatal management of these anomalies in the appropriate hospital setting.

Sonographic measurements for spleen size in healthy Saudi children and correlation with body parameters.

BACKGROUND: Ultrasound is commonly the first-line imaging modality for assessing the visceral organ dimensions without any risk of radiation. An abnormal size of the spleen may indicate disease, but the evaluation is challenging because of the normal size changes with age. In addition, published normal value charts for children may vary by population and methods.OBJECTIVES: Determine normal growth curves for splenic length in relationship to sex, age, body weight, height, body mass index, and body surface area of healthy children in Saudi Arabia.DESIGN: A retrospective review of ultrasonography imagesSETTING: Tertiary referral hospitalSUBJECTS AND METHODS: We included all normal ultrasonography exams of splenic length (from full-term neonates to 16 years of age) performed between 2003 and 2018. The greatest longitudinal distance of the spleen from the dome to the tip (splenic length) measured at the hilum in the coronal plane was obtained by ultrasonography.MAIN OUTCOME MEASURES: The relationship between the longitudinal length of the spleen and sex, age, height, weight, body mass index, and body surface area.SAMPLE SIZE: 1028 healthy childrenRESULTS: Height had the most significant correlation with the length of the spleen (P<.001). There was a consistent difference in splenic length according to sex. The spleen was longer in males than in females in children older than 2 years. In terms of splenic growth according to age, our data showed a statistically significant correlation between the ages of 0-24 months and 2-16 years. There was a significant difference between cases from Saudi Arabia, the United States (P=.023) and India (P=.045).CONCLUSION: We developed scatter plots between splenic lengths and body parameters among Saudi children for children from 0 day to 24 months and children from 2 years to 16 years for reference.LIMITATION: Retrospective study. Ultrasound techniques in patient positioning, and cursor placement differed from other studies, which can affect measurements of splenic length.CONFLICTS OF INTEREST: None.

Upper-Limb Entheses in Healthy Children: A Comprehensive Ultrasound Study.

Interest in ultrasound (US) as a diagnostic tool in spondyloarthritis is growing. Although the literature has provided detailed description of normal lower-limb entheses for adults and children, validated normal US findings of the pediatric upper-limb (UL) entheses are lacking. The aim of this study was to describe the sonographic characteristics of the UL entheses in healthy children to provide a basis for assessing pathologic findings in children with rheumatic diseases. This is an observational, cross-sectional study. The recruited healthy children were grouped according to age: group 1, 3-9 years (n = 22); group 2, 10 to 13 years (n = 12); and group 3, 14 to 18 years (n = 9). The following UL entheses were examined: (1) supraspinatus, (2) common flexor, (3) common extensor, and (4) triceps. Ultrasound examination was performed in B mode for morphology and structural abnormalities, and power Doppler was applied to detect vascularization within the enthesis and cartilage sites. Forty-three children with a median age of 9 years (6-13 years) were included; 55% were boys. A total of 344 entheses were evaluated; all of them presented a homogeneous hyperechoic fibrillar pattern. Tendon insertion thickness at the bone attachment site increased with age and was greater in males. No signs of chronic enthesopathy were detected. Doppler signal was observed in children from groups 1 and 2, but not in any of the patients in group 3. In group 1, intraentheses signal was detected in the common extensor tendon insertion as an isolated signal (n = 2 patients), whereas intracartilage signal was detected in the supraspinatus (n = 1 patient) and the triceps (n = 1 patient). In group 2, intracartilage signal was detected in the common extensor (n = 2 patients) and the common flexor (n = 2 patients). Interobserver agreement for the entheses' thickness was as follows: intracorrelation coefficient class (ICC) of 0.86 (95% confidence interval [CI], 0.84-0.92) for the supraspinatus enthesis, ICC of 0.83 (95% CI, 0.83-0.90) for the common extensor enthesis, ICC of 0.96 (95% CI, 0.91-0.97) for the common flexor enthesis, and ICC of 0.94 (95% CI, 0.89-0.95) for the triceps enthesis. The κ index was 0.93 for the detection of the power Doppler signal. Intraobserver agreement was good for all evaluated entheses: ICC of 0.85 (95% CI, 0.83-0.91) for supraspinatus, ICC of 0.86 (95% CI, 0.84-0.91) for common extensor entheses, ICC of 0.89 (95% CI, 0.86-0.93) for common flexor entheses, and ICC of 0.96 (95% CI, 0.90-0.97) for evaluation of triceps entheses. The κ index was 0.91 for the detection of Doppler signal. The current study provides a comprehensive description of the structural appearance and vascularization of UL entheses on healthy children. This information can be useful as reference for interpretation of pathological findings in children with musculoskeletal diseases in clinical practice.

