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INTRODUCTION: Posterior leukoencephalopathy syndrome (PRES) is a rare neurological disease possibly associated with the use of calcineurin inhibitors like cyclosporine A (CSA). Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) responsible for the outbreak of coronavirus disease 19 (COVID-19) can cause neurological manifestations. We described a case of CSA-related PRES whose diagnosis was difficult due to a concurrent infection with SARS-CoV-2. OBSERVATION: The 16-year-old patient was known to have corticosteroid-resistant nephrotic syndrome secondary to minimal change disease. CSA was introduced, and on the fifth day of treatment, the patient presented with seizures followed by fever. Biological and magnetic resonance imaging data were in favor of SARS-CoV-2 encephalitis. Relief of immunosuppression by discontinuation of CSA was decided and the patient was put on anticonvulsants. After being declared cured of COVID-19, which was without other clinical signs, the CSA was reintroduced but the patient presented with seizures the next day. This allowed the physicians to rectify the diagnosis and relate the seizures to a CSA-related PRES. CONCLUSION: Infection with SARS-CoV-2 could be a differential diagnosis of a PRES related to calcineurin inhibitors.
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COVID-19 , Ciclosporina , Síndrome da Leucoencefalopatia Posterior , Humanos , Síndrome da Leucoencefalopatia Posterior/induzido quimicamente , Síndrome da Leucoencefalopatia Posterior/diagnóstico , COVID-19/complicações , COVID-19/diagnóstico , Ciclosporina/efeitos adversos , Ciclosporina/uso terapêutico , Diagnóstico Diferencial , Adolescente , Imunossupressores/efeitos adversos , Imunossupressores/uso terapêutico , Masculino , Convulsões/etiologia , Convulsões/diagnóstico , Inibidores de Calcineurina/efeitos adversos , Encefalite Viral/diagnóstico , Encefalite Viral/tratamento farmacológico , Imageamento por Ressonância MagnéticaRESUMO
BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological entity most frequently described in young- or middle-aged adults with a rare occurrence among children. AIM: To determine the clinical, radiological features and outcome of PRES in children admitted to a Tunisian tertiary care pediatric department. METHODS: we retrospectively reviewed records of all children under 18 years old diagnosed with PRES and admitted to the PICU of the Pediatric department of Sahloul University Hospital from January 2000 to August 2021. RESULTS: Sixteen patients were enrolled in this study. The mean age of the study population at PRES onset was 10 years (range: 4-14 years) and the male female ratio was 3. The most frequent neurological signs were seizures (n = 16 cases), headache (n = 8 cases), and impaired level of consciousness (7 cases). Visual disturbances were found in one patient. Arterial hypertension was the most underlying cause (16 cases). Brain MRI showed vasogenic edema, mostly localized in the parietal (13 cases) and occipital (11 cases) lobes. Moreover, cytotoxic edema (2 cases), pathologic contrast enhancement (1 case), and hemorrhage (3 cases) were isolated on MRI. The outcome after specific management was favorable after the first onset in 13 cases and death occurred in 3 patients. Relapses were observed in 4 patients. CONCLUSION: Clinical features presented by children with PRES are variable and non-specific. MRI typically shows reversible posterior cerebral edema. However, in some cases, atypical neuro-imaging findings, such as cytotoxic edema infarction, hemorrhage and contrast enhancement can be observed.
