Comparison of the sacral ratio between children with Hirschsprung disease and non-Hirschsprung

Background: Hirschsprung's disease is a congenital gastrointestinal disorder that is the most common cause of intestinal obstruction in children. One of the factors that influence gastrointestinal disorders is the sacrum bone. The sacral index can review the evaluation of the sacral bone, an instrument that can evaluate the development of the sacral bone in different clinical conditions. This study assessed the value of the sacral ratio in children with Hirschsprung's disease and non-Hirschsprung's disease at Dr. Hospital. Hasan Sadikin Bandung.

Methods: This study was a descriptive-analytic study with a case-control design. Sampling was done retrospectively by collecting medical record data and the enema barium readings from October 2019 to July 2020. The study was conducted at the Department of Radiology Hospital Dr. Hasan Sadikin Bandung from September to October 2020. Data processing uses an unpaired T-test.

Results: The total sample was 58 objects, consisting of 29 with Hirschsprung’s disease and 29 non-Hirschsprung, with an average age of Hirschsprung’s disease was 3.14±3.905 years. In the Hirschsprung’s disease group, the mean sacral ratio value was 0,62 and, and the mean sacral ratio in the non-Hirschsprung group was 0,74. Statistical test results with unpaired T-test showed significant mean differences in the case and control groups (p=0.0001).

Conclusion: The sacral ratio value in children with Hirschsprung’s disease compared to non-Hirschsprung were different significantly. 

A rare case of porokeratosis mibelli in 3-year-old boy

Introduction: Porokeratosis mibelli is a rare, chronic, and slowly progressive genodermatotic characterized by abnormality of keratinization. The clinical manifestations are hyperkeratotic papules or plaques surrounded by a thread-like elevated border that widens centrifugally. Rarity of this case means the science behind it is underdeveloped. Thus, we present this case as an educational vehicle and to further develop the body of knowledge surrounding this disease.

Case report: We reported a 3 years old boy, suspect of porokeratosis mibelli. His mother complained of a few reddish spots and patches that sometimes itchy, healed into white and brownish patches with atrophic centers in the last 5 months before admission. Physical examination: few lesions on the back, waist, left side of buttock, back of the neck, and lateral upper left arm. Hypopigmented and hyperpigmented patches with atrophic centers, and erythematous plaques with central erosion were found. No abnormality of internal organs or laboratory findings. Histopathology examination showed epidermis contains of invagination rete malpighia filled keratin. Slightly thinned stratum germinativum with corneum contains basket weave pattern. Topical therapy with 1% 5-fluorourasil cream twice daily showed mild improvement after 10 days.

Conclusion: Although clinical examinations of the lesions on this patient did not fit the criteria of porokeratosis mibelli, however, the results of histopathology examination supported the diagnosis. Treatment with 1% 5-fluorourasil cream showed slow but progressive healing on the patient