Neoadjuvant Peptide Receptor Radionuclide Therapy in a Rare Case of Pediatric Primary Hepatic Gastrinoma.

ABSTRACT: Gastrinomas with predilection for the adult male population are located in the gastrinoma triangle (>90%). Primary hepatic gastrinoma especially in pediatric population is very rare. Peptide receptor radionuclide therapy has shown benefit in metastatic gastroenteropancreatic neuroendocrine tumors (NETs) with an increasing interest in expanding its role as neoadjuvant treatment modality to improve the surgical candidature in inoperable NETs. There is currently no literature supporting its role in the pediatric NET patients. We present a rare case of a young boy with primary hepatic gastrinoma where 177Lu-based peptide receptor radionuclide therapy in the neoadjuvant setting contributed to his final disease-free status.

Goal-directed fluid therapy guided by plethysmographic variability index versus conventional liberal fluid therapy in neonates undergoing abdominal surgery: A prospective randomized controlled trial.

BACKGROUND: Intraoperative fluid therapy maintains normovolemia, normal tissue perfusion, normal metabolic function, normal electrolytes, and acid-base status. Plethysmographic variability index has been shown to predict fluid responsiveness but its role in guiding intraoperative fluid therapy is still elusive. AIMS: The aim of the present study was to compare intraoperative goal-directed fluid therapy based on plethysmographic variability index with liberal fluid therapy in term neonates undergoing abdominal surgeries. METHODS: A prospective randomized controlled study was conducted in a tertiary care centre, over a period of 18 months. A total of 30 neonates completed the study out of 132 neonates screened. Neonates with tracheoesophageal fistula, congenital diaphragmatic hernia, congenital heart disease, respiratory disorders, creatinine clearance <90 mL/min and who were hemodynamically unstable were excluded. Neonates were randomized to goal-directed fluid therapy group where the plethysmographic variability index was targeted at <18 or liberal fluid therapy group. Primary outcome was comparison of total amount of fluid infused intraoperatively in both the groups. Secondary outcomes included intraoperative and postoperative arterial blood gas parameters, biochemical parameters, use of vasopressors, number of fluid boluses, complications and duration of hospital stay. RESULTS: There was no significant difference in total intraoperative fluid infused [90 (84-117.5 mL) in goal-directed fluid therapy and 105 (85.5-144.5 mL) in liberal fluid therapy group (p = .406)], median difference (95% CI) -15 (-49.1 to 19.1). There was a decrease in serum lactate levels in both groups from preoperative to postoperative 24 h. The amount of fluid infused before dopamine administration was significantly higher in liberal fluid therapy group (58 [50.25-65 mL]) compared to goal-directed fluid therapy group (36 [22-44 mL], p = .008), median difference (95% CI) -22 (-46 to 2). In postoperative period, the total amount of fluid intake over 24 h was comparable in two groups (222 [204-253 mL] in goal-directed fluid therapy group and 224 [179.5-289.5 mL] in liberal fluid therapy group, p = .917) median difference (95% CI) cutoff -2 (-65.3 to 61.2). CONCLUSION: Intraoperative plethysmographic variability index-guided goal-directed fluid therapy was comparable to liberal fluid therapy in terms of total volume of fluid infused in neonates during perioperative period. More randomized controlled trials with higher sample size are required. TRIAL REGISTRATION: Central Trial Registry of India (CTRI/2020/02/023561).

Isolated Fetal Ventriculomegaly- Postnatal Outcomes and Proposed New Prognostication Classification.

Background: Outcome of Isolated ventriculomegaly diagnosed antenatally depends on size of ventricles and associated malformations. There is scarcity of literature on the guidelines for prognostication based on outcomes as per the ventricle size. Aim: The aim of this work was to study outcome of antenatally detected isolated ventriculomegaly in terms of medical termination, postnatal neuro-developmental milestones, and mortality; and also to propose a new prognostication classification. Methods: Prospective and retrospective observational study on antenatal mothers with isolated ventriculomegaly diagnosed in fetus. Outcomes in terms of termination of pregnancy, postnatal mortality, need of surgery, and morbidity were recorded. Patients were categorized into four groups: Group 1--ventricle size <10 mm, II--11-15 mm, III--16-20 mm, and IV > 20 mm and neuro-developmental milestones were co-related. Association with chromosomal anomalies, congenital heart disease, and maternal infection were also analyzed. Results: 521 antenatal females were referred with fetal anomalies with 163 having CNS malformations. Isolated ventriculomegaly was seen in only 44. Patients of groups 1 and 2 had 100% normal neuro-developmental milestones without any intervention. Group 3 patients had normal neurodevelopmental milestones in 60% only while shunt surgery was required in 40% of patients. All patients of group 4 had poor outcome with only 50% survival. No association with chromosomal anomalies and heart disease was found. Conclusions: Prognosis of isolated ventriculomegaly depends upon size of ventricles and its progressive increase on serial ultrasounds. New proposed classification is simple and would be useful for the treating surgeons to explain the prognosis to parents so as to relieve them of anxiety.