Ureteral endometriosis – Our experience

Ureteral endometriosis is a rare disease affecting women of childbearing age, which presents with nonspecific symptoms and may lead to severe morbidity. The ureters can be involved either extrinsically and/or intrinsically by endometriosis. In this study we have retrospectively assessed the patients presenting to us with ureteral endometriosis. Between July 2009 and December 2019, (17) women of childbearing age presented to the urological services of the hospital with complaints of lower abdominal pain, dysmenorrhea, dysuria and a range of other symptoms. Pain in lower abdomen was the most common symptom and was cyclical in the majority of the patients. Only 2 patients had undergone surgery (diagnostic laparoscopy) previously for pain in abdomen and normal ultrasonography. Ureteric endometriosis is rare but a cause of severe morbidity in those that have deep infiltrating endometriosis. Can lead to ureteric obstruction and at times loss of function. An index of suspicion in women in child bearing age is important. Hormonal manipulation along with surgical intervention is beneficial in most patients.

Nephrocalcinosis and Renal Dysfunction in Pediatric Intestinal Failure

Outcomes of pediatric intestinal failure (PIF) have improved recently, with other comorbidities, such as increased echogenicity/nephrocalcinosis on ultrasound (US) in long-term survivors now evident. We evaluated the significance of nephrocalcinosis over time in PIF and its impact on renal function. Retrospective analysis on a cohort of PIF patients was performed. Presence of nephrocalcinosis and/or increased renal echogenicity (identified on US), estimated glomerular filtration rate (eGFR; ml · min · 1.73 m2), renal tubular function, PN volume (ml · kg · day) and PN exposure time (hours/day) were reviewed annually over a follow-up period of 2 years. Outcomes in the nephrocalcinosis versus normal US groups were compared. Forty patients (28 boys, median age 2.7 years) were followed for 2 years. Fifteen (38%) had either increased echogenicity or nephrocalcinosis (group 1) at initial US. US were normal in the remaining 25 (62%) on initial assessment (group 2). eGFR did not differ between group 1 and group 2 at baseline (118 vs 133, P = 0.51) and year 2 (130 vs 131, P = 1.00). The percentage of patients with abnormal markers of tubular function was similar in both groups at year 2 (high urine calcium: creatinine 33 versus 30, P = 0.83; high urine calcium: citrate 39 versus 42, P = 0.87; low urine citrate: creatinine 15 versus 17, P = 1.00; high urine oxalate: creatinine 39 versus 25, P = 0.77). A large proportion of PIF patients with a history of parenteral nutrition (PN) exposure have nephrocalcinosis and/or increased echogenicity on US. Over a 2-year follow-up period, however, these abnormalities had no impact on eGFR or renal tubular function.

A normal screening ultrasound does not provide complete reassurance in infants at risk of hip dysplasia; further follow-up is required.

