Neurologic Complications in Patients With Lymphoreticular Malignancy: A Descriptive Cohort Study.

OBJECTIVES: The objectives of this study were to study the spectrum of neurologic complications in children with lymphoreticular malignancy (acute lymphoblastic leukemia, Hodgkin, and non-Hodgkin lymphoma) at diagnosis and during treatment and to determine the etiology of these complications. MATERIALS AND METHODS: In this descriptive cohort study, conducted between November 2018 and March 2020, 204 children with a diagnosis of lymphoreticular malignancy were enrolled. The baseline investigations were done in all the cases. Those who developed neurological symptoms were evaluated with cerebrospinal fluid examination and radiologic and electrophysiologic studies as per indication and were managed according to standard management guidelines. RESULTS: Of the 204 patients, 30 (14.7%) developed neurological complications. The majority of these complications (n=20/30; 87%) occurred during the intensive chemotherapy period. Common complications included acute methotrexate neurotoxicity (n=7), vincristine-induced neurotoxicity (n=7), central nervous system (CNS) relapse (n=4), and posterior reversible encephalopathy syndrome (n=2). L-asparaginase-induced thrombosis (n=1), intramedullary compression syndrome (n=1), CNS infection (n=2), CNS hemophagocytic lymphohistiocytosis (n=1), and steroid-induced myopathy (n=1) were also observed. The complications resolved in 21/30 (70%) patients after receiving appropriate treatment while the neurological complication persisted in 2/30 (6.7%) patients. Three patients (10%) abandoned the treatment, and 4 (13.3%) patients expired. CONCLUSIONS: Neurologic complications in patients with lymphoreticular malignancy are quite variable, having common presenting symptoms but varying imaging abnormalities. By close follow-up and effective treatment, the morbidity and mortality of these complications can be minimized.

Results of Injection Sclerotherapy with Bleomycin in Pediatric Lymphatic Malformations.

AIM: The aim of the study was to evaluate the results of injection sclerotherapy with bleomycin in pediatric patients with lymphatic malformations. MATERIALS AND METHODS: In this prospective cohort study, all consenting pediatric patients with macrocystic lymphatic malformations were managed with injection bleomycin sclerotherapy (0.5 mg/kg, not exceeding 5 mg at a time) under ultrasound (US) guidance. After aspirating the cyst fluid bleomycin was instilled intralesionally in a ratio of 5:1 (aspirated cyst fluid volume: diluted bleomycin solution volume). Patients were reassessed at three weekly intervals. The response to therapy was assessed clinically as well as by size and volume on ultrasound Doppler study. The response was classified as excellent response, i.e., complete regression, good response >50% regression, and poor response <50% regression. RESULTS: Sixty patients with lymphatic malformations were enrolled in the study, the mean age was 3.22 years, and the male-to-female was 2.5:1. The most common site of lesion was in the neck (43.3%), followed by the axilla (15%) and flank (8.3%). The responses were excellent, good, and poor in 43 (71.6%), 12 (20%), and five (8.3%) patients, respectively. Two patients underwent surgical excision of the residual lesion. Complications noted were fever in six, local pain in five, and residual lesion in three patients. CONCLUSION: Sclerotherapy with bleomycin is simple, safe, and effective in the first line of management for macrocystic lymphatic malformations in children.

Clinical Profile and Microbiologic Spectrum of Febrile Neutropenic Episodes in Children With Severe Aplastic Anemia.

BACKGROUND: Febrile neutropenia (FN) is a common life-threatening complication in patients with severe aplastic anemia (SAA). However, few studies have examined the spectrum of infections in FN in patients with SAA, especially in children. Therefore, the current study was planned to study the clinicomicrobiologic profile of FN episodes in these children. MATERIALS AND METHODS: Data of 38 episodes of FN that occurred in 31 children with SAA from November 2015 to April 2017 were collected prospectively and analyzed. RESULTS: FN episodes occurred more frequently (54.8%) in patients on immunosuppressive therapy. Clinically documented infections accounted for 21 (55.26%) episodes, microbiologically documented infections for 15 (39.47%), bacteremia for 13 (34.21%), and invasive fungal diseases for 6 (15.78%) episodes. Among clinically documented infections, the lower respiratory tract was the commonest site in 23.68% episodes, followed by skin and soft tissue infections. No focus of infection could be identified in 12 (31.57%) episodes. Gram-negative bacteria (71.42%) were the predominant isolates (commonest Klebsiella pneumoniae) over Gram-positive bacteria (commonest coagulase-negative Staphylococcus). High prevalence of aminoglycoside, piperacillin-tazobactam, and carbapenem resistance was noted among Gram-negative organisms. Gram-positive organisms showed excellent sensitivity to vancomycin, linezolid, and clindamycin. The overall mortality rate was 42%. CONCLUSIONS: Empirical antimicrobial therapy should include adequate coverage for Gram-negative pathogens. The antimicrobial regimen should be modified according to the results of the culture and sensitivity testing.

