Outcomes of patients with exomphalos and associated congenital heart diseases.

INTRODUCTION: Exomphalos is an anterior abdominal wall defect resulting in herniation of contents into the umbilical cord. Severe associated chromosomal anomalies and congenital heart disease (CHD) are known to influence mortality, but it is not clear which cardiac anomalies have the greatest impact on survival. METHODS: We performed a retrospective review of the treatment and outcome of patients with exomphalos over a 30-year period (1990-2020), with a focus on those with the combination of exomphalos major and major CHD (EMCHD). RESULTS: There were 123 patients with exomphalos identified, 59 (48%) had exomphalos major (ExoMaj) (defect > 5 cm or containing liver), and 64 (52%) exomphalos minor (ExoMin). In the ExoMaj group; 17% had major CHD (10/59), M:F 28:31, 29% premature (< 37 weeks, 17/59) and 14% had low birth-weight (< 2.5 kg, 8/59). In the ExoMin group; 9% had major CHD (6/64), M:F 42:22, 18% premature and 10% had low birth-weight. The 5-year survival was 20% in the EMCHD group versus 90% in the ExoMaj with minor or no CHD [p < 0.0001]. Deaths in the EMCHD had mainly right heart anomalies and all of them required mechanical ventilation (MV) for pulmonary hypoplasia prior to cardiac intervention. In contrast, survivors did not require mechanical ventilation prior to cardiac intervention. CONCLUSION: EMCHD is associated with high mortality. The most significant finding was high mortality in those with right heart anomalies in combination with pulmonary hypoplasia, especially if pre-intervention mechanical ventilation is required.

Children with congenital colorectal malformations during the UK Sars-CoV-2 pandemic lockdown: an assessment of telemedicine and impact on health.

PURPOSE: This pilot study was designed to assess bowel function and quality of life (QoL) in children and adolescents with congenital colorectal malformations (CCM) during the first UK COVID lockdown period. METHODS: Changes in health were assessed through semi-structured interviews, gastrointestinal functional outcomes using Krickenbeck scoring and QoL by the modified disease-specific HAQL (Hirschsprung's disease anorectal malformation quality of life questionnaire). The State-Trait Anxiety Inventory (STAI)™ for adults was used to assess parental anxiety. RESULTS: Thirty-two families were interviewed; 19 (59%) reported no change in their child's health during the lockdown, 5 (16%) a deterioration and 8 (25%) an improvement. Neither the severity of the CCM, nor the degree of bowel dysfunction, correlated with any deterioration. The HAQL score was not correlated to a change in health. Anxiety scores ranged from no anxiety to clinical concerns. Telemedicine was well accepted by 28/32 parents (88%); however, in-person appointments were preferred if there were clinical concerns. CONCLUSION: In the follow-up of children and adolescents with CCM during the first UK lockdown using telemedicine we found that over half had stable health conditions. Patients needing additional care could not be predicted by the severity of their disease or their bowel function alone.

Patency of neck veins following ultrasound-guided percutaneous Hickman line insertion.

PURPOSE: Venous occlusion following permanent central venous catheter (CVC) insertion by open cutdown or the landmark percutaneous technique has been reported between up to 25 %. However, there are no published data on the equivalent rate following ultrasound-guided percutaneous CVC insertion. The purpose of this study was to document the rate of venous occlusion associated with ultrasound-guided percutaneous CVC insertion in children. METHOD: From 1 April 2010 to 1 December 2011, all children having elective or emergency removal of a Hickman line by the vascular access team had a Doppler ultrasound of their neck veins. Only Hickman lines inserted by the ultrasound-guided percutaneous route were included. Internal jugular, innominate and subclavian veins were scanned and recorded as patent, reduced or absent. RESULTS: We identified 100 consecutive children. Median age was 6 years (range 21 days to 16 years). Indication for insertion was chemotherapy (60), parenteral nutrition (15), blood products (12), renal replacement (3) and other indications (10). Three children had absent flow at the time of line removal (median age 4 months, range 3-6 months), with 2 out of 3 requiring removal for infection. The venous occlusion rate following ultrasound-guided insertion of CVC is 3 % in our study. CONCLUSIONS: We conclude that (1) complete venous occlusion is associated with younger age and CVC infection. (2) In our study, the venous occlusion rate of 3 % is significantly lower than the published series of either open cutdown or the landmark technique.