Screening for developmental hip dysplasia (DDH) continues to evolve with the use of ultrasound (US) in either selective or universal screening methods. The possibility of delayed evidence of DDH, and thus the need for radiographic follow-up at a later stage of development have been suggested by some authors. The aim of this review was to evaluate the number of patients in our hospital network with a normal screening US at 6weeks with evidence of DDH at the time of radiographic review at 6months. Secondary aim; to determine the outcomes for these patients. A retrospective review was done to infants undergoing DDH ultrasound screening between January and December 2015. Initial US and radiographs at 6months were reviewed. Patients with normal screening US who had subsequent radiographs were included for analysis. In total, there were 829 patients included for analysis. Sixty-three patients (8%) had evidence of DDH at 6months, representing 34% of all DDH diagnoses for the study period. Five of the 63 patients were lost to follow-up. The remaining 58 babies were treated in Boston bracing. Four patients with evidence of persistent DDH were referred for tertiary review. The osteotomy rate in the radiograph diagnosed group was 2%, versus 6% and 3% in the unstable and US diagnosed groups, respectively. Eight percent of patients with a normal screening US had evidence of DDH at time of radiograph at 6months, reflecting 34% of all our DDH cases for the year. Based on these findings, patients in our hospital network undergo radiographic evaluation at 6months even if the initial screening US is normal.

Thyroid dysgenesis as a risk factor for patients with poorly controlled congenital hypothyroidism

Objective Our aim was to search for the risk factors of cases with poor controlled congenital hypothyroidism (CH) in correspondence to their clinical aspect. Background CH is largely caused by thyroid gland dysgenesis, and there is lack of a standardized approach for diagnosis, follow-up, education, and genetic counseling of patients with CH. Patients and methods Genetic counseling and thyroid ultrasonography (US) were done. Blood samples were collected from 56 children (30 girls and 26 boys) with CH, diagnosed on the basis of neonatal screening program done by Egyptian Ministry of Health and Population, Central Health Laboratories, Hormones and Tumour Marker Department. All patients confirmed biochemically with low free thyroxine and high thyroid-stimulating hormone levels were enrolled as cases (group I), along with 56 apparently healthy individuals as controls (group II), who attended the Genetic laboratory of Genetic and Endocrinology Unit, Paediatric Department, Faculty of Medicine, Menoufia University, Egypt. Results A total of 16 (28.6%) patients had poor response to treatment. They were on a high dose of L-thyroxin (100 μg/day or more), with their US findings ranging from normal thyroid sonar in six patients, hypoplastic gland in four patients, and thyroid agenesis in six patients. All healthy control children showed normal US findings. Conclusion US is the first-choice imaging method in revealing the etiology of CH. Family counseling is a key component for enhancing the care of patients with CH.

FRI0599 USEFUL II: DERIVATION OF THE LUPUS ARTHRITIS AND MUSCULOSKELETAL DISEASE ACTIVITY SCORE (LAMDA) USING DATA FROM A MULTICENTRE LONGITUDINAL STUDY

