Fatma Rabah

Brain is not always the last fortress; osteosarcoma with large brain metastasis.

Osteosarcomas are the most common malignant primary bone tumors in children and adolescents. Brain metastases of osteosarcoma are very rare and carry a dismal prognosis. We report a case of chondroblastic osteosarcoma of right humerus presented with right frontal lobe metastasis in a 10-year-old girl with small pulmonary lesions.

Postvaricella thrombosis-report of two cases and literature review

Varicella (chickenpox) is a common disease of childhood, caused by varicella-zoster virus. Postviral thromboembolism is a rare complication of varicella-zoster virus in childhood. We describe 2 children who developed lower limb deep venous thrombosis shortly after varicella infection, along with a review of 130 previously reported cases.

Echocardiography in PICU: when the heart sees what is invisible to the eye

OBJECTIVE Echocardiography has become an indispensable bedside diagnostic tool in the realm of pediatric intensive care units (PICU). It has proven to be an influential factor in the formula of clinical decision-making. This study aimed to delineate the impact of echocardiography on the management of critically ill pediatric patients in the PICU at Sultan Qaboos University Hospital, Oman. METHOD This was a retrospective cohort study conducted in a five-bed PICU. Patients admitted to the PICU from January of 2011 to December of 2012 were reviewed. Those who have undergone bedside echocardiography during their ICU stay were recruited. Electronic patient record was used as data source. RESULTS Over a-24-month period, 424 patients were admitted in this PICU. One hundred and one clinically indicated transthoracic echocardiograms were performed. 81.8% of these presented new findings (n=82) that significantly impacted the clinical decision of patient management, namely, alteration in drug therapy and procedure, whereas no difference in the management was yielded in the remaining 17.8% of the studied cases. CONCLUSIONS Echocardiography had a significant impact on the management of PICU patients. Such salutary effect was consequently reflected on the outcome. Pediatric intensivists are encouraged to acquire such bedside skill.

A child with phenylketonuria and focal segmental glomerulosclerosis, the bright side of proteinuria

Phenylketonuria (PKU) is the most common inborn error of amino acid metabolism. Phenylalanine hydroxylase is the underlying deficient enzyme. If left untreated, growth failure, microcephaly, global developmental delay, seizures and severe intellectual impairment would characterize the clinical picture of PKU. On the other side of protein homeostasis lies nephrotic syndrome. It is a well-known quantitative defect due to significant proteinuria. Focal segmental glomerulosclerosis (FSGS) is a special congenital variant affecting children and adults. Hereby, we describe a three- year old male child who presented with generalized edema and global developmental delay. Investigations revealed PKU along with FSGS. We assume that congenital nephrosis ameliorated the picture of PKU, and had a salutary effect on the growth and development. Such coexistence between PKU and FSGS hasn’t been described before.

Amlodipine: The double edged sword

Amlodipine, a calcium channel blocker became one of the most popular antihypertensive medications used in the paediatric population. This is mainly due to its long half‐life making it easy to use as a single daily dose. Amlodipine is associated with common side effects like flushing, headache and dizziness. The incidence of amlodipine‐related oedema was noted to be lower in the paediatric population compared to adults. We emphasise the importance of monitoring the development of oedema in patients treated with amlodipine.

Artigo originalEchocardiography in PICU: when the heart sees what is invisible to the eyeEcocardiografia na UTIP: quando o coração vê o que é invisível aos olhos☆

Objective Echocardiography has become an indispensable bedside diagnostic tool in the realm of pediatric intensive care units (PICU). It has proven to be an influential factor in the formula of clinical decision‐making. This study aimed to delineate the impact of echocardiography on the management of critically ill pediatric patients in the PICU at Sultan Qaboos University Hospital, Oman.

A Demyelinating Disease; What Lies Beneath?