Wrist Joint Skeletal Changes in Children With Transfusion-dependent Thalassemia.

BACKGROUND: Arthropathies and bone deformities are well known to occur in patients with thalassemia major and have been attributed to the disease or to its therapy. Before the advent of chelation therapy, these children developed widened diploic space and "hair-on-end" pattern in skull, "cobweb" pattern in the pelvis, and the lack of the normal concave outline in the long bones because of extensive marrow proliferation. After the introduction of iron-chelation therapy, these patients were noted to develop metaphyseal abnormalities and vertebral changes resembling spondylo-metaphyseal dysplasia. Only one study has shown some association of deferiprone (chelating agent) use with distal ulnar changes in these children. Our study was done to describe the skeletal changes and deformities in wrist joints of children with transfusion-dependent thalassemia and correlate them with age, mean pretransfusion hemoglobin level, mean serum ferritin level, and type and duration of chelation therapy in these children. METHODS: A total of 60 children with transfusion-dependent thalassemia from the thalassemia daycare center were examined. These children were divided into 3 groups on the basis of their age (group A: 2 to 6 y, group B: 6 to 10 y, and group C: 10 to 14 y). Detailed history, including treatment history, number of blood transfusions received over the last 1 year, clinical examination, and radiologic assessment of both forearm with wrists were done. RESULTS: The clinical and radiologic differences in radial and ulnar lengths increased significantly with the increasing age of these patients, the ulna being short. There was some correlation between increasing negative ulnar variance and distal radial articular angle with deferiprone consumption. CONCLUSION: Chelation therapy, particularly with deferiprone, may cause distal ulnar growth arrest causing ulnar shortening and progressive radial bowing in these children. LEVEL OF EVIDENCE: Level IV-case series.

The Syndromic Spectrum of COVID-19 and Correlates of Admission Parameters with Severity-outcome Gradients: A Retrospective Study.

BACKGROUND: Clinical and laboratory features of COVID-19 may have regional variations. This study aimed to discern their association with severity of illness and mortality in tertiary setup of Delhi, India. METHODS: Retrospective data of hospitalised COVID-19 patients over 3 months (end March to June 2020) were evaluated for symptom profile, blood investigations and chest radiograph data and classified according to COVID-19 severity and as survivors and non-survivors. RESULTS: Average age (n=182) was 46.1 years, male to female ratio 1.4:1. Fever (51.1%), cough (49.4%) and breathlessness (48.3%) were the commonest symptoms, and frequency of all the three increased with severity of COVID-19. Fever duration, leucocytosis, neutrophilia, elevated blood urea, transaminitis and higher Brixia score on chest X-ray were also more in severe COVID-19 compared to mild and moderate categories. Higher age, more comorbidities, fever, breathlessness and chest pain; longer duration of fever, leucocytosis, neutrophilia, lymphopenia, high neutrophil to lymphocyte ratio, elevated serum urea, creatinine, transaminases and hyperglycemia, and higher radiographic Brixia score were observed in non-survivors compared to survivors. CONCLUSION: Greater prevalence of symptoms (alone and in combination) and derangements in blood biochemistry are seen in severe COVID-19 compared to mild or moderate cases, and also in non-survivors compared to survivors.

Radiologic Evaluation of Uterovaginal Abnormalities in Girls with Congenital Pouch Colon.