Background:Musculoskeletal (MSK) disease is the commonest manifestation of SLE. We showed that the MSK components of the BILAG index and SLEDAI have limited sensitivity, specificity and responsiveness compared to ultrasound (US) synovitis. The USEFUL study evaluated response to glucocorticoids in SLE patients with inflammatory pain.Objectives:To develop a disease activity tool for lupus MSK manifestations that is continuous, responsive, sensitive, and correlates with US-synovitisMethods:133 patients who received depomedrone 120mg IM were assessed at 0, 2 and 6 weeks for 66/68 swollen and tender joint counts, BILAG2004 index, SLEDAI-2K, physician global and MSK-VAS, inflammatory markers, patient pain and disease activity-VAS. Total US score (OMERACT-EULAR) in the hands and wrists was calculated blinded to patient and clinical assessor. Patients reported overall response using a Likert scale.The LAMDA was developed by modelling a core set of clinical variables against total US score using penalized (Lasso) regression. Responsiveness was compared between LAMDA and other variables at week 6 using effect sizes. Minimum clinically important difference (MCID) was explored using the SEM and minimal disease activity threshold using ROC.Results:The variables selected for the LAMDA score were swollen joint count, patient MSK pain VAS, physician MSK disease activity VAS and ESR. A continuous score was derived. This had a theoretical range from 0 to 26.5 based on maximum ESR of 100. The highest value observed in USEFUL was 15. LAMDA was significantly higher in patients with active US (mean (SD) 5.71 (2.67), n=78) compared to patients with normal US (3.27 (1.77), n=55; difference (95% CI) -2.45 (-3.26, -1.63), t=-5.93, p&lt;0.001). This difference remained significant in patients with no swollen joints (difference (95% CI) -0.71 (-1.40, -0.02), t=-2.06, p=0.044).Effect size was greater for the LAMDA (0.37) than the BILAG-MSK (0.31), SLEDAI-MSK (0.27) and total US score (0.33). In patients with active US at baseline, LAMDA’s effect size was 0.42.The MCID was 0.71 and correlated with patient-reported change in pain. A threshold for minimal disease activity of 3.23 optimized sensitivity (0.77 (0.65, 0.89)) and specificity (0.80 (0.68, 0.92)) against US score &gt;0.Conclusion:The LAMDA score is a novel continuous disease activity instrument for MSK manifestations of SLE derived from variables familiar to rheumatologists. The LAMDA score is sensitive to imaging detected synovitis without swelling and more responsive than other instruments. . LAMDA may improve the ability of clinicians to accurately determine therapeutic efficacy in clinical trials and practice. Future work will validate the LAMDA score in independent cohorts and randomized trials.Acknowledgements:This project was funded by Lupus UKDisclosure of Interests:Khaled Mahmoud: None declared, Ahmed Zayat: None declared, Md Yuzaiful Md Yusof: None declared, Coziana Ciurtin Grant/research support from: Pfizer, Consultant of: Roche, Modern Biosciences, Chee-Seng Yee: None declared, David Isenberg Consultant of: Study Investigator and Consultant to Genentech, Lee-Suan Teh: None declared, Katherine Dutton: None declared, David d’cruz Grant/research support from: GlaxoSmithKline, Nora Ng: None declared, Philip G Conaghan Consultant of: AbbVie, BMS, Eli Lilly, EMD Serono, Flexion Therapeutics, Galapagos, GSK, Novartis, Pfizer, Speakers bureau: AbbVie, Eli Lilly, Novartis, Pfizer, Paul Emery Grant/research support from: AbbVie, Bristol-Myers Squibb, Merck Sharp &amp; Dohme, Pfizer, Roche (all paid to employer), Consultant of: AbbVie (consultant, clinical trials, advisor), Bristol-Myers Squibb (consultant, clinical trials, advisor), Lilly (clinical trials, advisor), Merck Sharp &amp; Dohme (consultant, clinical trials, advisor), Novartis (consultant, clinical trials, advisor), Pfizer (consultant, clinical trials, advisor), Roche (consultant, clinical trials, advisor), Samsung (clinical trials, advisor), Sandoz (clinical trials, advisor), UCB (consultant, clinical trials, advisor), Christopher Edwards Grant/research support from: Abbvie, Biogen, Roche, Consultant of: Abbvie, Samsung, Speakers bureau: Abbvie, BMS, Biogen, Celgene, Fresenius, Gilead, Janssen, Lilly, Mundipharma, Pfizer, MSD, Novartis, Roche, Samsung, Sanofi, UCB, Elizabeth Hensor: None declared, Edward Vital Grant/research support from: AstraZeneca, Roche/Genentech, and Sandoz, Consultant of: AstraZeneca, GSK, Roche/Genentech, and Sandoz, Speakers bureau: Becton Dickinson and GSK

OR22-02 PTEN Hamartoma Tumor Syndrome in Pediatrics: Triggers for Evaluation and the Value of Surveillance