Getting it right first time: implementation of laparoscopic pyloromyotomy without a learning curve.

INTRODUCTION: Laparoscopic pyloromyotomy is now an accepted procedure for the treatment of pyloric stenosis. However, it is clear that during the implementation period there are significantly higher incidences of mucosal perforation and incomplete pyloromyotomy. We describe how we introduced a new laparoscopic procedure without the complications associated with the learning curve. MATERIALS AND METHODS: Five consultants tasked one surgeon to pilot and establish laparoscopic pyloromyotomy before mentoring the others until they were performing the procedure independently; all agreed to use exactly the same instruments and operative technique. This involved a 5mm 30-degree infra-umbilical telescope with two 3mm instruments. Data were collected prospectively. RESULTS: Between 1 January 2013 and 31 December 2017, 140 laparoscopic pyloromyotomies were performed (median age 27 days, range 13-133 days, male to female ratio 121:19). Fifty-five per cent of procedures were performed by trainees. Complications were one mucosal perforation and one inadequate pyloromyotomy. There were no injuries to other organs, problems with wound dehiscence or other significant complications. The median time of discharge was one day (range one to six days). CONCLUSION: Our rate of perforation and incomplete pyloromyotomy was 1.4%, which is equivalent to the best published series of either open or laparoscopic pyloromyotomy. We believe that this resulted from the coordinated implementation of the procedure using a single technique to reduce clinical variability, increase mentoring and improve training. This approach appears self-evident but is rarely described in the literature of learning curves. In this age of increased accountability, new technologies should be incorporated into routine practice without an increase in morbidity to patients.

Congenital mesoblastic nephroma: a single-centre series.

BACKGROUND: Congenital mesoblastic nephroma is a rare disease. Treatment is surgical in the first instance. Chemotherapy has traditionally been thought not to have a role. Recent literature suggests a 50% mortality rate for recurrent/metastatic disease. MATERIALS AND METHODS: This study is a retrospective case review of prospectively collected data. Demographics, histopathology, treatment, outcomes and follow up were reviewed. RESULTS: Nine patients, 6 male and 3 female, were included. The median age at presentation was one month (range 0-7 months); follow-up was for a median of 21.5 months (range 16-79 months). Two patients had mixed and classical subtypes and the other five had the cellular subtype. Surgery was completed by an open procedure in eight patients and laparoscopically in one. There were three recurrences; two were local and one was pulmonary. Recurrences were treated with a combination of chemotherapy, radiotherapy and surgery. One patient with recurrent disease died from acute-on-chronic respiratory failure secondary to lung irradiation but was disease free. The other eight are disease free, alive and well with no sequelae at latest follow-up. CONCLUSIONS: Surgery remains the mainstay of management with chemo- and radiotherapy reserved for unresectable tumours or adjuvant management of recurrent disease. Specimen-positive margins are not an indication for instituting chemotherapy. The tyrosine kinase pathway seems to be a potential target for future chemotherapeutic agents although it is too early to assess how that will impact on the management of congenital mesoblastic nephroma.

Long-term outcome in patients with short bowel syndrome after longitudinal intestinal lengthening and tailoring.