Factors affecting stress levels in parents of surgical neonates: A prospective observational study.

BACKGROUND: There is a paucity of research focusing on the stress levels in parents of newborns undergoing surgery. Resource challenged systems have to deal with overcrowding, a shortage of workforce along with demographic and socioeconomic issues like delayed presentations and out of pocket expenses. The primary objective of this study was to understand the factors associated with stress in the parents of these congenitally malformed neonates. METHODOLOGY: This was a prospective cohort study, which was conducted in a neonatal surgical ICU of a tertiary care teaching hospital. Factors affecting stress levels in parents of surgical neonates were studied in 100 participants. A multi-dimensional questionnaire - The PSS: NICU score was utilized in the study. The parents were interviewed on Day 3-5 after surgery. RESULT: 59% of the respondents were fathers. The majority of the parents were in the age bracket of 24 to 35 years. The mean scores for the subscales sights and sounds, looks and behavior and alteration in the parental role were 3.24±0.8, 3.52±0.63, 3.55±0.8 and 2.8 ± 0.9 respectively. The highest level of stress was found in the domains of alteration of parental role and infant appearance and behavior. Comparisons showed significantly higher maternal scores in all the domains. Overall stress scores were highest for abdominal wall defects. CONCLUSION: Parents of neonates undergoing surgery suffer from significant stress levels and appropriate counseling targeted towards specific stressors is required to allay this important parental issue.

Efficacy of dexmedetomidine as an adjunct to ropivacaine in transversus abdominis plane block for paediatric laparoscopic surgeries: A double-blinded randomised trial.

BACKGROUND AND AIMS: α2 agonists have been utilised in regional blocks, but very little data is available for their use in transversus abdominis plane (TAP) block in paediatric laparoscopic (LAP) surgeries. This study investigated the analgesic effect of ropivacaine alone versus its combination with dexmedetomidine for TAP block in children undergoing LAP surgery. METHODS: A randomised, double-blind trial was conducted in 50 American Society of Anesthesiologists (ASA) 1 and 2 children of 2-8 years undergoing LAP abdominal surgery. Children were randomised to receive a total volume of 0.5 ml/kg of 0.2% ropivacaine (LA group) or 0.2% ropivacaine with 1 µg/kg dexmedetomidine (LAD group) for performing ultrasound-guided bilateral TAP block postoperatively (PO). Patients were monitored PO for vital signs, pain, sedation, time to first rescue analgesic and total analgesic consumption for 24 h. Time to first rescue analgesic was expressed as mean ± standard deviation (SD) and analysed using Kaplan-Meier survival analysis. Pain and sedation scores were expressed as median [interquartile range (IQR)] and analysed using Mann-Whitney U test. RESULTS: First rescue analgesic demand was significantly longer (P = 0.001) in LAD (474.8 min) versus LA group (240.9 min) but total analgesics consumption in first 24 h was comparable. Pain scores were significantly lower (P < 0.05) in LAD compared to LA group at all times PO. Each group had comparable but significantly lower sedation scores up to 24 h PO. CONCLUSION: Addition of dexmedetomidine to ropivacaine in TAP block prolongs the time to first analgesic requirement without a difference in the total analgesic consumption.

Role of urodynamics in male patients of high-anorectal malformations: a prospective study.