Context: PTEN Hamartoma Tumor Syndrome (PHTS) comprises a collection of rare clinical disorders characterized by germline mutations in the tumor suppressor gene PTEN. Current guidelines recommend screening for thyroid tumors beginning in pediatric age at the time of PHTS diagnosis; however, the benefit of early surveillance has not been well defined. Patients/Objective: We conducted a retrospective, single-site cohort investigation of patients followed at the Children’s Hospital of Philadelphia with diagnosis of PHTS between January 2003 - June 2019. In total, 81 patients under 18 years of age were identified. Clinical features, PTEN mutation codon, thyroid and gastrointestinal (GI) features were extracted from the electronic health record. The aim of the study is to assess genotype-phenotype, the incidence of thyroid and gastrointestinal disease, and to determine whether current recommendations for thyroid surveillance are improving outcomes. Results: The most common clinical feature at presentation was macrocephaly (85%) followed by impaired development (42%), skin/oral lesions (31%), and autistic spectrum disorder (27%). GI polyps were the presenting feature in 5 patients, with 14 of 81 patients ultimately diagnosed secondary to constipation (71%), rectal bleeding (64%), and/or abdominal pain (50%). All polyps were benign. A total of 58 of 81 patients underwent thyroid surveillance, with 30 patients (52%) found to have a nodule(s). Ultimately, 16 patients underwent thyroidectomy, with 31% (5/16) diagnosed with thyroid cancer. All thyroid cancer patients were greater than 10 years of age at diagnosis and all displayed low-invasive behavior (ATA low-risk). Of the patients < 10 years at the time of thyroid ultrasound (US) surveillance, 74% (14/19) had a normal US. The remaining five patients who underwent thyroid surgery all had benign histology. No genotype-phenotype relations were found; however, patients with identical mutations were found to have similar clinical features. Conclusions: Patients with macrocephaly associated with impaired development, skin/oral lesions, thyroid nodules and/or early onset GI polyps should undergo germline testing for PHTS. There does not appear to be a clinical advantage to initiating thyroid US surveillance prior to 10 years of age. Early detection may not improve outcome of thyroid cancer as the majority of thyroid cancers display low-invasive behavior. In PHTS patients with a normal physical exam, thyroid ultrasound surveillance can be delayed until after 10 years of age. Early onset GI polyps may be the presenting diagnosis of PHTS.

Advanced Ultrasound Screening for Temporomandibular Joint (TMJ) Internal Derangement.

Purpose To present an advanced ultrasound (US) technique and propose its use as a screening diagnostic tool for temporomandibular joint (TMJ) internal derangement. Materials and Methods The technique is based on maintaining the US probe parallel to the articular disc, rather than traditional axial and coronal views, with the position described relative to a clock face. Validation was achieved by direct comparison with magnetic resonance imaging (MRI). A total of 61 patients, with age ranging from 13 to 67 years, were prescreened for TMJ pain and internal derangement, underwent US imaging for screening, and MRI evaluation for final diagnosis. Results 29 of the 61 patients had disc pathology on MRI. US screening produced no false positive results and only 6 false negative results, corresponding to a sensitivity of 79% and specificity of 100%. Half of the false negative cases involved disc pathology with a medial component to the disc displacement. Conclusion US is both a sensitive and a specific screening tool for TMJ dysfunction when used by an appropriately trained operator, with the exception of medially displaced discs. If TMJ assessment is found to be abnormal, the patient should be referred for MRI, and any patient scheduled for surgery must have the diagnosis confirmed by MRI. If a component of medial disc displacement is suspected, MRI should be performed despite a normal screening US.

Ultrasound of Normal and Injured Ligaments and Retinacula of the Hand.

Clinical diagnosis of ligament and retinacular injuries of the hand may be challenging. Ultrasound (US) enables detailed high-resolution, dynamic, and real-time evaluation of these structures. This article is a comprehensive review of the intricate anatomy, optimal imaging technique, and normal US appearances of these ligaments and retinacula. The US features, pertinent biomechanics, clinical presentation, and differential diagnosis of injuries affecting the annular pulleys, differentiating from climber's finger; extensor hood including sagittal band and central slip rupture; proximal interphalangeal and metacarpophalangeal collateral ligaments including the Stener lesion and associated volar plate injury; and the anterior oblique or beak ligament of the trapeziometacarpal joint are reviewed. Emphasis is placed on optimal transducer positioning, value of dynamic US, injury severity, surgical indications, and other US findings with important management implications. US facilitates a timely accurate diagnosis of hand ligament and retinacular injury to ensure optimal patient management and minimize the risk of functional impairment of the hand.