OBJECTIVES: Longitudinal intestinal lengthening and tailoring (LILT) is a well-established surgical treatment for short bowel syndrome. It has been shown to enhance peristalsis, decrease bacterial overgrowth, and extend mucosal contact time for nutrients. We present the results of a long-term follow-up of patients who underwent LILT and define prognostic parameters for the survival of these patients. PATIENTS AND METHODS: Between 1987 and 2006, 53 patients underwent LILT in our institution. The main diagnoses were gastroschisis, intestinal volvulus, intestinal atresias, and necrotizing enterocolitis. LILT was performed at a mean age of 24 months (range 4144 months). The follow-up time was 79.76 months (range 6234 months). RESULTS: After LILT, 41 of 53 patients survived, and 36 of 41 surviving patients were successfully weaned from parenteral nutrition (PN). In long-term follow-up 79% stayed free of PN. The overall survival rate was 77.36%. Weight gain occurred in 58% of the patients after LILT. The quality of life after LILT is on a high level, with most patients having normal physical strength and participating in normal social life and education. Prognostic factors for survival after LILT in short bowel syndrome are length of small intestine (0.06582 + 0.0131 x bowel cm), length of large bowel (P = 0.039), preoperative liver function, and successful weaning from PN within 18 months postoperatively (P = 0.0032). CONCLUSIONS: Patients undergoing LILT in short bowel syndrome have a high survival rate, weight gain, and a high quality of life. Autologous gastrointestinal reconstruction remains therefore the first choice in the treatment of patients with short bowel syndrome.

Management of kidney injuries in children with blunt abdominal trauma.

BACKGROUND/PURPOSE: The authors analyzed the incidence and the course of renal injuries encountered in a cohort of pediatric patients with blunt abdominal trauma. This review focuses on the early diagnostic and therapeutic approach rather than the long-term outcome and draws conclusions for an effective initial management. METHODS: From 1976 to 1996, the charts of 308 children with blunt abdominal trauma that were admitted to the authors' department were reviewed. The patients initially were evaluated using urinalysis, ultrasonography, and abdominal paracentesis (until 1984) and in specific cases iv-urography, computed tomography (CT), and angiography. The authors retrospectively classified the renal trauma after the widely used Organ Injury Scaling (OIS) into 5 grades and correlated the diagnostic value of various techniques as well as the diagnostic approach. RESULTS: Sixty-nine serious abdominal traumas were encountered. Thirty-six patients suffered renal lesions grade 2 (G2) or higher; 20 children were polytraumatized. There were 67 renal lesions including 28 G1, 22 G2, 8 G3, 5 G4, 1 G5, and 3 lesions of the lower urinary tract. Ultrasonography and urinalysis were found to be the optimal diagnostic methods for screening and following the course of renal injury. CT scan proved to be most reliable for detecting and exactly classifying renal lesions grade 2 or higher and superseded consecutively iv-urography. In cases in which CT scan failed to show renal excretion of contrast agent, angiography was performed. Ten patients proceeded to operative therapy. CONCLUSIONS: Ultrasonography and urinalysis proved to be the optimal initial evaluation tool for excluding renal injury both as a screening method and for further controls. Exact classification was possible by CT scan. During the reviewed time period a shift from surgical to conservative management was notable. If lesions were G4 or G5, surgical treatment with tendency toward minimally invasive therapy always was indicated.

Long-term total parenteral nutrition-induced hepatobiliary dysfunction in a rabbit model.

BACKGROUND/PURPOSE: Currently, the reason for hepatobiliary dysfunction associated with long-term total parenteral nutrition (TPN) is much debated and still unclear. No agreement can be achieved about whether bacteriotoxins and sepsis, enteral starvation, consequences of abdominal operations, or the TPN solution itself is the real cause for the disease. Animal models were criticized for their short period of TPN and their failure to demonstrate cholestasis and bile duct proliferation. The aim of this study was to establish an animal model for long-term TPN in which the same alterations of the hepatobiliary system as observed in humans could be produced. METHODS: In this model, rabbits could be kept for the first time under continuous TPN for 4 weeks. Three serial liver biopsy sections were taken operatively from each animal and biochemical analyses were performed four times. A control group of enterally fed rabbits underwent exactly the same procedure in respect to operations and handling, so that differences in macroscopical, biochemical, and histological changes between both groups could be attributed exclusively to TPN. RESULTS: Only in the TPN group gallbladder distension developed in all animals after 1 week. After 3 and 4 weeks, viscous dark bile, sludge and stones, a slight rise in direct bilirubin, and a decline in plasma albumin and alkaline phosphatase was noted. In both groups liver biopsy results showed a similar degree of mild portal inflammation and single-cell necrosis at equivalent time points. These changes could be caused by antiseptics, antibiotics, anesthesia, and operations. Although mild to moderate proliferative changes and no hydropic degeneration developed in the control group during the same time, the TPN group generated marked proliferative and degenerative changes. We noted as early as 1 week after starting TPN a severe hydropic degeneration in 90% of the animals. Fibrosis and bile duct proliferation increased from a slight degree after 1 week up to a moderate to severe degree after 3 and 4 weeks, respectively. CONCLUSIONS: The hepatobiliary alterations associated with TPN in children, which cannot be separated clinically from consequences of multiple other factors, can almost identically be reproduced in our rabbit model as a clear consequence of TPN. Furthermore, the hydropic degeneration of the liver cells begins in zone 3 and is an early predominant feature of hepatobiliary dysfunction in rabbits and infants. It must be rated as a response to a direct cytotoxic effect on the liver cell.

