An Unusual Antenatal Presentation of a Mature Pericardial Teratoma Masquerading as Congenital Pulmonary Airway Malformation.

We report an antenatal presentation of a huge pericardial mature teratoma that was referred as congenital pulmonary airway malformation (CPAM) in the late third trimester of pregnancy. Initial ultrasound evaluation revealed a huge predominantly cystic lesion with mixed echogenicity in the left hemithorax. A provisional diagnosis of pleural tumor was considered in view of previous scans at 20‒28 weeks being normal and associated pleural effusion. Magnetic resonance imaging of the fetus reported the lesion to be CPAM which was supported by postnatal computed tomographic imaging done on day 2 of life. However, intraoperatively, the lesion was found to be of pericardial origin which on subsequent histopathological examination was confirmed to be mature teratoma. We recommend considering potential differential diagnosis other than CPAM, especially when the lesion is found for the first time in the late third trimester.

Consensus on the Management of Posterior Urethral Valves from Antenatal Period to Puberty.

The need for successful management of posterior urethral valves always captivates the minds of pediatric surgeons. Its success, however, depends on several factors ranging from prenatal preservation of upper tracts to postoperative pharmacological compliance. Regardless of measures available, some cases do not respond and progress to end stage. The management depends on several issues ranging from age and severity at presentation to long-term follow-up and prevention of secondary renal damage and managing valve bladder syndrome. This article is based on a consensus to the set of questionnaires, prepared by research section of Indian Association of Paediatric Surgeons and discussed by experienced pediatric surgeons based in different institutions in the country. Standard operating procedures for conducting a voiding cystourethrogram and cystoscopy were formulated. Age-wise contrast dosage was calculated for ready reference. Current evidence from literature was also reviewed and included to complete the topic.

Invasive mycosis due to species of Blastobotrys in immunocompromised patients with reduced susceptibility to antifungals.

Cases of invasive mycosis due to Blastobotrys serpentis and B. proliferans identified by sequencing in a preterm patient and a rhabdomyosarcoma patient, respectively, are reported. Both species revealed elevated fluconazole and echinocandin MICs by the CLSI broth microdilution method. Additionally, B. serpentis exhibited high amphotericin B MICs, thus posing serious therapeutic challenges.

Laparoscopic pyeloplasty for children with pelvic ureteric junction obstruction: an institutional experience.

AIMS AND OBJECTIVES: To give a report of 36 consecutive children who underwent laparoscopic Anderson-Hynes dismembered pyeloplasty by a single lead surgeon. PATIENTS AND METHODS: The diagnosis of pelviureteric junction obstruction was firmly established in all patients based on history, clinical examination, renal sonography and scintigraphy. Transperitoneal laparoscopic Anderson-Hynes pyeloplasty was performed in all the patients. Age at surgery, duration of operation, complications and outcome were documented. Children were followed up for symptoms, and diuretics renography was repeated at 3 months. RESULTS: A total of 36 children 5 months to 11 years (25 boys and 11 girls) under- went laparoscopic Anderson- Hynes pyeloplasty over a 4- year period. Mean age at surgery was 41 months (range 7 to 144). Seventeen (47%) cases were antenatally diagnosed. The mean operating time was 247 min. No patient required blood transfusion, and there were no intra-operative complications. The mean postoperative hospital stay was 5.8 days. There were 7 postoperative complications including urinary tract infection (n=6) and shoulder pain (n=1). The symptoms improved in 32(89%) children. There were 3 conversions, 2 due to non rotated kidney and one due to double right moiety. One child had failed pyeloplasty with deteriorating renal function. He had a redo open pyeloplasty. The mean split renal function before surgery and at follow up diuretic scan was 36.2 vs 42.1, P=0.001. The mean follow up period was 30 months. CONCLUSION: Laparoscopic Anderson-Hynes pyeloplasty is safe and effective in the management of children with pelvi-ureteric junction obstruction.

ATF5, a possible regulator of osteogenic differentiation in human adipose-derived stem cells.

The regulatory pathways involved in maintaining the pluripotency of embryonic stem cells are partially known, whereas the regulatory pathways governing adult stem cells and their "stem-ness" are characterized to an even lesser extent. We, therefore, screened the transcriptome profiles of 20 osteogenically induced adult human adipose-derived stem cell (ADSC) populations and investigated for putative transcription factors that could regulate the osteogenic differentiation of these ADSC. We studied a subgroup of donors' samples that had a disparate osteogenic response transcriptome from that of induced human fetal osteoblasts and the rest of the induced human ADSC samples. From our statistical analysis, we found activating transcription factor 5 (ATF5) to be significantly and consistently down-regulated in a randomized time-course study of osteogenically differentiated adipose-derived stem cells from human donor samples. Knockdown of ATF5 with siRNA showed an increased sensitivity to osteogenic induction. This evidence suggests a role for ATF5 in the regulation of osteogenic differentiation in adipose-derived stem cells. To our knowledge, this is the first report that indicates a novel role of transcription factors in regulating osteogenic differentiation in adult or tissue specific stem cells.