BACKGROUND/PURPOSE: The radiologic investigations of 25 girls with congenital pouch colon (CPC), managed over 17 years, were retrospectively reviewed. In 13 girls who form the study group, the investigations provided information about the anomalous uterovaginal (UV) anatomy and these findings were studied. MATERIALS AND METHODS: Age at presentation was 2 months to 10 years. The subtypes of CPC were Type I (n = 2), Type II (n = 9), and not recorded (n = 2). All patients had a double vagina and a unicornuate uterus on each side in the pelvis. The radiologic studies, performed at varying periods after surgery, included an intravenous urogram (IVU) (n = 4), micturating cystourethrogram (MCU) (n = 3), distal ileostogram/colostogram (n = 6), and magnetic resonance imaging (MRI) (n = 7). RESULTS: IVU and MCU showed retrograde filling of the vaginas with contrast during micturition with a small-capacity urinary bladder and a relatively open bladder neck. The two vaginas were quite apart, but symmetrical in appearance and position. A distal dye study showed filling of the colonic pouch, its terminal fistula, and the two vaginas in six patients. Opacification of the bladder was seen in 3/6 girls, including one girl with left-sided Grade IV vesicoureteral reflux. MRI (n = 7) showed a monocornuate uterus on each side in the pelvis. The upper vaginas on each side were apart, being widely apart (n = 5) and somewhat closer (n = 2). The lower vaginas were closer with an intervaginal septum. Other findings were a widely open bladder neck and urethra in two girls with urinary incontinence and visualization of the terminal fistula of the colonic pouch (n = 2). The lumbosacral spine was normal in all patients. CONCLUSIONS: In girls with CPC, retrograde reflux of contrast into the vaginas during a distal dye study or an IVU/MCU may provide useful details of the anatomy of the vaginas. An MRI scan is recommended as essential for comprehensive evaluation of the anomalous UV anatomy.

Approach to the diagnosis of skeletal dysplasias: Experience at a center with limited resources.

PURPOSE: A fetus with skeletal disorder poses diagnostic challenges in a resource-poor setting with limited management options. The objective of the study was to develop a step-by-step approach for the diagnosis of skeletal dysplasia in light of the limited resources available. METHODS: An algorithmic approach was used. The assessment for lethality was the first step, followed by the evaluation for fractures. In cases without evidence of fracture, severe constriction of thorax or associated polydactyly were searched for. In cases without severe thoracic constriction, the severity of micromelia was evaluated. After delivery, fetal examination was done to ascertain the etiology. RESULTS: During the 6-year period, 41 cases with shortened long bones were fully evaluated. Lethality was suspected in 30 cases. Fracture and beading were present in eight cases, and severe thoracic constriction with polydactyly was observed in seven cases. Mild micromelia was seen in 19 cases and severe micromelia in 7 cases. Among lethal skeletal dysplasias, thanatophoric dysplasia was most common (six cases). Among nonlethal skeletal dysplasias, achondroplasia was seen in eight cases. CONCLUSIONS: Lethality of skeletal dysplasia could be predicted on prenatal ultrasound with 100% accuracy. The step-by-step approach was helpful to characterize skeletal dysplasias. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 44:529-539, 2016.

Hepatic cysticercosis: a rare entity.

Hepatic cysticercosis is a very rare entity; only four cases have been reported to date. High-resolution ultrasonography of the abdomen is the initial and most reliable modality for evaluation of hepatic cysticercosis. Medical therapy is the mainstay of treatment. We report a case of hepatic cysticercosis in a 28-year-old male who presented with right upper quadrant pain, fever, and jaundice. The article also describes the imaging patterns of hepatic cysticercosis based on different stages of evolution.

Imaging spectrum of horseshoe lung: A series of four cases.

Horseshoe lung (HL) is a rare congenital anomaly represented by the fusion of both lungs, posterior to the heart, and is typically associated with various bronchopulmonary and cardiovascular malformations. Multi-detector contrast enhanced CT is the imaging modality of choice to demonstrate the pathology and associated malformations. There has been inconsistency in the nomenclature used for such cases in literature. To resolve ambiguity, the authors emphasise that only two terms: HL and pseudo-HL be used on imaging to describe variants of this congenital malformation. Contribution: A description of the imaging features in four cases of HL, with their associated malformations and a review of the nomenclature.

Distal ulnar changes in children with thalassemia and deferiprone related arthropathy.

BACKGROUND: Regular blood transfusion and iron chelation are the standard of care for children with thalassemia. Deferiprone is an effective oral iron chelator but is known to cause significant arthropathy. Though clinical and radiographic features of deferiprone related arthropathy have been described, the long-term effects are not known. PROCEDURE: Routine radiographs of left wrist and hand done for bone age estimation in 40 children with thalassemia were evaluated and revealed unique radiographic changes in 13 children (10 males: 3 females) with previous or current deferiprone related arthropathy. Subsequently, these children underwent radiographs of both the knee joints. RESULTS: The changes on wrist X-ray included lucency and thinning of the ulnar metaphysis, small ulnar epiphysis, deformation and impaired growth of the physeal cartilage leading to reduced distance between the epiphysis and metaphysis. The knee radiograph showed subchondral flattening of femoral and tibial condyles with irregular articular margins. CONCLUSIONS: Bony dysplasia, deformation and impaired growth of ulnar epiphyses, metaphyses and physes may be an expression of deferiprone related arthropathy in children with thalassemia major.

