Sternal cleft is a very rare congenital anomaly, which can occur as an isolated or associated with other anomalies. We report a patient with a mesh-repaired complete sternal cleft complicated by infection with a multi-resistant Pseudomonas aeruginosa. The patch was surgically removed.
Sternal cleft; Mesh; Infection
Pneumothorax, though rare, is a recognized cause of respiratory distress in the immediate newborn period. It may occur spontaneously or secondary to various underlying lung diseases. Here we share our experience of a neonate with spontaneous pneumothorax with mild to moderate respiratory distress, who recovered completely with conservative management with an oxygen-enriched atmosphere and no surgical intervention.
Intercostal drain; Newborn; Oxygen supplementation; Spontaneous pneumothorax
Clear cell sarcoma of kidney (CCSK) is an aggressive renal neoplasm. We report two boys aged three and half, and three years with CCSK, one of whom had a disease free survival of four years and eight months. These patients were managed with surgery, chemotherapy and radiotherapy. One of the patients discontinued treatment early and lost to follow up. Aggressive multimodality therapy is the keystone to improved outcome.
Clear cell sarcoma; Kidney; Wilm's tumor
Lipomas can occur almost anywhere in the body, but retroperitoneal lipomas are extremely rare. They are slowly growing benign tumors and can attain an enormous size due to silent course of the disease. Total excision of the mass is the treatment of choice and is curative for benign retroperitoneal lipomas. We treated an 11-month-old female patient with giant retroperitoneal lipomas by surgical excision. Histopathology confirmed it as fibrolipoma.
Retroperitoneal lipomas; Liposarcoma; Infant
A neonate with patent urachus (PU) who later developed hypertrophic pyloric stenosis (HPS) is being reported. The newborn was first operated for PU; post-operatively he developed persistent vomiting and radiological workup confirmed HPS. Pyloromyotomy was performed with an uneventful recovery.
Patent urachus; Hypertrophic pyloric stenosis; Neonate
Ureteral tumors are rare and benign tumors are even rarer. Most of such tumors are diagnosed after nephroureterectomy assuming a malignant lesion, but with modern technological advancements like contrast enhanced CT scan or MR urography and ureteroscopic biopsy, benign nature of such tumors can be established preoperatively with an aim to preserve the renal unit. Here we report a case of 10-year old boy who presented with chronic right loin pain. DTPA scan showed complete loss of function of the right kidney. He was diagnosed to have an inflammatory myofibroepithelial polyp of right lower ureter and treated by nephroureterectomy.
Ureteral tumors; Fibroepithelial polyp; Myofibroepithelial polyp; Ureteroscopy
Ingestion of foreign bodies is a common presenting complaint in the pediatric emergency department. We present a case of a child in whom disc battery ingestion was suspected initially. The immobility of the foreign body on few days of conservative management raised the suspicion of two magnets. At operation, two magnets were found in the bowel causing a transmesenteric entero-enteric fistula.
Intestinal perforation; Magnets; Enteric fistula
Gastro-esophageal reflux (GER) is one of the common problems of neonatal intensive care units. Although this condition does not always need to be treated, it occasionally causes clinically serious consequences. Initial management is medical; however, in some cases surgery might be required. A premature neonate with birth weight of 1370 grams was managed in our ICU. The patient was mechanical ventilator dependent due to GER. The patient needed Nissen fundoplication for successfully weaning off the ventilator.
Nissen fundoplication; Gastro-esophageal reflux; Premature
Gossypiboma (GP) or retained surgical sponge is one of the rare surgical complications which can happen despite precautions. Because of the medico-legal issues, it is under-reported. An 8-year-old thalassemic girl, with a history of splenectomy and cholecystectomy, presented to us with acute intestinal obstruction and required surgical exploration. Intraluminal gossypiboma obstructing the ileum was found. Though a rare cause, gossypiboma should also be included in the differential diagnoses of postoperative intestinal obstruction.
Gossypiboma; Retained surgical sponge; Children; Thalassemia; Splenectomy
Any mass on the chest wall may not always be the primary local pathology. A case of lymphoma with an aggressive course may involve the sternum through local invasion and can mimic a chest wall tumour. A 15-year-old boy with mediastinal lymphoma presented with a sternal mass. Partial sternectomy with replacement by methyl methacrylate prosthesis was performed.
Lymphoma; Sternum; Prosthesis
Congenital cutis laxa is a genetically heterogeneous condition presenting in the newborn with loose, redundant skin folds, decreased elasticity of the skin, and general connective tissue involvement. A 2-day-old full term neonate with congenital cutis laxa presented with respiratory distress. Investigations revealed huge hiatal hernia. Patient underwent loose Nissen’s fundoplication. In postoperative period patient developed intussusception which was manually reduced at re-surgery.
Congenital cutis laxa; Hiatal hernia; Postoperative intussusceptions
The wandering spleen is a rare condition characterized by the absence or underdevelopment of one or all of the splenic suspensory ligaments that resulting in increased splenic mobility and rarely torsion. Preventing infarction is the aim of a prompt surgery by splenopexy. We report a case of salvage splenopexy in torsion of a wandering spleen in a three year old girl presented with severe abdominal pain for three days.
Splenic torsion; Wandering spleen; Salvage splenopexy
Lumbo-costo-vertebral syndrome (LCVS) is a set of rare abnormalities involving vertebral bodies, ribs, and abdominal wall. We present a case of LCVS in a 2-year-old girl who had a progressive swelling over left lumbar area noted for the last 12 months. Clinical examination revealed a reducible swelling with positive cough impulse. Ultrasonography showed a defect containing bowel loops in the left lumbar region. Chest x-ray showed scoliosis and hemivertebrae with absent lower ribs on left side. Meshplasty was done.
Congenital lumbar hernia; Hemivertebra; Absent ribs; Meshplasty
We present a 2-day-old female neonate with cleft of the upper sternum, patent ductus arteriosus (PDA), atrial septal defect (ASD), and subglottic hemangioma. Dimensional and Doppler echocardiography, abdominal ultrasonography, and imaging were performed. She underwent a surgical repair of sternal cleft in neonatal life. After 8 months, she developed respiratory distress, apnea due to subglottic hemangioma. She underwent urgent tracheostomy. Subglottic hemangioma was treated with the KTP532 laser.
Sternal cleft; Subglottic hemangioma; PHACES syndrome
Mayer-Rokitansky-Kuster-Hauser Syndrome (MRKHS) is characterized by various abnormalities of paramesonephric duct structures; vaginal aplasia being the commonest anomaly in the spectrum. We report a 17-year-old girl; a case of MRKHS with vaginal agenesis. The cervix was present but atretic; uterus, fallopian tubes and ovaries were normal. There were no associated renal or skeletal defects. Colovaginoplasty was done to bridge the gap between uterus and introitus. Postoperatively, small part of colovaginoplasty flap became necrotic posteriorly, which was ultimately managed by insetting of labial flap.
Mayer-Rokitansky-Kuster-Hauser Syndrome; Vaginal agenesis; Colovaginoplasty