A simple and safe technique for trocar positioning in vesicoscopic ureteric reimplantation.

PURPOSE: Anchoring of the urinary bladder to the anterior abdominal wall is essential to prevent perivesical gas leak and dislodgement of trocar. Existing techniques have the risk of going through the peritoneal cavity and injuring the bowel. Our aim was to find a safe technique to anchor the bladder wall to the abdominal wall. MATERIALS AND METHODS: Nineteen cases were studied prospectively. A curved urethral dilator was introduced and made to point on the anterior abdominal wall. Abdominal wall was incised over the tip of the dilator till the bladder was seen which was then sutured to the anterior abdominal wall. The bladder was then incised and the trocars were railroaded on the dilator into the bladder. Cohen's reimplantation was then done. RESULTS: Out of the 19 cases, conversion to open was done in two early cases. In one case, the trocar got pulled out, but was easily reintroduced. Mean operating time was 210 min. Mean follow up was 20.9 months. There was a resolution of reflux in 13, reduction in the grade of reflux in 1 and no failures. In five cases, post op MCU is awaited. CONCLUSION: This technique of fixing the bladder to the anterior abdominal wall is safe and easy to perform. It is recommended while doing vesicoscopic reimplantation.

Deep learning model to predict the need for mechanical ventilation using chest X-ray images in hospitalised patients with COVID-19.

OBJECTIVES: There exists a wide gap in the availability of mechanical ventilator devices and their acute need in the context of the COVID-19 pandemic. An initial triaging method that accurately identifies the need for mechanical ventilation in hospitalised patients with COVID-19 is needed. We aimed to investigate if a potentially deteriorating clinical course in hospitalised patients with COVID-19 can be detected using all X-ray images taken during hospitalisation. METHODS: We exploited the well-established DenseNet121 deep learning architecture for this purpose on 663 X-ray images acquired from 528 hospitalised patients with COVID-19. Two Pulmonary and Critical Care experts blindly and independently evaluated the same X-ray images for the purpose of validation. RESULTS: We found that our deep learning model predicted the need for mechanical ventilation with a high accuracy, sensitivity and specificity (90.06%, 86.34% and 84.38%, respectively). This prediction was done approximately 3 days ahead of the actual intubation event. Our model also outperformed two Pulmonary and Critical Care experts who evaluated the same X-ray images and provided an incremental accuracy of 7.24%-13.25%. CONCLUSIONS: Our deep learning model accurately predicted the need for mechanical ventilation early during hospitalisation of patients with COVID-19. Until effective preventive or treatment measures become widely available for patients with COVID-19, prognostic stratification as provided by our model is likely to be highly valuable.

Experience with laparoscopy-assisted retroperitoneal pyeloplasty in children.

PURPOSE: To describe a laparoscopy-assisted retroperitoneal pyeloplasty (LARP) and results of initial experience. METHODS: Port placement used by Farhat in retroperitoneal-assisted laparoscopic pyeloplasty was modified for better cosmetic results. Surgery was done using 2-cm incision for 5-mm camera port and two 3-mm working ports. Dissection was done anterior to the kidney. The ureteropelvic junction was brought out through the 2-cm trocar site and the pyeloplasty was performed extracorporeally. Between January 2004 and February 2008, a total of 39 kidneys in 38 children with mean age of 4.1 months underwent LARP. The operative time, hospital stay, functional outcome and follow-up renogram studies were reviewed. RESULTS: The mean operative time was 147 min. 2-cm incision was extended in one patient with malrotated kidney. There was improvement in function in 37 (95%) with no failure. The mean split renal function, preoperative and at follow-up were 35.7 and 44.2%, respectively (P = 0.000). The mean glomerular filtration rate (ml/min), preoperative and at follow-up were 27.4 and 39.1%, respectively (P = 0.000). Mean follow-up period was 24 months. CONCLUSION: LARP is safe in treating UPJ obstruction in infants. It is recommended especially in small babies where laparoscopic pyeloplasty is difficult.

Role of alpha adrenergic blocker in the management of posterior urethral valves.