P51 Serious pathology mimicking GCA: two cases of carcinoma tongue

Abstract Background Jaw and tongue pain with constitutional symptoms and raised inflammatory markers are considered pathognomonic ischemic features of giant cell arteritis (GCA). Temporal artery ultrasound (US) (or biopsy) available in GCA fast-track clinics (FTC) for a rapid assessment of patients with suspected GCA. Atypical presentation and negative US or biopsy require further workup to look for an alternative diagnosis. ENT pathology can occur as a mimic of GCA. Herein we discuss two clinical cases of squamous cell cancer tongue presenting with signs and symptoms that resemble GCA. Methods We have put together a case report. Results Case-1: A 58-year-old male with a recent presumed diagnosis of relapsing GCA was referred with worsening visual symptoms and right eye pain despite ongoing steroid treatment (60mg), for consideration of Tocilizumab (TCZ). His initial presentation was 5 months ago with a right parietal and retro-orbital headache and blurred vision. He had a normal eye examination. His C-reactive protein (CRP) was raised (24). He was started on prednisolone 40 mg for GCA with complete resolution of symptoms with normalised CRP within weeks. Several weeks later, symptoms reoccurred. CT brain, abdomen and pelvis was normal and temporal artery biopsy negative. His prednisolone was increased to 60 mg. 6 months later, his jaw and tongue pain worsened, and he was treated with pulsed methylprednisolone. Due to partial response to steroids, he was referred to consider TCZ. He had tender left TMJ with normal temporal artery US. Urgent MRI head and neck revealed a left posterior tongue mass with the histology confirmed poorly differentiated squamous cell carcinoma. He was managed with chemo and radiotherapy. Case-2: A 75 years old female, presented with right scalp pain, tongue pain, painful swallowing and chewing. Her blood investigations were normal except a raised ESR (48) and presumed GCA she was started on steroids (60 mg). she had initially good response but, within a few weeks, her symptoms returned. She was then referred to our FTC. Temporal artery US and biopsy were normal. MRI of the head and neck showed a large mass seen in the right half of the posterior tongue extending into the deep aspect of the anterior tongue. Histology confirmed poorly differentiated Squamous cell carcinoma. She was treated with a combination of chemotherapy and radiotherapy. Conclusion GCA mimics represent a major diagnostic dilemma. FTC helps to stratify the GCA from mimics. Careful evaluation of the history, examination as well as a temporal artery US helps to exclude GCA and aids prompt requesting of appropriate tests to find an alternative diagnosis such as tongue cancers as in our cases. We have now introduced negative weightage for consideration of alternative diagnoses in our GCA probability score. Disclosures A. Kayani None. A. Sebastian None. L. Borukhson None. M. Whitlock None. B. Dasgupta Consultancies; Roche, Sanofi. Grants/research support; Roche.

Voiding Cystourethrogram in Children With Unilateral Multicystic Dysplastic Kidney: Is It Still necessary?

To evaluate the value of the voiding cystourethrogram (VCUG) in children with multicystic dysplastic kidney (MCDK) who have a normal versus abnormal contralateral kidney and bladder ultrasound (US), and assess the risk of having vesicoureteral reflux (VUR) or urinary tract infection (UTI) based on the US results. A retrospective chart review including children with unilateral MCDK with postnatal US and VCUG available at our institution between January 2008 and September 2017 was performed. Analysis was done to find association between abnormal contralateral US and contralateral VUR and UTI. One hundred and fifty-six children were analyzed; 118(75.6%) patients had a normal contralateral kidney US, while 38(24.4%) had abnormal US. The rate of severe contralateral VUR (grade IV and V) was 2 (1.7%) and 5 (13.2%) in children with normal and abnormal contralateral US, respectively. The risk analysis demonstrated a significant association between severe VUR on the contralateral kidney and an abnormal contralateral US (odds ratio=7.73; 95%CI: 1.43-41.81; P=0.018) and no significant association with UTI (odds ratio=1.58; 95%CI: 0.50-4.94; P=0.435). Our data suggests, the rate of severe contralateral VUR in children with unilateral MCDK and normal contralateral kidney is low. VCUG should be considered for infants with proven MCKD and alterations on the contralateral kidney on US. Following patients with MCDK and normal contralateral kidney without the use of VCUG is a reasonable approach, unless there is development of signs and symptoms of recurrent UTI or deterioration of the renal function. We found that abnormal contralateral kidney US was associated with severe VUR.

