Ultrasonography in Children With Congenital Pyriform Sinus Fistula: Analysis of 31 Cases.

OBJECTIVES: Congenital pyriform sinus fistula (CPSF) is a rare disease that can be easily misdiagnosed. This study investigates the value of ultrasonography in the early diagnosis and treatment of CPSF in children. METHODS: Clinical features and ultrasonography images of 31 CPSF pediatric patients confirmed by operation were retrospectively analyzed, different sonographic features during the infection period and the quiescence period were summarized and the consistency test of ultrasonic recognition and diagnosis between observers was conducted. RESULTS: In this study, 25 CPSF children had thick-walled cystic masses during the infection period, and cystic masses of 8 cases showed gas echo inside; after the modified valsalva maneuver, gas echo was found in another 5 cases. The detection rate of gas can be enhanced through the modified valsalva maneuver and infants' cry so as to provide an important basis for the diagnosis of pyriform sinus fistula. During the quiescent period of inflammation of 6 cases, fistula can be completely shown, and the wall structure has not been completely destroyed, so that the running position of fistula can be clearly seen. Ultrasonography boasted a good inter-observer consistency in identification and determination (Kappa:0.799-0.857; P<0.001). CONCLUSION: Ultrasonography could clearly reveal the position and direction of CPSF fistula. Different ultrasonic characteristics in different periods could provide relevant information for the selection of clinical operation timing and evaluate the post-operative effects.

Diagnosis of neonatal neurofibromatosis type 1: a case report and review of the literature.

BACKGROUND: Neurofibromatosis Type 1 (NF1) is a rare genetic disorder characterized with the development of multiple benign tumors on the nerves and skin. CASE PRESENTATION: This report described a neonatal case with a large mass observed on the left side of the maxillofacial and cervical region at birth. Meantime, multiple cafe-au-lait macules (CALMs) were seen on the trunk and both lower extremities. CONCLUSIONS: In this case, the clinical features of the rare NF1 neonate are discussed along with its ultrasound findings.

Ultrasonographic analysis of Langerhans cell histiocytosis in children: a report of 55 cases.

OBJECTIVE: To explore the value of ultrasonography in the diagnosis and treatment of Langerhans cell histiocytosis (LCH) in children. METHOD: The clinical and imaging features of 55 children with pathologically confirmed LCH were retrospectively analyzed. RESULTS: Thirteen patients had bone LCH and 42 had multisystem LCH. Among the 13 cases of bone LCH, 8 cases involving the skull and 2 involving the scapula were characterized by osteolytic bone destruction, 1 case involving the clavicle and 1 involving the iliac bone showed multiple irregular bone destruction, and 1 case involving the tibia showed local hypoechoic cortical bone. Soft tissue echo filling was present in the local areas of bone destruction. Among the 42 cases of multisystem LCH, 33 involved the bone, 35 showed an enlarged liver, 15 involved the spleen, 2 involved the pancreas, 3 involved the lung, 3 involved the thymus, and 21 affected the lymph nodes in different regions. CONCLUSIONS: Ultrasonography of the flat bones in children with LCH mainly showed punched-out osteolytic bone destruction. Long bone lesions were characterized by fan shell changes in the endosteum of long bones, and some also showed bone destruction. Multisystem LCH can affect almost any organ. Ultrasonography is important for early diagnosis.