PURPOSE: Association of spinal or vertebral anomalies and the iatrogenic denervation during surgical correction of anorectal malformation patients especially in boys can lead to neurogenic bladder inthese subset of patients. The paucity of literature with regard to urodynamic studies focusing exclusively in male children with high-anorectal malformations (HARM) lead us to analyze the urodynamic changes. The objective was to study urodynamic profile in male patients who have undergone surgery for anorectal malformation. METHODS: Male high-anorectal malformation patients who had completed all the stages of repair were prospective studied. Following the basic work up, all patients based on the urodynamics were categorized into 2 groups as safe or unsafe bladders. Unsafe bladder was defined as detrusor pressure > 40 cm (high detrusor pressure) or pressure variability of 15 cm of water (detrusor overactivity) or significant post-void residue. MRI was limited to patients with only abnormal urodynamics to rule out spinal causes of neurogenic bladder and due to financial constraints, it could not be offered to all patients. RESULTS: 41 HARM meet the exclusion criteria. All patients were asymptomatic with none having history of urinary tract infections. Ultrasound showed bladder wall thickening in 31.7% patients. UDS revealed reduction in bladder capacity and compliance was noted in 31.7% and 30% patients, respectively. Elevated detrusor pressures (> 40 cm of water) were noted in 10% (4/41), detrusor overactivity with concomitant elevated detrusor pressures in 19.5% (8/41) and normal UDS in 70% (29/41). 13 patients (31.7%) had abnormal cystometric parameters with 12(30%) having unsafe bladders. MRI confirmed sacral agenesis in 1 patient with unsafe bladder. CONCLUSION: Urodynamics can demonstrate occult neurovesical dysfunction in patients with HARM. This would help in early renal protective therapy and prevent the burden of long-term sequelae of neurovesical dysfunction in HARM patients.

TIMP-2 as a noninvasive urinary marker for predicting neurogenic bladder in patients under follow-up for spina bifida.

INTRODUCTION: Neurogenic bladder (NB) post-meningomyelocele (MMC) repair is a major challenge and needs lifelong follow-up. Many cytokines have been implicated in the pathogenesis of NB. To avoid repeated urodynamic studies (UDS) and renal scans, we studied urinary tissue inhibitors of metalloproteinases-2 (TIMP-2) levels and correlated with urodynamic profiles to establish their efficacy. MATERIALS AND METHODS: Prospective case-control study on children between 6 months to 12 years of age, who were at least 6 months post-MMC repair and had NB on UDS. Patients were evaluated under 4 cohorts of 20 patients each: Group A (NB on treatment), Group B (NB not on treatment), Group C (no NB), and Group D (Controls). All groups underwent radiofrequency thermocoagulation, urine culture, ultrasonography. Urine samples were stored at -800°C and analyzed using a validated Human ELISA kit for TIMP-2. RESULTS: Eighty patients with a mean age of 3.54 ± 2.1 years were studied. A common ultrasound finding was a thickened urinary bladder (33.3%). All UDS parameters showed a statistically significant differences between groups with NB (Groups A and B) and a group without NB (Group C). Analysis of TIMP-2 levels between individual groups was statistically significant. The area under the receiver operating characteristic curve between urinary TIMP-2 and cystometric parameters indicated that urinary TIMP-2 levels are highly diagnostic of NB. TIMP-2 value of 358.5 pg/ml was found to be the least value with 93.5 sensitivity and 86.2% specificity. CONCLUSION: This study highlights the potential of urinary marker TIMP-2 as noninvasive and cost-effective test to initially diagnose and predict the progression of disease in NBs with reasonable sensitivity and specificity.

Outcome of antenatally diagnosed cystic hygroma - Lessons learnt.

PURPOSE: This study is carried out to characterize the prognosis and outcome of antenatally diagnosed cystic hygroma in a series of registered pregnancies. METHODS: This is a prospective cohort study, carried out over a period of 4 years (Jan 2016-Sept 2019). All the pregnancies referred from the department of obstetrics with antenatally detected cystic hygroma or increased nuchal thickness on level II ultrasonography suggestive of lymphangiomas were registered in the Pediatric Surgery outpatient department. Amniocentesis, fetal ECHO and fetal MRI (if indicated)were done. Prognosis was explained to the family and mode of delivery was planned as per the obstetric indications. Postnatal evaluation included general physical examination along with ultra sound (USG)doppler of the lesion. The neonates were admitted in neonatal surgical ICU for the administration of intralesional bleomycin in a dose of 0.3 IU/kg under strict observation. All the babies were followed up at 3, 6 and 12 months. Results were segregated as excellent, good and still in follow up cohort as per the final outcome. The fetal neck masses detected antenatally and the age at first dose of bleomycin were compared with the number of sclerotherapy doses required to achieve good response. RESULTS: Only nine patients out of 626 registered pregnancies (1.4%) were diagnosed with cystic hygroma. One antenatal mother opted for termination of pregnancy at 19 weeks of gestation and another patient was lost to follow up after receiving two doses of bleomycin in postnatal period. Maternal hypothyroidism was noted in two pregnancies. Antenatal USG shows increased nuchal thickness in 2 cases (first trimester) and 6 cases in level II scan. Amniocentesis shows normal fetal karyotyping in all these pregnancies. Fetal ECHO suggests normal biventricular function in 100% cases while fetal MRI done in one case completely ruled out any aerodigestive compromise. Mode of delivery is found to be planned elective LSCS in 57% of cases while normal vaginal delivery attained in 43% of cases as per the obstetric guidelines. Six cases achieved good response with >50% reduction in size out of which 4 cases received the dose at the first 10 days of life. Complete disappearance of lesion at the end of 6 months of follow up were seen in two babies. CONCLUSIONS: Antenatal screening for early detection of fetal cystic hygroma with possible associated congenital aneuploidies helps in prognostication and planning the mode of delivery. A multimodality approach during intra and postpartum increases safety margin. Even huge neck masses without associated anomalies carry fair prognosis. Intralesional bleomycin is safe and prevents surgical morbidity.

