Aplasia cutis congenita with rare association and unusual presentation

Aplasia cutis congenita [ ACC] is a congenital absence of skin most commonly affecting scalp. In very rare instances it can involve extremities and trunk. It is usually diagnosed clinically at birth. Most cases are sporadic with a few reports of familial occurrence. Here, we present two cases of which one had scalp involvement correlating to antenatal methimazole intake and the other one had unusual presentation with involvement of deep tissues up to the periosteum of right lower limb. Both cases were managed conservatively and successfully discharged home. But unfortunately were lost to follow-up. ACC is a rare condition with varying degree of severity depending upon the extent and depth of the involvement of the skin and underlying tissues. Mild cases do not need specific intervention while severe cases need to be managed by a multidisciplinary team of pediatrician, dermatologist and plastic surgeon. Methimazole is a known teratogen associated with this condition hence should be avoided in pregnancy

Gullian-barre syndrome with bilateral facial nerve palsy.a rare entity

Bilateral facial nerve paralysis, as compared to unilateral palsyis an extremely rare presentation with serious aetiologies including post infectious like in Guillain-Barre syndrome, infectious including Lyme disease, Infectious Mononucleosis, autoimmune, malignancy [leukaemia] and traumas

We present a rare case of bilateral facial paralysis due to Guillain-Barré syndrome which was successfully managed at Department of Paediatrics, Liaquat National Hospital. Patient presented in June 2015, she is on regular follow-up with last visit in march 2016

Success of locally devised continuous positive pressure ventilation [CPAP] alone or in combination with surfactant therapy in the management of respiratory distress syndrome in preterm neonates

Objective: To study the effect of implementing locally devised CPAP alone or in combination with INSURE protocol [Intubation, Surfactant, Extubation] in preterm neonates with respiratory distress syndrome in a tertiary care hospital

Methods: The study was conducted from August 2012 to August 2014 in Neonatal Intensive Care Unit [NICU] of Liaquat National Hospital, Karachi. Total 113 neonates were enrolled in the study. They were grouped in two categories on the basis of severity of respiratory distress syndrome [RDS]. Infants with clinical evidence of respiratory distress and radiological evidence of patchy reticulogranularpattern were given a trial of bubble CPAPalone. Neonates with severe RDS were given INSURE protocol along with bubble CPAP. The intervention was considered to be successful if respiratory distress improved and the baby could be successfully weaned off from CPAP

Results: In this study 113 neonates were enrolled. Fifty neonates were initially managed with bubble CPAP alone out of which 29 [58%] improved. Neonates with severe RDS were 63 and received bubble CPAP along with INSURE protocol out of which 45 [71.4%] improved

Conclusion: Locally devised Bubble CPAP along with selective surfactant therapy is a safe and effective intervention in the management of respiratory distress syndrome in preterm. It decreases the need for mechanical ventilation and its subsequent complication