Impact of pharmacotherapy on lymphocyte volumes and activity of the Na+/H+ exchanger in patients with congestive heart failure.

Previous studies in patients with congestive heart failure (CHF) have revealed abnormalities of cellular volume that might have an impact on the dysregulation of peripheral vascular resistance. Human mononuclear leukocytes (HML) represent a model for the study of cellular volume regulation. We investigated the impact of enalapril and carvedilol on HML volume and on the activity of the Na+/H+ exchanger in 26 patients with CHF and 20 volunteers. Over a period of 4 weeks, 18 patients received enalapril in addition to the previous therapy while 8 patients additionally received carvedilol. HML diameters and the activity of the Na+/H+ exchanger were measured by a Coulter Counter. Both patient groups showed abnormally increased initial volumes of HML compared to the volunteer group at baseline. Four weeks of therapy with enalapril in addition to therapy with diuretics and digoxin did not result in a statistically significant reduction of lymphocyte volume, whereas add-on therapy with carvedilol to therapy with ACE inhibitors, diuretics and digoxin reduced the volume significantly. Alterations could not be found in the activity of the Na+/H+ exchanger in either patient group compared to volunteers. Supplementary drug therapy with carvedilol in patients with CHF leads to a reduction of the increased lymphocytic volume, possibly reflecting the beneficial effect of beta-blockade.

[Diagnostic and therapeutic consequences of kidney injuries in pediatric blunt abdominal trauma]. / Diagnostische und therapeutische Konsequenzen nach Nierenverletzungen bei kindlichem stumpfem Bauchtrauma.

Renal lesions are frequently encountered in blunt pediatric abdominal trauma. In this retrospective study, we analyzed the incidence of renal trauma in these trauma patients to determine which diagnostic and therapeutic approaches were most predictive. From 1976 to 1996, 308 children sustaining blunt abdominal trauma were admitted to our department. Patients were evaluated using abdominal paracentesis, ultrasonography, and urinary analysis. In specific cases, i.v. urography, CT scan, and/or angiography were applied. We used the Organ Injury Scale (OIS) for classification of renal trauma into five grades. We encountered 69 serious abdominal traumas. Thirty-six patients sustained renal lesions grade 2 (G2) or higher; 20 children were polytraumatized. There were 67 renal lesions with 28 G1, 22 G2, 8 G3, 5 G4, 1 G5, and 3 lesions of the urinary tract. Ultrasonography and urinary analysis were found to be optimal diagnostic means for screening and observing the course of renal lesions. For lesions G2 or higher, CT scan was the most reliable in classifying and diagnosing renal lesions. This superseded i.v. urography. If no contrast medium was excreted in the CT scan, angiography was indicated. Only ten patients proceeded to operative therapy. During the period reviewed, a shift from operative to conservative treatment was notable with a tendency toward minimally invasive therapy. If lesions were G4 or G5, operative treatment was always indicated.