Robotic Assisted Vesicoscopic Cohen's Reimplantation in Pediatric Patient: Nuances of Technique, Experience, and Outcome.

Aim: Vesicoscopic Cohen's reimplantation closely replicates the open procedure and is reported to have equally good results. Robotic assisted vesicoscopic reimplantation (RAVR) is a new procedure, and the information on technique of RAVR is sparse. We present our technique, experience, and early outcome with RAVR and review of literature. Patients and Methods: We retrospectively evaluated patients with vesicoureteric reflux grade II and above. These patients were subjected to RAVR. Selection criteria for this procedure were age >4 years, preserved renal function, and bladder capacity >250 mL. Patients with previous bladder surgery or urethral issues were excluded. Patients were operated by DaVinci Si system, and the technique is described in detail. Three ports were placed by use of bougie. The ureters were adequately mobilized before placing in submucosal tunnel. Results: Total of 5 ureters were reimplanted in 4 patients (2 females and 1 bilateral). Ureteral tapering was done in 1 patient. Mean port placement time was 25 minutes with 12 minutes of docking time. Mean console time was 70 minutes for unilateral cases and 100 minutes for the bilateral case. There was resolution of reflux in all patients. The mean follow-up duration is 9 months. Conclusions: Robotic assisted vesicoscopic reimplantation is a feasible and safe method of replicating Cohen's reimplantation. There is a learning curve with the procedure, and nuances related to the technique need to be learned by the surgeon. This is the third report on RAVR and provides the necessary technical details required by a robotic surgeon to replicate this procedure.

Thoracoscopic Transdiaphragmatic Excision of Hepatic Hydatid in Patients with Synchronous Pulmonary Hydatid: A Novel Operative Approach.

Background: We wish to describe a novel technique of transdiaphragmatic excision of hepatic hydatid in patients with synchronous ipsilateral lung hydatid. Patients and Methods: Two patients, aged 11 and 6 years, presented with large synchronous hydatid cyst in right lung and liver. Second patient had two large cysts in right lobe of liver in posterior-superior segment of VII and VIII. The patients underwent thoracoscopic excision of lung cyst, and in same sitting the liver hydatid was seen bulging through the diaphragm. The diaphragm was incised to expose the cyst beneath, with excision of hydatid. Port-in-cyst technique was used to excise the membranes. Results: Both lung and liver cyst were successfully removed through thoracoscopic approach and a laparotomy was altogether avoided. Both patients did well in follow-up (3 years and 6 months). There are no respiratory issues in these patients. Conclusions: This is a novel approach for multiple synchronous hepatic and pulmonary hydatid. This can be applied in specific scenario as described. It gives a significant advantage of avoiding a laparotomy and thoracotomy in patients and thus reducing morbidity.

Pediatric Trauma: Management and Lessons Learned.

AIM: The aim is to prospectively study 125 trauma patients admitted in the pediatric surgery ward in our institute. MATERIALS AND METHODS: Pediatric patients admitted in the ward after initial resuscitation in the triage room were included. Isolated neurosurgical and orthopedic injuries were excluded. X-ray cervical spine, hip, and chest and a focused assessment with sonography in trauma ultrasound were done for all patients. Computed tomography of the abdomen or chest was done where relevant. Injury profile and surgical intervention when needed were analyzed. RESULTS: Road traffic accidents and fall from height caused 73.6% of the injuries. School-going children were most commonly affected (60.8%). Distinctive injuries were noted such as abdominal wall hernias and delayed bladder perforation. All solid organ injury irrespective of grade treated conservatively. Forty percent of the children required surgical intervention. Five patients after laparotomy were found to have surgical conditions unrelated to trauma, whereas another 14 required delayed surgery. Five patients had injuries secondary to sexual abuse. All except two patients were discharged in a satisfactory condition and are doing well in the follow-up. CONCLUSION: In spite of extensive injuries and the need for multiple surgeries, children with trauma have a good prognosis. Close observation during admission and also in follow-up are essential, as many patients may require delayed surgery ≥1 week from injury.