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Síndrome da Leucoencefalopatia Posterior , Adulto , Pessoa de Meia-Idade , Humanos , Masculino , Criança , Feminino , Adolescente , Pré-Escolar , Síndrome da Leucoencefalopatia Posterior/diagnóstico por imagem , Síndrome da Leucoencefalopatia Posterior/etiologia , Estudos Retrospectivos , Centros de Atenção Terciária , Imageamento por Ressonância Magnética , EdemaRESUMO
BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disorder of the central nervous system. Little information is available about the clinical and neuroradiological profile or the follow-up of this disease in Tunisian children. AIM: To determine the clinical, laboratory, and radiological features and the outcome of ADEM in children admitted to the pediatrics department of a university hospital in Tunisia. METHODS: All children ≤ 18 years old presenting with ADEM and admitted to the tertiary referral center for pediatrics at Sahloul University Hospital from January 2000 to December 2020 were included in the study. The diagnosis of ADEM was confirmed according to the international pediatric multiple sclerosis study group criteria. RESULTS: A total of 20 patients (13 girls and 7 boys) fulfilled the diagnostic criteria for ADEM. The mean age at diagnosis was 5.6 years. The clinical presentation included polyfocal neurological signs such as cranial hypertension (45%), seizures (35%), and motor weaknesses (55%). Pyramidal tract signs and cranial nerve palsies were noted in 55% of cases. Brain magnetic resonance imaging showed particular features, namely, a relapsing tumor-like form in one case, and optic neuritis and demyelinating lesions of the white matter in the brain and the spinal cord with gadolinium cerebral ring-like enhancement in another case. The treatment consisted of intravenous immunoglobulin in 16 cases (80%) and corticosteroid in 19 cases (95%). Plasmapheresis was used for one patient. Complete recovery was observed in 12 patients (60%); 19 patients (95%) had a monophasic course of the disease while only one patient developed multiphasic ADEM. CONCLUSIONS: ADEM remains a difficult diagnosis in children. Nevertheless, after prompt diagnosis and adequate treatment, most children with ADEM have a favorable outcome with restitutio ad integrum.
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Encefalomielite Aguda Disseminada/diagnóstico , Adolescente , Corticosteroides/uso terapêutico , Criança , Pré-Escolar , Encefalomielite Aguda Disseminada/classificação , Encefalomielite Aguda Disseminada/epidemiologia , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Lactente , Masculino , Pediatria/métodos , Pediatria/estatística & dados numéricos , Estudos Retrospectivos , Tunísia/epidemiologiaRESUMO
Tfifha M, Kamoun T, Mama N, Mestiri S, Hassayoun S, Zouari N, Jemni H, Abroug S. Childhood sclerosing cholangitis associations in a Tunisian tertiary care hospital: a many-faceted disease. Turk J Pediatr 2019; 61: 905-914. Sclerosing cholangitis (SC) is a liver disorder affecting children and adults, causing chronic cholestasis and secondary biliary cirrhosis. The purpose of this study was to present different associated diseases to SC in a Tunisian tertiary care hospital. Six patients were identified with SC associated with other diseases, four males and two females. The first symptom was liver enlargement in all cases with abnormal liver biochemistry. A moderate increase in AST and ALT levels was registered in all cases with moderate cholestasis in 4 patients. Three of them presented an auto-immune condition. Two patients were diagnosed with auto-immune hepatitis prior to SC and Crohn disease in only one patient. One developed linear IgA bullous dermatosis. Three patients were diagnosed with Multisystemic Langerhans Cell Histiocytosis (LCH). The primary site of LCH was the liver associated secondary to insipidus diabetes (one case), mastoiditis (two cases) and chest localization (one case). The outcome of those patients was variable with poor prognosis especially for SC secondary to LCH. No patient underwent liver transplantation. SC is a rare disorder with variable clinical presentations. To our knowledge, this is the first report of this condition in Tunisian and North African children. Diagnosis and treatment of SC and its associations remains a challenge, especially because there is still no effective medical therapy aimed at preventing disease progression. Pediatric liver transplantation is the only life-extending therapeutic alternative for patients with end-stage liver failure. Liver transplantation has not been performed on young children in our country.
