Role of oral sildenafil in neonates with persistent pulmonary hypertension of newborn

Persistent pulmonary hypertension of newborn is a devastating condition and leads to morbidity and mortality. Even after the increasing use of' NO, ECMO and HFO many patients succumb to death. About 50% of the patients either have rebound hypertension or do not respond to the treatment. Hence the role of phosphodiesterase inhibitors (sildenafil) need to be evaluated. Methods is authors report a retrospective case series of 24 patients with PPHN admitted in NICU and treated with oral sildenafil. Results sildenafil was started on all patients on a mean of 1.46 days and stopped on mean of 3.8 days. Initial fio2 was 100%, which after starting sildenafil decreased gradually to 50% on mean of 10 days. Average length of stay in NICU was 20 days. 17(70.8%) patients survived whereas 7 (29%) expired No improvement in oxygen Index after 48 hours (p<0.05) was the independent predicting risk factor for PPHN related mortality in the expired patients.Conclusion are oral sildenafil can be a used in conjunction with other treatment modalities for PPHN especially in resource limited settings.

A rare case of Poland: Mobeius syndrome in an infant

Mobius syndrome is a rare condition of unclear origin, characterized by a unilateral or bilateral congenital facial weakness with impairment of ocular abduction, which is frequently associated with limb anomalies. Poland Syndrome is a rare condition that is evident at birth (congenital). Associated features may be extremely variable from case to case. However, it is classically characterized by absence (aplasia) of chest wall muscles on one side of the body (unilateral) and abnormally short, webbed fingers (symbrachydactyly) of the hand on the same side (ipsilateral). In those with the condition, there is typically unilateral absence of the pectoralis minor and the sternal or breastbone portion of the pectoralis major. In females, there may be underdevelopment or absence (aplasia) of one breast and underlying (subcutaneous) tissues. In some cases, associated skeletal abnormalities may also be present, such as underdevelopment or absence of upper ribs; elevation of the shoulder blade (Sprengel deformity); and/or shortening of the arm, with underdevelopment of the forearm bones (i.e., ulna and radius). Other associated abnormalities may include dextrocardia, diaphragmatic hernia and renal anomalies etc. Poland Syndrome affects males more commonly than females and most frequently involves the right side of the body. The exact cause of the condition is unknown. The combination of Poland-Mobius syndrome is rare, with an estimated prevalence 1:500 000.