Osseous Rosai-Dorfman disease of tibia in children:A case report

BACKGROUND Rosai-Dorfman disease(RDD),or sinus histiocytosis with massive lymphadenopathy,is a benign histiocytic disorder.Extranodal involvement is common,occurring in>40%of patients,but bone involvement occurs in<10%of cases.In addition,primary bone RDD is extremely rare.The majority of patients are adolescents and young adults,and the mean age at onset is 20-years-old.CASE SUMMARY We report an 8-year-old Chinese girl who presented to our hospital with an insidious onset of swelling and pain in the middle shaft of her right tibia for 4 mo.We performed total surgical resection of the right tibia lesion and allograft transplantation.A good prognosis was confirmed at the 6 mo follow-up.Pain and swelling symptoms were totally relieved,range of motion of her right knee and ankle returned to normal,and there was no clinical evidence of lesion recurrence at last follow up.Our case is the second reported case of osseous RDD without lymphadenopathy in the shaft of the tibia of a child.CONCLUSION Extranodal RDD is a rare disease and can be misdiagnosed easily.Lesion resection and allograft transplantation are an option to treat extranodal RDD in children with good short term result.Pediatric orthopedist should be aware of this rare disease,especially extranodal involvement.

Early surgical intervention for structural infantile spasms in two patients under 6 months old:a case report

Background:Infantile spasms(IS)are the most common childhood epileptic encephalopathy.Focal cortical dysplasia(FCD)and gray matter heterotopias(GH)are common structural causes of IS.The recommended first-line treatment for IS patients with structural causes is surgical intervention,according to the International League Against Epilepsy(ILAE)commission guidelines.However,there is currently no consensus on appropriate timings of surgery.Case presentations:Two structural IS cases are presented here:one was caused by FCD,and the other by GH.Both patients exhibited recurrent seizures at the age of 2 months,had poor responses to various antiepileptic drugs(AEDs)and displayed severe mental and motor developmental retardation.Seizure types included focal seizures and spasms.Brain magnetic resonance imaging showed abnormal gray signal or suspicious FCD lesions that coincided with the origin of the focal seizures.The patients underwent lesion resection before the age of 6 months.Follow-up observation showed that seizures of both patients were completely controlled several days after the surgery.All AEDs were gradually reduced in dosage within 1 year,and the mental and motor development almost returned to normal.Conclusion:Early resection of lesions in structural IS patients has benefits of effectively controlling convulsions and improving developmental retardation.Infants at several months of age can well tolerate craniotomy,and their cognitive development is more likely to return to normal after early surgery.