A 4 month-old female presented with decreased movement of the right lower extremity. Exam revealed a flexed, abducted right leg and pain with passive motion. CBC, inflammatory markers and CK were normal and the patient was afebrile. Initial xrays and ultrasound of the hip and femur were unremarkable. An MRI was consistent with severe myositis of the iliopsoas muscle. She was admitted, treated with clindamycin IV and discharged 6 days later on oral clindamycin. The patient re-presented 2 days later due to lack of improvement. Repeat MRI showed persistence of the myositis. Discussion with Radiology, Oncology, Rheumatology, Neurology and Infectious Disease concluded that given her age, exam and imaging, infectious myositis was still most likely. Antibiotics were switched to vancomycin without clinical improvement; therefore an ultrasound-guided needle-biopsy of the psoas was pursued, revealing muscle edema and mild inflammation. Tissue bacterial and fungal cultures were negative. Physical exam remained unchanged, but given the absence of other signs of infection, vancomycin was stopped after 7 days. At that time, mild anemia and thrombocytopenia were noted; however, Coombs testing, liver function tests, lactate dehydrogenase and uric acid were normal, and the counts improved on a repeat CBC. A multi-disciplinary family meeting was held, and the decision was made to discharge the patient home with close outpatient follow-up.
On follow-up, she was found to have worsening thrombocytopenia and later developed a violaceous discoloration of the perineal area, as well as mild coagulopathy, consistent with Kasabach-Meritt phenomenon. MRI revealed localized extension. Vascular Surgery performed an open biopsy and based on histopathology and imaging, a diagnosis of Kaposiform hemangioendothelioma (KHE) was made.
KHE is a vascular, infiltrative tumor usually presenting in infancy. The most common presenting features are an enlarging cutaneous lesion (75%), thrombocytopenia (56%) and musculoskeletal pain (23%). Here, the diagnosis was delayed due to the absence of these most common manifestations on presentation.
This case demonstrates the importance of a patient-centered approach to providing medical care. The family’s participation in the diagnostic process allowed the development of a strong relationship with the health care providers, permitting the family to feel the child was safe even when discharged without a definitive diagnosis. In situations of diagnostic uncertainty, open communication between the family and medical team can prevent feelings of anger or frustration that can easily arise. It also encouraged compliance with outpatient appointments that were crucial to the child’s diagnosis.
In diagnostic dilemmas, the family must remain actively involved with decision making throughout the diagnostic process. This creates trust toward the medical team, even with diagnostic ambiguity, encourages compliance, and enhances patient and family satisfaction.
To cite this abstract:Ktena Y, Herrmann L, Parikh K. Mysterious “Myositis”: Engaging the Family in a Diagnostic Dilemma. Abstract published at Hospital Medicine 2015, March 29-April 1, National Harbor, Md. Abstract 421. Journal of Hospital Medicine. 2015; 10 (suppl 2). https://www.shmabstracts.com/abstract/mysterious-myositis-engaging-the-family-in-a-diagnostic-dilemma/. Accessed July 16, 2019.