PURPOSE: To evaluate the effect of Terazosin (alpha1 adrenergic blocker) on bladder emptying in children with posterior urethral valves. MATERIALS AND METHODS: Forty-two children with significant post void residual urine after valves ablation were placed on Terazosin ranging from 0.25 to 2 mg. Post void urine at the commencement and at follow up was monitored with abdominal ultrasound. RESULTS: Post void residual urine significantly reduced in 40 patients (95%) who were put on Terazosin. Mean pretreatment PVR was 15.7 ml and mean PVR at the last follow up was 2.4 ml (P = 0.000). This was a reduction of 85% in the pretreatment post void residual urine volume. All the patients had improvement in urinary stream. One patient reacted to Terazosin with hypotension necessitating its withdrawal. Mean follow up was 17 months. CONCLUSION: Terazosin has proved to be safe and results in significant improvement in bladder emptying in our patients with posterior urethral valves. Randomized controlled trial and long-term follow up are necessary to further define the role of alpha1 adrenergic blocker therapy in children with posterior urethral valves. This study will become the justification for such a study.

Plexiform Neurofibroma of Clitoris.

The most frequent genital presentation of neurofibromatosis in females is clitoromegaly. We report a case of a 5-year-old girl with neurofibromatosis type 1 with clitoral plexiform neurofibromatosis. Clitoroplasty was done, and the histopathology confirmed the diagnosis. Though rare, plexiform neurofibroma of clitoris should always be considered as a differential diagnosis in children with clitoromegaly before embarking on detailed investigations.

A simple and safe technique for pneumatic reduction of intussusception.

BACKGROUND: Pneumatic reduction has a higher success rate and lower incidence of complications compared to barium enema and hydrostatic reductions. What is deterrent to its common use is the cumbersome technique. Our aim is to develop a simple technique that can be used in any hospital with locally available facilities. METHODS: An intercostal drainage bottle and an enema can were used to pass air into the rectum at a controlled pressure determined by the height of the enema can. Water running in from the enema can displaces the air in the intercostal drainage bottle into the rectum effecting the reduction of intussusception. RESULTS: This system was tried in 12 patients. Successful air delivery was obtained in all cases. In two cases, the intussusception could not be reduced. Laparotomy revealed these to be ileoileal intussusceptions. CONCLUSION: The technique described is easy to assemble, safe and effective. We recommend it for regular use in pneumatic reduction of intussusception.

Congenital Cystic Adenomatoid Malformation: A Tertiary Care Hospital Experience.

INTRODUCTION: Congenital Cystic Adenomatoid Malformation (CCAM) is an uncommon developmental deformity affecting the terminal respiratory structures. It is characterized by broncho pulmonary foregut malformations. The reason behind it is an arrest in lung development between 4th and 7th week of fetal life. AIM: The present study was conducted to assess the clinical and radiological profile and also to study the role of surgical intervention in patients with CCAM. MATERIALS AND METHODS: All patients with clinical suspicion or provisional diagnosis of CCAM were included in the study. A clinical questionnaire was prepared to collect data. Computed Tomography (CT) chest with High Resolution Computed Tomography (HRCT) was done for all the patients. Patients were assessed by paediatric surgeon and eligible patients were operated. The procedure conducted was usually open thoracotomy under general anaesthesia. The affected lobes were removed and specimens were sent for histopathological analysis. All included patients were followed up prospectively to find out about their current level of health. Via telephonic interview they were asked about their overall growth, quality of life, activity, rate of respiratory infections and requirement of hospital admission. RESULTS: Total 15 patients with diagnosis of CCAM were included in the study. Of them, 8 (53.3%) were male. The commonest presentation was cough 13(86%), breathing difficulty 11(73%), fever 9(60%), recurrent pneumonia 4(26%), hypoxia requiring oxygen supplementation 6(40%), others 2(12%). Thirteen patients required surgical intervention and underwent lobectomy. There were 2 cases of type I, one each of type II and III, 3 case of type IV while 5 were intermediate type. There was no procedure related mortality. The median duration of hospital stay and all were successfully discharged with median duration of stay 11±16 days. CONCLUSION: The study concludes that if recognized early, surgical removal of affected lung prevents the complications like recurrent pulmonary infections. The surgery is well tolerated without any post-operative mortality or morbidity.

Thoracoscopic repair of eventration of diaphragm.

pen thoracotomy and plication of eventration of diaphragm leads to hypoventilation due to pain and lung contusion due to retraction. We present two cases, 8 month and 4 years old; in whom plication was done thoracoscopically. Both had smooth recovery, early extubation and excellent cosmetic result.

A safer way of suturing in Foker's technique.