Anhydramnios, but prenatally normal kidneys: renal tubular dysgenesis – patient with mutations in the renin-angiotensin system gene AGTR1

Abstract Background Prenatally detected oligo- or even anhydramnios may – beside other reasons – be indicative for a diminished or absent urine production. The resulting clinical picture is a “Potter sequence” with arthrogrypotic joint contractions, a flat face and most importantly pulmonary hypoplasia. In severe cases this pulmonary hypoplasia can be life-limiting irrespective of the underlying lesion. Case presentation Our patient initially presented with anhydramnios and normal kidneys at external ultrasonography after 31 weeks of pregnancy. Following spontaneous birth after 37 weeks of gestation, the baby boy was born with all the clinical signs of a “Potter-sequence” along with a severe pulmonary hypoplasia leading to insufficient oxygenation and ventilation. Despite all measures taken, the child died after 8 h of life. Beside life-limiting pulmonary hypoplasia postmortem examination again confirmed macroscopically normal kidneys, but microscopy showed compact and variable sized glomeruli, numerically reduced and immature tubules, a structurally altered renal vascular bed and an expanded medullar interstitium. Immunohistochemical studies revealed the absence of proximal convoluted tubules, shortened proximal straight tubules beside an immaturity and dysmorphogenesis of the other segments of the nephron, and thus proved renal tubular dysgenesis (RTD) as the underlying disease. A homozygous mutation c.377G&gt;C (p.Arg126Pro) in exon 2 of the AGTR1 gene was found, leading to the exchange of a highly conserved arginine to proline. This mutation has not been reported in public databases so far. As expected, both consanguineous parents were heterozygous for this mutation. Conclusion RTD has to be considered in an anuric fetus with apparently normal renal sonography in order to allow adequate prenatal counseling and – if indicated – palliative postnatal care.

Evaluating the effectiveness of the national hip dysplasia early diagnosis and treatment program

A significant decrease in late diagnosis and requirement for surgical treatment have been observed due to the implementation of the National Developmental Hip Dysplasia (DDH) Early Diagnosis and Treatment Program, which has been in existence for about 10 years. However, patients with delayed diagnosis are still admitted to our clinic for treatment. In this study, we aimed to evaluate the effectiveness of this program and reveal its deficiencies. Patients and methods: Sixty-one patients diagnosed or treated for ddh later than six months of life were included in the study. Patients born between 2011 and 2014 (Group 1) and between 2015 and 2018, during which time the early screening program was performed intensively (Group 2), were compared. The patients’ risk factors, whether a hip ultrasound (US) was performed, and the reasons for the delay in diagnosis and treatment were questioned retrospectively. The number of DDH patients in Group 2 decreased by approximately 3 times (45/16) (p = 0.0009). Although 37 (61%) patients had a hip US assessment within the first 6 months, their treatment was delayed, and normal USs were reported for 20 (33%) of them. Hip US was not performed in 24 (39%) patients within the first 6 months. The number of late-diagnosed and -treated babies with DDH decreased significantly in recent years, but this is not sufficient. Preventing late diagnoses should be the main goal. This study detected families’ negligence and lack of information, family physicians’ non-compliance with the program, and improper US assessment as the confounding reasons.

Role of pre IVF hysteroscopy in women with unexplained infertility for detecting unsuspected intrauterine lesions and effect on pregnancy outcome

