Renal lymphangiectasia

Renal lymphangiectasia (RL), also known as renal lymphangiomatosis, is a rare benign condition characterized by ectasia of peripelvic, perirenal and intrarenal lymphatic vessels. It accounts for approximately 1% of all lymphangiomas.1 The pathophysiology of renal lymphangiectasia remains unclear. However, it is hypothesized that the failure of draining into the larger retroperitoneal lymphatic channels causes abnormal cystic dilatation of the peripelvic, perirenal and intrarenal lymphatic ducts.2 RL can manifest at any age, with males and females equally affected. Lymphangiectasia can involve both kidneys, albeit unilateral involvement is common. Patients may be asymptomatic or present with flank pain, abdominal distention, lower limb oedema, hematuria and hypertension. Extreme presentation such as renal failure has also been documented. Due to the classical imaging features, computed tomography (CT) scan becomes the best diagnostic modality for the diagnosis, which can be confirmed by aspiration of chylous fluid.3 In the pediatric patient, the differential diagnosis includes cystic diseases of the kidney, nephroblastomatosis, and hydronephrosis with perinephric urinoma. Depending on the presentation, the management varies, including conservative, percutaneous aspiration, marsupialization, and nephrectomy.4 We describe gross and microscopic features of renal lymphangiectasia in a 4-year-old male child who presented with gradually progressive abdominal distension for one month with accompanying vague flank pain and fatigue. No hematuria or bladder bowel complaints were noted. Family and perinatal history were not significant. An ill-defined large mass of approximately 20x10cm occupying the left flank and hypochondrium was palpable on bimanual palpation. The renal function test was within normal limits for this age. Ultrasonography (USG) abdomen revealed bilateral multi-loculated, anechoic, cystic lesions in the perirenal and parapelvic region. Raised cortical echoes indicated the loss of corticomedullary distinction. CT scan revealed bilateral non-enhancing multiloculated cystic collection in the perirenal and parapelvic locations. Exploratory laparotomy showed a well-defined thick-walled sac of 20x15cm encasing the left kidney. Intraoperatively, the left kidney was hard to feel, and the architecture was distorted. A Left nephroureterectomy was done and submitted for histopathological evaluation. The kidney with perinephric fat measures 15x9x5.5cm, and the ureter was 5cm in length. The perinephric fat showed multiple collapsed cysts that enclosed the whole kidney ranging in size from 4 to 8cm in the largest dimension. The cut surface of the kidney exhibited well-demarcated multiloculated cysts in the cortex and medulla. The cysts are of variable size measuring 0.5 to 4cm in maximum dimension and shows thickness of 0.2cm and intervening thin septa (Figure 1A). The luminal aspect appeared smooth, contained brownish serous fluid, and did not show any papillary excrescences or growth. The adjacent spared kidney showed indistinct cortico-medullary junction and focal thinning of the cortex (0.3 to 0.4cm). Microscopically, numerous and variably sized cystic spaces were present in the cortex, medulla, pelvis, and perinephric fat (Figure 1B). Glomeruli and tubules in the intervening renal parenchyma showed no abnormal pathology on microscopy; however, the interstitium appears oedematous containing dilated lymphatic channels (Figure 1C). Cystic spaces were lined by a discontinuous layer of flat endothelial cells as highlighted by D2-40 (Figure 1D), CD31, and CD34 immunostains. Neuromatoid hyperplasia was seen in the sections from renal pelvis. Following clinical-radio-pathological correlation, a diagnosis of renal lymphangiectasia was offered. The patient was on close imaging follow-up to keep track of the right kidney

Cystic adenomatoid malformation of the lung: A diagnostic dilemma

Congenital cystic adenomatoid malformation (CCAM) of the lung is an uncommon anomaly that arises from excessive disorganised proliferation of tubular bronchial structures excluding the alveoli. These are believed to represent focal pulmonary dysplasia because skeletal muscle may be identified from within the cyst wall. This report describes a case of an infant operated for presumed diagnosis of congenital diaphragmatic hernia. Diaphragm was found to be normal and further investigations revealed cystic mass in the lower lobe of the left lung. Thoracotomy was done to resect the lesion that revealed a type II CCAM on histopathological examination. The case stresses the need for better clinical examination and advanced radiological investigations in doubtful cases

Isolated cleft sternum: Neonatal surgical treatment

Isolated cleft sternum is a rare congenital defect of the anterior chest wall and is the result of failed ventral midline fusion of sternal bands. We present two cases operated 18 and 24 days after birth with satisfactory results. Surgery is indicated to protect the heart and mediastinal contents

Case Report - Isolated cleft sternum: Neonatal surgical treatment

Isolated cleft sternum is a rare congenital defect of the anterior chest wall and is the result of failed ventral midline fusion of sternal bands. We present two cases operated 18 and 24 days after birth with satisfactory results. Surgery is indicated to protect the heart and mediastinal contents

Delphi Method Analysis and Consensus of Prevalent Distinctive Practices for Biliary Atresia Management after Kasai Portoenterostomy

Background: Extrahepatic biliary atresia (BA) is seen in infants, with an incidence of 1 in 15,000 live births. The presentation is progressive jaundice, dark-colored urine, and clay-colored stools. Kasai portoenterostomy (KPE) is the commonly performed surgical procedure in these patients. Postoperatively, phenobarbitone, ursodeoxycholic acid (UDCA), steroids, and other drugs are given to improve bile drainage and prevent inflammation and fibrosis. However, a definitive protocol regarding the need for different drugs, dosage, and duration varies across individual surgeons and centers. No universally accepted protocol exists for postoperative management after KPE. Aim: The aim of this study was to know the prevailing postoperative management of BA by subject experts and use the Delphi process to know if the experts want to change their practice based on the results from the survey. Material and Methods: A questionnaire was made after discussing with two experts in the field of BA. The questionnaire was mailed to 25 subject experts. The first survey data were analyzed and shared with all responders. In the second survey, change in the management based on the results from the first survey was assessed. Results: The Delphi questionnaire was answered by 17 experts. Postoperatively, prophylactic antibiotics are prescribed for 6–12 weeks by around 40% and >12 weeks by 30% of respondents. Phenobarbitone is prescribed for 15 KPE per year. The second survey noted that a significant percentage of responders want to change their practice according to consensus. Conclusion: From our Delphi survey, an overview of the postoperative management of BA could be made. However, multicentric studies are required for uniform protocol on the postoperative management of BA

Retrocaval ureter: Clinical images

Retrocaval ureter (RCU) is a rare congenital anomaly with the ureter looping around inferior vena cava (IVC). We present the case of 10-year-old boy. Diagnosis was confirmed with ultrasound and CT. He underwent retroperitoneoscopic uretero-ureterostomy

Does all small bowel intussusception need exploration?

Small bowel intussusception (SBI) in infants and children are ususally associated with a lead point. With increased use of radiological investigations, more idiopathic SBIs are identified. As reduction by hydrostatic or air enema are less successful in these cases, most of them require surgical exploration in children. However, now many cases of SBI have been found to reduce spontaneously. We report two cases of SBI with spontaneous reduction and review the literature for the management guidelines