Role of gabapentin and anticholinergics in management of neurogenic bladder after repair of spina bifida - a randomized controlled study.

BACKGROUND: Anticholinergics are well established in the management of neurogenic bladders. However, some patients do have sub-optimal response or severe side effects. This study is designed to assess and compare efficacy of gabapentin with oxybutynin in neurogenic bladders after surgery for spina bifida. METHODS: Patients were randomized into three groups after urodynamic studies and started on oxybutynin, gabapentin, and combination of both, respectively. Thorough clinical and urodynamic reassessment was done at 6months and one year after starting treatment. RESULTS: Forty-four patients (3-19years) were studied. Improvement was noted in symptoms as well as urodynamic parameters in all groups. Maximal improvement of symptom score was with combination of drugs at 1year. In urodynamic studies, compliance, pressures, and capacity showed improvement, which was significant between the groups at both six months and 1year for bladder pressures and volume. Improvement in compliance though marked was not statistically significant. Best response was seen in group receiving both drugs. Gabapentin was better tolerated than oxybutynin. CONCLUSION: Gabapentin is a good alternative to oxybutynin for management of neurogenic bladder, both as monotherapy and as an add-on therapy. It has potential application in patients with inadequate response to anticholinergics. LEVEL OF EVIDENCE: prospective competitive treatment study - level II.

A rare case of isolated Cushing syndrome in a 3-month-old boy.

Adrenocortical carcinoma (ACC) is a rare malignancy in children. Most of these are endocrinologically active tumors, with virilizing features being typically dominant. Its presentation with isolated Cushing syndrome is rare. We report a 3-month-old infant with Cushing syndrome without the clinical features of androgen or mineralocorticoid excess consequently diagnosed with ACC. We discuss the different presentations, diagnosis, and management of this rare tumor with a rarer presentation.

Vesicoureteral reflux: Endoscopic therapy and impact on health related quality of life.

AIM: To evaluate the health related quality of life (HRQOL) after endoscopic injection treatment for vesico ureteral reflux (VUR) in children. MATERIALS AND METHODS: Fifty four children received treatment and were prospectively evaluated for their quality of life scores, according to resolution of reflux on cystograms and status of renal scars. RESULTS: Of the 81 refluxing units, 72 (89%) had resolution of reflux whereas 9 (11%) did not resolve. The total average QOL was higher for the patients in the resolved group as compared to the non resolved group. Comparison of pre and post procedure renal DMSA scans in 44 patients showed status quo in 26, regression of scars in six, progression in two and formation of new scars in 6. The total increase in HRQOL was highest in regression group (67.91), and lowest in progression group (36.45). CONCLUSIONS: Successful endoscopic treatment of VUR is associated with improved quality of life, as indicated by higher HRQOL scores in the resolved group.

Glial heterotopia of maxilla: A clinical surprise.

Glial heterotopia is a rare congenital mass lesion which often presents as a clinical surprise. We report a case of extranasal glial heterotopia in a neonate with unusual features. The presentation, management strategy, etiopathogenesis and histopathology of the mass lesion has been reviewed.

Peripheral primitive neuroectodermal tumor of the chest wall in childhood: clinico-pathological significance, management and literature review.

Peripheral primitive neuroectodermal tumor of the chest wall is a rare malignant tumor usually occurring in children. The tumor shares a similar histology, immunohistology and cytogenetics to Ewing's sarcoma. The location of the tumor in the chest wall poses a major challenge with respect to the diagnostic workup and treatment which involves multidisciplinary management. Neoadjuvant chemotherapy is given initially and is followed by complete surgical resectioning of the mass followed by repeat chemotherapy with or without radiotherapy. We report a case of peripheral primitive neuroectodermal tumor of the chest wall in a 5-year-old boy and review the literature regarding its diagnosis and management.

Small bowel hemangiomas: Diagnostic role of capsule endoscopy.

Vascular anomalies involving the small bowel are an uncommon cause of gastrointestinal bleeding in childhood. We present here an 11-year-old boy who presented with severe anemia and malena. The routine investigations did not reveal any pathology. A capsule endoscopy study was performed, which clinched the diagnosis and identified two intestinal hemangiomas. The hemangiomas were resected and the child recovered.

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.