Meconium pseudocyst revisited

Meconium peritonitis is defined as a sterile chemical or foreign-body peritonitis that is caused by escape of meconium from the intestinal tract into the peritoneal cavity during the fetal or perinatal period. Although meconium peritonitis is indicative of intrauterine perforation of the intestine, it may occur as early as the 4th to 6th month of INTRA NATAL life and as late as several hours after birth. It can be classified into three pathological variations: fibro-adhesive; cystic and generalized. The cystic type has a meconium filled pseudocyst that may rupture in the peritoneal cavity. Intra-abdominal calcification is pathognomonic for the diagnosis. Here, author reported a classical case of meconium peritonitis with pseudocyst formation, which was treated successfully conservatively.

Brown Vialetto Van Laere syndrome, a fatal disease with a simple solution: a case series

Brown Vialetto Van Laere (BVVL) syndrome is an extremely rare neuro metabolic disorder postulated to be caused by a defect in riboflavin transporter. The disease is characterized by progressive hearing loss with ataxia and difficulty in swallowing and breathing. The diagnosis of the disease requires great deal of suspicion on the part of treating physician. Here authors present 2 cases of BVVL who presented to us with dysphagia and hearing loss and responded to therapy. Brown Vialetto Van Laere (BVVL) syndrome is an extremely rare neuro metabolic disorder postulated to be caused by a defect in Riboflavin transporter. The disease is characterised by progressive hearing loss with ataxia and difficulty in swallowing and breathing. It is a subset in multiple acyl CoA dehydrogenase deficiency (MADD disorder). Age of onset is generally first to third decade of life. Lower cranial nerve involvement with LMN and UMN signs concomitantly is the striking feature. There is no specific treatment for BVVL except supportive care. Response to high dose riboflavin (20mg/kg/day) has produced promising results but the results may take anywhere from 1 week to 12 months to appear.

Congenital lobar emphysema: a rare cause of respiratory distress in a neonate

Congenital lobar emphysema (CLE) is a congenital condition characterized by distension and air trapping of the affected lobe of the lung. It is one of the causes of infantile respiratory distress, which may require surgical resection of affected lobe. Case characteristics: 3-day-old neonate with ventilation refractory respiratory distress. Imaging was suggestive of decreased lung tissue on the right side with ipsilateral mediastinal shift. Intervention/ outcome: Early surgical lobectomy was done to improve lung functions and the child improved dur to early intervention. Message: An early diagnosis with high index of suspicion helps patients with this rare congenital anomaly. Early intervention is the key to good long-term outcome. More awareness about the entity and treatment options available would greatly help improving the outcome and disease burden.