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Colangite Esclerosante/epidemiologia , Centros de Atenção Terciária/estatística & dados numéricos , Criança , Pré-Escolar , Progressão da Doença , Feminino , Humanos , Incidência , Lactente , Masculino , Tunísia/epidemiologiaRESUMO
Neurofibromatosis type 1 (NF1) is one of the most common genetic disorders. Gastrointestinal manifestations of NF-1 are seldom thought of in routine clinical practice and might thus be significantly under-recognised. Their heterogeneous spectrum ranges from localised microscopic proliferative lesions to grossly recognizable mass-forming neurofibromas, neuroendocrine and gastrointestinal stromal tumours (GIST). The aim of this study is discussing the imaging evaluation and characterisation of the abdomen lesions in patients with NF1. TEACHING POINTS: ⢠Neurofibromatosis type (NF-1) is one of the most common single gene disorders. ⢠Every organ system can be involved and intra-abdominal manifestations are underestimated. ⢠The NF1 abdominal manifestations comprehend five categories of tumours. ⢠Neurogenic tumours including with neurofibromas are the most common type. ⢠Early diagnosis of abdominal manifestations of NF-1 based on imaging patterns is necessary for appropriate treatment to avoid serious organic complications related to tumour mass.
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Langerhans cell histiocytosis (LCH) is a rare condition mostly seen in children and adolescents. Eosinophilic granuloma (EG) is one of its three clinical entities and is considered as a benign osteolytic lesion. Many reports of patients with spine histiocytosis are well documented in the literature but it is not the case of atlantoaxial localization. We report here a new observation of atlantoaxial LCH in a 4-year-old boy revealed by persistent torticollis. He was successfully treated with systemic chemotherapy and surgery. Inter-body fusion packed by autologous iliac bone was performed with resolution of his symptoms. It is known that conservative treatment is usually sufficient and surgery should be reserved for major neurologic defects in spine EG. In atlantoaxial lesion, surgical treatment should be frequently considered.
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Tfifha M, Gaha M, Gamaoun W, Chemli J, Mabrouk S, Hassayoun S, Zouari N, Jemni H, Abroug S. Clinical and imaging features of malignant infantile osteopetrosis. Turk J Pediatr 2017; 59: 452-457. Human osteopetrosis is a rare genetic disorder caused by osteoclast failure. It encompasses a group of highly heterogeneous forms, ranged widely in severity. Patients with autosomal recessive osteopetrosis are the most severely affected osteopetrotic patients. Here we describe Tunisian children with severe phenotype. They are native from the same geographic region, born to consanguineous parents. Clinical features were cranio-facial dysmorphy, macrocephaly, hepatosplenomegaly, severe anemia and thrombocytopenia with precocious onset of neuronopathic complications, blindness and deafness. Retinal atrophy, reported in a minority of forms is highlighted. Skeletal radiographs revealed generalized increase in bone density and abnormal metaphyseal remodeling, and superimposed rickets resulting from the defect in osteoclasts to provide a normal Ca/P balance. We report an exceptional association with congenital hypothyroidism. Multi-organ failure due to sepsis is one the most severe complications observed. The issue was fatal without hematopoietic stem cell transplantation.
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Anormalidades Múltiplas/diagnóstico por imagem , Anormalidades Múltiplas/etiologia , Osteopetrose/complicações , Osteopetrose/diagnóstico por imagem , Pré-Escolar , Hepatomegalia/diagnóstico por imagem , Hepatomegalia/etiologia , Humanos , Lactente , Recém-Nascido , Masculino , Esplenomegalia/diagnóstico por imagem , Esplenomegalia/etiologiaRESUMO
Crohn's disease (CD) is characterized by transmural inflammation of the gastrointestinal tract, which predisposes to the formation of fistula. Duodenal involvement occurs in less than 5% of cases and often leads to clinically relevant strictures. However, fistula formation in the duodenum is exceptional. Herein, we report an unusual case of duodenobiliary fistula due to CD occurring in a 65-year-old patient who was successfully treated by anti-tumor necrosis factor (TNF) agents. This case report highlights the efficacy of anti-TNF alpha agents in the treatment of a bilioenteric fistula because it increases the probability of clinical remission and mucosal healing and therefore reduces the need for surgical treatment which may be associated morbidity.