Postnatal outcome of congenital anomalies in low resource setting.

OBJECTIVE: This study aimed to determine the postnatal outcome of congenital malformations in a tertiary care hospital of India. MATERIAL AND METHODS: This was a prospective study of all women with prenatally detected major congenital malformations. Postnatal follow-up of live born babies was carried out for 1 year. RESULTS: There were 574 cases with major congenital anomalies, 523 of which were fully followed. Only 69 women (13.6%) had the initial scan before 20 weeks of gestation. Craniospinal defects were the most common (42.7%), followed by genitourinary anomalies (28%). There was no live birth in cases such as anencephaly, iniencephaly, bilateral renal agenesis, gastroschisis, and cystic hygroma. Survival at 1 year was less than 25% in spina bifida, bilateral cystic kidneys, complex cardiac disease, and non-immune hydrops fetalis. In cases with mild hydrocephalus or unilateral and mild renal disease, the survival was over 75%. CONCLUSION: In India, the majority of congenital anomalies present late in gestation. Although fetal outcome is invariably poor for severe defects, existing legislation in the country leaves pregnancy continuation as the only option.

Cystic lung tuberculosis in children: A series of five cases.

Frequent imaging manifestations of pulmonary tuberculosis are airspace or interstitial nodules with or without tree-in-bud nodules, consolidation, cavitation, ground glass opacity, miliary nodules, lymphadenopathy and pleural effusion. It is unusual to encounter cystic changes in patients with pulmonary tuberculosis, and these findings should be differentiated from other cystic lung diseases. This case series describes five cases of cystic lung disease in children with tuberculosis (TB) with illustrative chest radiography and CT findings. Contribution: The manuscript highlights the need to consider tuberculosis as a possible cause of acquired cystic lung disease in appropriate clinical settings, particularly in endemic regions.

Pancreatoblastoma with Portal Venous Invasion: A Case Series.

We describe the sonography and computed tomography (CT) findings in three children with pancreatoblastoma and highlight the tendency of these tumors to invade the splenoportal axis. All three of our cases showed tumor thrombus within the portal vein which is a helpful diagnostic feature on imaging studies.

Ultrasound as a Sole Modality for Prenatal Diagnosis of Placenta Accreta Spectrum: Potentialities and Pitfalls.

Background Placenta accreta spectrum (PAS) is a significant cause of maternal and neonatal mortality and morbidity. Its prevalence has been rising considerably, primarily due to the increasing rate of primary and repeat cesarean sections. Accurate prenatal identification of PAS allows optimal management because the timing of delivery, availability of blood products, and recruitment of skilled anesthesia, and surgical team can be arranged in advance. Aims and Objectives This study aimed to (1) study the ultrasound and color Doppler features of PAS, (2) correlate imaging findings with clinical and per-operative/histopathological findings, and (3) evaluate the accuracy of ultrasound for the diagnosis of PAS in patients with previous cesarean section. Materials and Methods This prospective study was conducted in radiology department of a tertiary care hospital. After screening 1,200 pregnant patients, 50 patients of placenta previa with period of gestation ≥ 24 weeks and history of at least one prior cesarean section were included in the study. Following imaging features were evaluated: (1) gray scale covering intraplacental lacunae, disruption of uterovesical interface, myometrial thinning, loss of retroplacental clear space, and focal exophytic masses; and (2) color Doppler covering intraplacental lacunar flow, hypervascularity of uterine serosa-bladder wall interface, and perpendicular bridging vessels between placenta and myometrium. Study Design Present study is a prospective one in a tertiary care hospital. Results Of the 19 PAS cases, 18 were correctly diagnosed on ultrasonography (USG) and confirmed either by histopathological analysis of hysterectomy specimen or per-operatively due to difficulty in placental removal. PAS was correctly ruled out in 27 of 31 patients. The diagnostic accuracy of USG was 90%. The sensitivity, specificity, positive, and negative predictive values were 94.7, 87.1, 81.8, and 96.4%, respectively. Conclusion Ultrasound is indispensable for the evaluation of pregnant patients. It is an important tool for diagnosing PAS, thereby making the operating team more cautious and better equipped for difficult surgery and critical postoperative care. It can be relied upon as the sole modality to accurately rule out PAS in negative patients, thereby obviating unnecessary psychological stress among patients due to possible hysterectomy.