A 5-year-old boy presented with 1 mo history of excessive sleepiness, early morning headache and vomiting. Moreover, a 5-day history of increasing irritability was noted. Clinical examination demonstrated bradycardia, hypertension and right-side facial palsy. Subsequently, an urgent CT brain was done which revealed diffuse vasogenic cerebral edema with extensive white matter hypodensities. Such white matter changes were further defined by MRI (Fig. 1). Herpetic encephalitis and acute demyelinating encephalomyelitis were the top differential diagnoses. Accordingly, the child was given IVIG and IV acyclovir. Despite treatment, his Glasgow coma scale dropped gradually to 7 meriting intubation. The deteriorating clinical course necessitated exclusion of primary central nervous system lymphoma (PCNSL). Therefore, a stereotactic brain biopsy was executed. The pathology report was diagnostic of diffuse large B cell lymphoma (DLBCL). Further workup has unveiled spinal drop metastases. PCNSL constitutes 3 % of all brain tumors [1]. About 90–95 % of the cases are constituted by DLBCL, possibly affecting brain, spinal cord, eyes, meninges, and cranial nerves [2]. Our patient was commenced on chemotherapy protocolB LMB 89 group C^ with high dose methotrexate and whole brain irradiation [3]. He was brought into remission over the 3 y. MRI features of PCNSL in immunocompetent patients usually are non-specific; MRI shows a single lesion in 65 % of cases. Such a lesion is either homogenously isointense or hypointense to cortex on T1weighted images or hyperintense on T2-weighted images [4]. The borders of the lesion are either well defined or irregular with variable extent of surrounding edema and homogenous gadolinium enhancement [2, 4]. The lesions are frequently hyperintense on diffusion weighted imaging and hypointense on apparent diffusion coefficient maps. Such diagnostic radiologic feature is attributed to the restricted diffusion rate of unbound extracellular water molecules due to hypercellularity of PCNSL [5]. The rarity of such childhood malignancy coupled with the nonspecific presenting symptoms that could be explained by commoner benign diseases comprise a diagnostic challenge [1]. CNS malignancy is an important cause of diffuse demyelination that should be explored if the presentation of ADEM is compounded with persisting or worrisome clinical picture. Reaching such a diagnosis in a timely fashion is of crucial significance to patient’s outcome [1]. * Fatma Rabah fatmarabah@gmail.com

Bilateral hydroureters and hydronephrosis in a neonate

A newborn boy was diagnosed antenatally with bilateral hydronephrosis. Postnatal renal ultrasound scan (USS) measured a renal pelvic anteroposterior diameter (APD) of 12 mm on the left side and 7 mm on the right side. The baby had good urine stream. Parents missed the repeat USS at the age of 1 week. An ultrasound done at 4 weeks revealed progressive hydronephrosis, bilateral hydroureters, with increased renal echogenicity. Normal bladder wall thickness was noted but two intravesical lesions were seen (figures 1 and 2). The APD was 13.5 and 11 mm on the left and right side, respectively. Figure 1 Renal ultrasound scan of (A) left kidney (LT) and (B) right kidney (RT) showing bilateral hydronephrosis (white arrows) and hydroureters (red arrow). Increased renal echogenicity is not shown in the figure. Figure 2 Showing normal bladder wall thickness but two intravesical lesions were seen (white arrows). Question How would you describe the intravesical lesions in figure 2? Bilateral ureteroceles Bilateral vesicoureteral reflux (VUR) Bilateral pelvi-ureteric junction obstruction Posterior urethral valves (PUVs) Which complication(s) may you expect in such cases? Urinary tract infection (UTI) Obstructive voiding symptoms Failure to thrive Ureteral calculus All of the above How would you treat this problem? Endoscopic puncture Deflux surgery Pyeloplasty Vesicostomy Answers are on page ▪▪▪

Right iliac fossa pain: appendicitis is not always the answer.

The CT shows a target sign suggestive of an ileocecal intussusception. At laparoscopy, there was a cavitary proximal jejunal mass with brown ascitic fluid. Histopathology showed diffuse infiltration by intermediate-sized lymphoid cells that were positive for CD20, CD43, CD10, Bcl-2, and Bcl-6. Interspersed histiocytes gave a “starry sky” appearance. The k-light chain was the dominant surface immunoglobulin. PET scan was positive in the spleen, peritoneum, and bone marrow. CSF cytospin did not show any blasts. There were 74% blasts in the marrow. The final diagnosis was mature B-cell (Burkitt) leukemia. Complete blood count (CBC) performed before appendectomy showed: hemoglobin 12.2 g/dL, platelet count 376 10/L, WBC 5.9 10/L, and ANC 4.1 10/L. CBC taken before resection anastomosis showed: hemoglobin 11.5 g/dL, platelet count 363 10/L, WBC 23.5 10/L and ANC 13.2 10/L. Burkitt lymphoma is a rapidly growing malignancy. Usually arising in the lymph nodes, the gastrointestinal tract (ileum and jejunum) is the most common extranodal site.1,2 An intestinal lymphoma may serve as the leading point for intussusception. Thus, right lower quadrant pain should raise the possibility of malignancy, especially if there is a palpable mass or signs of intestinal obstruction. Indeed, many patients with Burkitt lymphoma are initially diagnosed as acute appendicitis.3 Prompt diagnosis is important as untreated Burkitt lymphoma may rapidly spread to the bone marrow and/or central nervous system.

Hypertrophic Obstructive Cardiomyopathy in An Infant with Hemophagocytic Lymphohistiocytosis; Answer to a Riddle

Familial hemophagocytic lymphohistiocytosis (FHLH) is a hereditary hyperinflammatory condition with T-cell and macrophage activation. Treatment consists of immunosuppressive therapy plus bone marrow transplantation. Cardiac manifestations of FHLH were scarcely mentioned in the literature with conflicting pathophysiological explanations. We report a case of hypertrophic obstructive cardiomyopathy associated with FHLH. Guided by such a case, a clear vision regarding the real cause is thought to be obtained in the cloudy landscape of pathophysiology.