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Adalimumab/uso terapêutico , Anti-Inflamatórios/uso terapêutico , Doenças dos Ductos Biliares/complicações , Fístula Biliar/complicações , Doença de Crohn/complicações , Doença de Crohn/tratamento farmacológico , Duodenopatias/complicações , Fístula Intestinal/complicações , Idoso , Doenças dos Ductos Biliares/tratamento farmacológico , Fístula Biliar/tratamento farmacológico , Duodenopatias/tratamento farmacológico , Feminino , Humanos , Fístula Intestinal/tratamento farmacológicoRESUMO
Duodenal adenocarcinoma is frequent. Aneurysmal dilatation of the small bowel is reported to be a lymphoma characteristic imaging finding. A 57-year-old male was found to have a duodenal adenocarcinoma with aneurismal dilatation on imaging which is an exceptional feature. On laparotomy, the wall thickening of the dilated duodenum extended to the first jejunal loop, with multiple mesenteric lymph nodes and ascites. Segmental palliative resection with gastro-entero-anastomosis was done. Histopathology revealed a moderately differentiated adenocarcinoma with neuro-endocrine differentiation foci. Wide areas of necrosis and vascular emboli were responsible for the radiological feature of the dilated duodenum with wall thickening.
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Adenocarcinoma/diagnóstico , Neoplasias Duodenais/diagnóstico , Adenocarcinoma/diagnóstico por imagem , Anastomose Cirúrgica , Bário/química , Dilatação , Neoplasias Duodenais/diagnóstico por imagem , Evolução Fatal , Humanos , Intestino Delgado/diagnóstico por imagem , Linfonodos/diagnóstico por imagem , Linfonodos/patologia , Masculino , Pessoa de Meia-Idade , Necrose , Tomografia Computadorizada por Raios XAssuntos
Carcinoma de Células de Transição/diagnóstico por imagem , Neoplasias Penianas/diagnóstico por imagem , Neoplasias Uretrais/diagnóstico por imagem , Idoso , Carcinoma de Células de Transição/patologia , Carcinoma de Células de Transição/cirurgia , Progressão da Doença , Evolução Fatal , Humanos , Imageamento por Ressonância Magnética , Masculino , Neoplasias Penianas/patologia , Neoplasias Penianas/cirurgia , Radiografia , Neoplasias Uretrais/patologia , Neoplasias Uretrais/cirurgiaRESUMO
Distant metastases of the parotid gland are uncommon. They arise from primary tumors located in the head and neck, mainly melanoma and epidermoid carcinoma. Other histological types of metastasis are very rare. We report an exceptional case of parotid metastasis of myxoid liposarcoma in a 42-year-old man and insist on the worse prognosis of this entity.
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OBJECTIVES: The authors present the various imaging features of renal hydatid cyst and define the place of the various imaging modalities. MATERIAL AND METHODS: This retrospective study was based on 41 cases of renal hydatid cyst treated between 1989 and 2003, including 19 men and 22 women with a mean age of 32 years. KUB x-ray was performed in every case, ultrasound was performed in 39 cases, computed tomography (CT) was performed in 13 cases and MRI was performed in 3 cases. RESULTS: The preoperative diagnosis of renal hydatid cyst was established in 39 cases (95%), based on ultrasound in 31 cases (75%), CT in 5 cases (12%) and MRI in 3 cases (7%). The diagnosis was confirmed histologically in 41 cases. CONCLUSION: The diagnosis of renal hydatid cyst is often simple in hydatid endemic countries and is based on a combination of KUB-ultrasound. CT constitutes the second-line examination, which should be reserved for complicated cysts. MRI is the modality of choice in the case of atypical forms ensuring the differential diagnosis with serous cyst and cystic renal cancer