Objective: The relevance of routine hysteroscopy before IVF is debatable and there is no consensus on the effectiveness of routine pre IVF hysteroscopy in improving pregnancy outcome. The purpose of this study is to assess the role of pre IVF hysteroscopy in women with unexplained infertility in detecting unsuspected intrauterine lesions and its effect on pregnancy outcome. Materials and Methods: This was retrospective observational study conducted at Medicover Fertility Center, New Delhi, India. In the study group (n = 60), women with unexplained infertility of more than three years with normal transvaginal ultrasonography were included, where hysteroscopy was done in post-menstrual period, 1–2 months before starting IVF treatment. In the control group (n = 60), pre IVF hysteroscopy was not done. Results: The study and control group did not differ for age, body mass index, duration and type of infertility. There was a high prevalence (31.6%) of unsuspected intrauterine abnormalities in women with unexplained infertility with normal transvaginal ultrasound findings. The clinical pregnancy rate was 48.3% (29/60) in study group and 36.7% (22/60) in control group. The clinical pregnancy rate (CPR) was observationally higher in women with pre IVF hysteroscopy, but the difference was not statistically significant (P value = 0.193). Conclusion: There was high prevalence rate of unsuspected intrauterine lesions in women with unexplained infertility. Clinical pregnancy rates were not significantly higher in patients who underwent pre IVF hysteroscopy. Further larger studies and randomized controlled trials are needed to verify the positive outcome of use of hysteroscopy prior to IVF.

Ultrasonographic evaluation of the diaphragm

BackgroundUltrasonography is a promising technique for structural and functional evaluation of the diaphragm. It is accurate, reproducible, and portable with no ionizing radiation. Multiple studies have reported ultrasonography as the modality of choice for evaluation of the diaphragm.ObjectiveThe aim was to assess the role of ultrasound (US) in the evaluation of the diaphragm, either normal or abnormal, through evaluating its morphology, integrity, and measuring different parameters such as diaphragmatic thickness, thickening fraction, and excursion with proper assessment of supra/infradiaphragmatic lesions that affect the diaphragm.Patients and methodsIn all, 118 patients were recruited from the Chest and Radiology Departments, Cairo University, in the period from January to July 2019. All patients were subjected to history taking, clinical examination, and ultrasonographic assessment of the diaphragm. High-frequency linear transducer of 7.5–12 MHz was used for imaging the diaphragm and measuring its thickness. A low-frequency curvilinear transducer with a frequency of 3.5–5 MHz was used for assessing diaphragmatic excursion.ResultsCases were grouped into two groups, those with normal US findings of the diaphragm represented group A, while patients with any diaphragmatic abnormality represented group B. Group B included 41 patients with intrinsic (56.09%) and extrinsic diaphragmatic abnormalities (46.34%). Five (12.2%) cases have thickened diaphragm; two (4.9%) cases have thinned-out diaphragm; two (4.9%) cases showed congenital diaphragmatic defects; three (7.3%) cases showed acquired diaphragmatic defects; 10 (24.4%) cases showed diaphragmatic weakness; and nine (21.95%) cases showed diaphragmatic paralysis (7.6%).ConclusionUS is the technique of choice for assessing diaphragmatic movement on suspicion of malfunctioning. Ultrasonography is a promising technique for structural and functional evaluation of the diaphragm.

Family history in developmental dysplasia of the hip: should we follow-up?

Objectives: Developmental dysplasia of the hip (DDH) is an important problem. Ultrasonography (US) is a proper method before 6 months of age. For older children, plain radiographs can be useful. Six risk factors are emphasized: breech presentation, female sex, a positive family history, being first-born, left hip affected, and mode of delivery. In some centers, clinicians prefer to perform a control US examination or pelvic radiographs after 6 months of age for the children having a positive family history. We aimed to evaluate the necessity of control US/direct radiography examinations. Methods: A total of 205 children with a positive family history for DDH are included. US examinations are performed according to Graf’s method. We have evaluated direct radiographs by using Hilgenreiner, Perkin, and Shenton lines, acetabular angle. Results: Initial US examinations are performed at a median age of 8.3 weeks. Seventy-four patients (36%) had a repeat ultrasound scan at a median age of 7 months; none of them demonstrated abnormal findings. One hundred and thirty-one patients (63.9%) had control radiographs at a median age of 8.2 months. Shenton line is considered as normal, and the upper femoral epiphysis is located in inferomedial quadrant according to Hilgenreiner and Perkin lines. Conclusions: A positive family history for DDH may be a less important reason for performing control US or radiographic examination. Patients with a normal screening US result and having risk factors can be discharged from follow up safely, so that unnecessary examinations and family anxiety will be reduced.