A Step-by-Step Sonographic Approach to Vascular Anomalies in the Pediatric Population: A Pictorial Essay.

Vascular anomalies are a common cause of soft-tissue masses in children and often referred for ultrasonographic (USG) evaluation. They are broadly classified as vascular tumors (hemangiomas, hemangioendotheliomas, and angiosarcomas) or vascular malformations (venous malformations, lymphatic malformations, and arteriovenous malformations). Findings on USG and Doppler imaging can be used to categorize vascular anomalies into high- or low-flow lesions, which forms the basis for further workup, diagnosis, and management. On careful evaluation of various sonographic features, in conjunction with clinical findings, an accurate clinicoradiological diagnosis can be made in most cases. Further imaging with magnetic resonance (MR) imaging or computed tomography (CT) helps in delineation of lesion extent, whereas MR or CT angiography is useful to map the vascular supply of high-flow lesions. We have illustrated and discussed a step-by-step approach to diagnose vascular anomalies using ultrasound and Doppler imaging.

A Pattern-based Imaging Approach to Pediatric Jaw Lesions.

Jaw lesions in the pediatric population, although infrequently encountered in clinical practice, can cause functional impairment and cosmetic disfiguring. It is further complicated by the difficulty in diagnosis due to complex anatomy and facial developmental process during infancy and childhood. Intraosseous pediatric jaw lesions may vary from odontogenic to nonodontogenic types with nonspecific clinical features in most cases. They deserve careful attention by a systematic approach to provide a relevant diagnosis or differential diagnosis for timely management. Imaging plays a major role in diagnosis with orthopantomograph being the foremost investigation, followed by cross-sectional imaging, essentially computed tomography as a problem-solving tool. This article highlights the imaging spectrum of various jaw lesions in the pediatric population with a pattern-based approach for radiological diagnosis.

Laparoscopic treatment of gastric bezoar.

A seventeen-year-old female presented with a symptomatic abdominal mass that was diagnosed by barium meal and computed tomography to be a gastric bezoar. She underwent laparoscopic removal of the bezoar, through an anterior wall gastrostomy in an endobag, which was extracted piecemeal through a 4-cm upper midline incision. The technique is described with a review of a few previous laparoscopic-assisted cases.

Evolving Concepts in Ultrasonography of Pediatric Intussusceptions: Unequivocal Differentiation of Ileocolic, Obstructive and Transient Small-Bowel Intussusceptions.

The main aim of the study was to elaborate on the various ultrasonographic parameters that can be used for definite differentiation of ileocolic, obstructive small-bowel and transient small-bowel intussusceptions (SBI). In this study, 90 children (63 boys and 27 girls) with suspected intussusception were evaluated. Of these, 54 cases were diagnosed as obstructive intussusceptions (33 ileocolic and 21 ileoileal), 15 cases were classified as transient SBIs and 21 cases were negative for intussusception. The mean fat core diameter was 1.45 ± 0.32 cm for ileocolic versus 0.37 ± 0.06 cm for obstructive SBI versus 0.29 ± 0.08 cm for transient SBI (p < 0.001). The mean lesion diameter was 3.23 ± 0.08 cm for ileocolic intussusceptions and 2.12 ± 0.038 cm for SBI (p < 0.001), and the ratio of inner fat core to outer wall thickness was greater than 1 for ileocolic intussusceptions and less than 1 for SBI. A statistically significant difference was found between segmental invagination of transient versus obstructive SBIs with mean values of 1.93 ± 0.39 cm and 3.17 ± 0.25 cm, respectively, and an "optimal" threshold at 2.5 cm.

Faun tail nevus with aplasia cutis congenita.

Faun tail nevus describes abnormal lumbar hypertrichosis, which may overlie on occult spinal abnormality and be a marker of asymptomatic underlying spinal dysraphism. We report a case of faun tail nevus, with dermal pits along with aplasia cutis congenita and asymptomatic spina bifida occulta, tethered conus, and diastematomyelia, a constellation of findings which to our knowledge has not been previously reported.