BACKGROUND/PURPOSE: Foker's technique allows esophageal lengthening facilitating end to end anastomosis in long gap esophageal atresia. The problem faced with this technique is that the traction sutures cut through the tissues leading to re-operations. Our aim was to find a technique of suturing that will prevent the sutures from cutting through the esophagus. METHODS: After dissection of the upper and lower esophageal pouches, purse string sutures were placed, two each on both pouches. Clips were applied at the ends of both the pouches. Sutures were brought out on the posterior chest wall and traction applied. This was tried in a total of three cases. Case 1 was a newborn with pure esophageal atresia, Case 2 was an eighteen month old child with cervical esophagostomy and gastrostomy, and Case 3 had esophageal atresia with distal fistula. Two cases were done thoracoscopically and the third one by thoracotomy. RESULTS: In all three cases sutures held and lengthening could be obtained. In the first case it took twelve days, in the second case six days, and in third case eight days for the ends to come together. CONCLUSION: This modification of traction sutures is simple and reduces the risk of suture disruption.

An unusual variant of urethral duplication: an addition to the Effman classification.

A rare association of horseshoe kidney with pyelic fusion with crossed single ureter along with a rare unusual variant of complete urethral duplication is described. We review the available literature on this rare association and present its management.

Laparoscopic inguinal hernia repair in children: a single-centre experience over 7 years.

BACKGROUND: To evaluate 209 consecutive children who underwent laparoscopic hernia repair over a 7-year period. Technical details and clinical results are reported. MATERIALS AND METHODS: A total of 284 open internal rings were closed laparoscopically in 209 children (142 boys and 67 girls, aged 30 days to 15 years, mean 44 months). One 5-mm and two 3-mm instruments were used to access the peritoneal cavity. A 270° anterolateral peritoneal incision was made. The internal inguinal ring was closed with a nonabsorbable suture. RESULTS: There were no significant intraoperative complications. Length of procedure ranged between 15 and 65 minutes with a mean of 30 minutes. Postoperative hospital stay ranged from 1 to 9 days. Thirty children who presented with a right-sided hernia and 23 with a left hernia (total of 53) were found to have a patent contralateral internal ring on laparoscopy. Mean follow-up was 30 months. There were 2.4% hernia recurrences and cosmesis was excellent. CONCLUSIONS: Laparoscopic hernia repair in children can be a routine procedure with increasing experience and better learning curve of surgeons. There is clear visualization of structures and vas remains untouched. The recurrence rate is comparable to that of the traditional open approach with a superior cosmetic result.

Posterior urethral valve.

BACKGROUND: Posterior urethral valve (PUV) is a significant cause of morbidity, mortality and ongoing renal damage in children. It accounts for end-stage renal disease in a proportion of children. This article aims at highlighting the current trend in the management of boys with posterior urethral valve. DATA SOURCES: PubMed/Medline and bibliographic search for posterior urethral valve was done. Relevant literatures on presentation, pathology, evaluation, management and outcomes of PUV were reviewed. RESULTS: PUV which is increasingly diagnosed prenatally presents a spectrum of severity. The varied severity and degree of obstruction caused by this abnormality depend on the configuration of the obstructive membrane within the urethra. The decision to intervene prenatally is dependent on gestational age, amniotic volume, and renal function of fetal urine aspiration. Identification of the patients who may benefit from early intervention remains inconclusive. Endoscopic ablation of the valve is the gold standard of treatment but use of Mohan's valvotome and other modalities are invaluable in developing countries where endoscopic facilities are limited. Proximal urinary diversion may result in poor bladder compliance and should be reserved for patients with persisting or increasing upper urinary tract dilatation, increasing serum creatinine or inappropriate instruments. The behavior of the bladder and its subsequent management after valve ablation may influence the long-term renal outcome in PUV patients. CONCLUSIONS: The care of children with PUV continues to improve as a result of earlier diagnosis by ultrasound, developments in surgical technique and meticulous attention to neonatal care. The ultimate goal of management should be to maximize renal function, maintain normal bladder function, minimize morbidity and prevent iatrogenic problems.

Intravesical pressure: A new prognostic indicator in congenital diaphragmatic hernia.

AIMS: To evaluate the usefulness of intravesical pressure as a prognostic indicator in congenital diaphragmatic hernia. MATERIAL AND METHODS: In 25 cases, bladder pressure was measured intraoperatively during repair. RESULTS: Cases were divided into three groups according to the intravesical pressure. Group 1: pressure <10 cm (n.9), Group 2: 10-15 cm (n.11) and Group 3: >15 cm (n.5). Number of ventilated days was tabulated against these groups. Median number of ventilated days for Group 1, with the lowest pressure, was 3 days, while that for Group 2 was 5 and for Group 3, with the highest pressure, was 10 days. This was significant, with a P-value of 0.016. CONCLUSION: Measurement of intravesical pressure is a reliable prognostic indicator in newborns with congenital diaphragmatic hernia. It also helps in predicting postoperative ventilatory requirement.