A rare case of functional Pancreatic Neuroendocrine tumor with multi-organ involvement in a 25-year old female

SIGNIFICANCE@#Pancreatic neuroendocrine tumors (pNET) account for 1-10% of tumors arising in the oancreas, with functional pNETs reported less commonly than their non-functional counterpart. Glucagonoma is an even rarer form of functional pNET which has a reported annual incidence of 0.01-0.1 per 100,000.@*CLINICAL PRESENTATION@#This is a case of a 25 year old female presenting with a two-year history of palpable epigastric mass, abdominal pain and weight loss. She came in at our institution with worsened signs and symptoms in which she already had chronic diarrhea, anemia, glossitis, ad dermatitis eventually leading to development of early onset diabetes, stroke and cardiomyopathy.@*MANAGEMENT@#Initial contrast-enhanced CT scan revealed a pancreatic mass and was confirmed by endoscropic sonography as a large solid encapsulated mass at the head of the body of the pancreas measuring 7.7x5.5 cm. Histopathologic and immunohistochemical tests to tissue specimen obtained by fine needle aspiration biopsy of the mass revealed a well-differentiated pancreatic neuroendocrine tumor confirmed as glucagonoma by a remarkably elevated plasma glucagon level. The mass was deemed non-resectable at the time of diagnosis, hence the patient was started on octreotide LAR depot injection. Multi-systemic complications caused by the functional tumor were also managed through multidiscliplinary approach. Medical management resulted to marked improvement of the signs and symptoms and lead to a better quality of life.@*RECOMMENDATION@#In rare cases such as this, diagnosis is often a dilemma and causes delay in treatment. Prompt diagnosis will lead to early intervention preventing life-altering complications, disease progression, and mortality.

Transanal pull-through procedure for Hirschsprung's disease: a 5-year experience.

BACKGROUND/PURPOSE: Transanal endorectal pull-through (TEPT) has become a widely used approach for the treatment of Hirschsprung's Disease. The technique is safe and, according to previous reports, it has a good clinical outcome. In this study our experience with TEPT in the early postoperative period is evaluated. METHODS: The clinical course of 34 children (28 boys and 6 girls) who underwent one-stage pull-through operation according to De la Torre for Hirschsprung's disease from January 2003 to December 2007 was reviewed. Their ages ranged from 2 months to 4 years. Complications occurring within the first four weeks after operation were analyzed. RESULTS: Eight of 34 children (24 %) had early complications in the form of dehiscences of the anastomosis. Two children (6 %) had symptomatic anastomotic dehiscences. One child had an almost full retraction of the colon that had to be pulled down and resutured. One child developed a retrorectal abscess three weeks postoperatively due to anastomotic leakage. The dehiscences of 6 children (18 %) were asymptomatic. These dehiscences were detected only with standardized routine examination. The dehiscences healed uneventfully after resuturing. Two other patients (6 %) developed an anastomotic stricture that could be treated with rectal dilatations. Four children (12 %) showed a single episode of postoperative enterocolitis. CONCLUSION: The rate of early clinical and particularly subclinical complications such as anastomotic dehiscences after TEPT is higher than previously estimated. Patients should be monitored carefully during the early postoperative period. Severe complications can only be avoided with a thorough examination. Early resuturing of dehiscences might be helpful to prevent hazardous sequelae.

The effect of a taurine-containing drink on performance in 10 endurance-athletes.

To determine the effect of a taurine-enriched drink "Red Bull" on performance, 10 endurance-athletes performed three trials. After 60 min. cycling at approximately 70% VO2 max, the subjects pedalled to exhaustion on a cycle ergometer. During each exercise, the subjects received 500 ml of a test-drink after 30 min. submaximal cycling: "Red Bull" without taurine, without glucuronolacton (U1), "Red Bull" without taurine, without glucuronolacton, without caffeine (U2) and "Red Bull" original drink containing taurine, glucuronolacton and caffeine (U3).The heart rate level was significantly lower in U3 (p = 0,0031) 15 min. after application. The plasma catecholamines increased slightly from begin of exercise to 15 min. after application of the drinks in all trials but remained on a significantly lower level in U3 (epinephrine (p = 0,0011) and norepinephrine (p = 0,0003). Endurance time was significantly longer with "Red Bull" original in U3 (p = 0,015). The results of this study show a positive effect of a taurine-containing drink on hormonal responses which leads to a higher performance.