A 3 year old Hispanic male presented with fevers, skin rash, left neck swelling and refusal to walk of several days duration. Physical exam revealed a febrile, fussy toddler with a tender, cystic lesion in the left submandibular region. Both ankles had tender cystic lesions on the lateral malleolar regions. Labs: WBC 33,000 with neutrophilia, bandemia, thrombocytosis, and increased ESR and CRP. MRI of the neck and ankles revealed cystic lymphatic malformations with no communication with the joints.ENT specialist was consulted and neck cystic lesion was aspirated to rule out a septic focus. Bone scan of the lower extremities ruled out infectious or malignant etiology. He was started on multiple antibiotics with a presumed diagnosis of sepsis. An ECHO on the 4th hospital day showed a pericardial effusion which required a pericardial window. He also developed bilateral pleural effusions which resolved with supportive treatment. Aspirates from the cystic lesions, pericardial fluid, blood and urine cultures were sterile. Even in the second week of hospital stay, he continued to spike high fevers (Tmax 107) with high white counts and left shift inspite of treatment with antibiotics. A diagnosis of systemic onset juvenile idiopathic arthritis (SOJIA) was made with input from rheumatologist. Antibiotics were discontinued and steroids were started with good response. Cystic lesions were treated with percutaneous sclerotherapy with doxycycline. He was discharged home on oral steroids, NSAID’s and weekly methotrexate. Etanercept was added to decrease dependence on oral steroids. He remains in good health 2 years since initial presentation. Discussion: A febrile toddler who refuses to walk is a common clinical presentation in pediatrics. Differential diagnosis of such a patient includes osteomyelitis, septic arthritis, acute rheumatic fever, leukemia and non-accidental trauma. The presence of systemic extra-articular symptoms as in our patient must alert the pediatrician to systemic onset juvenile rheumatoid arthritis (SOJIA). It accounts for 10-20% of all juvenile idiopathic arthritis (JIA) patients with an incidence of 0.4-0.8 per 100,000. SOJIA differs from other conditions in its multisystem involvement. Clinical features like pleurisy, pericarditis, spiking fevers, hepatosplenomegaly and lymphadenopathy overshadow the joint symptoms. The joint involvement may be completely absent or may be a late clinical feature. These patients have leukocytosis, thrombocytosis and high inflammatory markers simulating a septic focus. In our patient, the accurate diagnosis was complicated by the confounding presence of multiple cystic lymphatic lesions. Treatment of SOJIA is challenging. Oral steroids, NSAID’s, methotrexate, etanercept and the newer anakinra have been used with varying success. Our case underlines the importance of considering a diagnosis of systemic onset JIA in a febrile toddler even in the absence of overt joint involvement.

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In einer retrospektiven Studie über den Zeitraum von 1990-2000 wurden in der Klinik für Kinderchirurgie des Universitätsklinikums Charité, Berlin die Kinder untersucht, bei denen eine lymphatische Malformation aufgetreten war. Es ergaben sich insgesamt 18 Patienten. Parallel wurden aus der Klinik für Gynäkologie und Geburtshilfe, Abteilung für Pränatale Diagnostik und Therapie des Universitätsklinikums Charité, Berlin retrospektiv für den oben genannten Zeitraum 31 Patientinnen untersucht, bei deren Feten ein zystisches Nackenhygrom im Verlauf der Schwangerschaft diagnostiziert worden war. Ziel der Arbeit war es, die geeigneteste Therapieform in der Behandlung von lymphatischen Malformationen im Kindesalter in besonderen Hinsicht auf die Prognose, Rezidivfreiheit und Spätergebnisse zu untersuchen. Parallel galt es zu evaluieren, ob in Anbetracht der pränatalen Diagnostizierbarkeit lympahtischer Malformationen und dem postnatal sich daraus ergebenden Krankheitsverlauf eine Schwangerschaftsunterbrechung in Erwägung gezogen werden sollte. / A ten year retrospective study of 18 children with lymphatic malformation and 31 pregnant women with fetal cystic hygromas was carried out at the Department of Pediatric Surgery and the Department of Obstetrics and Gynaecology at the University Hospital Charité, Berlin. The objective of this study was to analyze the most effective therapy for lymphatic malformation in children with regard to the children prognosis, the recurrences after treatment and the long-time-follow-up. Another object to analyze was the point of prenatal diagnosis of fetal cystic hygromas and the adverse fetal outcome.