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Background: Is vascular training in paediatric surgical oncology considered desirable ?, Methods: A voluntary survey of work practice was undertaken with the surgeon membership of The International Society Of Paediatric Surgical Oncology (IPSO) using a structured designed questionnaire., Results: A total of 149 IPSO surgeon members completed the survey. 57% (N = 84) of surgeons surveyed had no specific training in vascular surgery. 43% surgeons (N = 63) stated they had acquired some skills in residency training and/or with transplantation surgery. 65% (N = 96) of respondent surgeons stated that vascular surgical training must be incorporated into pediatric surgical oncology training and 27% (N = 40) agreed that it was considered desirable. 89% (N = 133) of surgeon respondents had encountered major vascular injury during work practice while operating on pediatric solid tumors. Vascular injury repairs were undertaken and attempted by pediatric surgeons though expert assistance of vascular surgeons proved crucially essential in many instances. Emergent operations included patch repairs, vessel ligation techniques and insertion of vascular graft prostheses. Interventional radiology services to arrest life-threatening hemorrhage were also reportedly utilized by respondents., Conclusion: Vascular injuries have significant potential for devastating patient outcomes including never event 'mortality'. The IPSO surgeon survey highlights that there are visible 'gaps' in skills training. Training to be a pediatric oncology surgeon must incorporate acquisition of skill sets proficiency in vascular surgery., (Copyright © 2024 Elsevier Inc. All rights reserved.)

The world's resources are unequally divided. Investment in healthcare in Low- and Middle-Income Countries (LMICs) has traditionally involved vertical public health strategies with little emphasis on the delivery of safe and effective surgery and anesthesia. The past 20 years has witnessed greater awareness of the numbers of people who have no access to surgery, the morbidity and mortality of such limited access, and the cost to the individual and society if a solution is not found. Global surgery has become a hot topic and The Millennium Development Goals (MDG) and the Sustainable Development Goals (SDG) have underlined the importance of surgery in national health care plans. The harsh metrics reported by the Lancet Commission has framed the problem in more concrete terms and as of today, over 5 billion people lack access to safe surgery, over 143 million extra surgeries are needed annually to affect the imbalance and more than 15,000 children die each day in Sub Saharan Africa and Southeast Asia from preventable disease, including the lack of access to safe surgery. Horizontal integrated healthcare that includes surgical provision is the new norm, but its introduction is expensive and will need time. The ambition of universal healthcare access for all people (SDG) with a corresponding reduction in child and maternal mortality will be difficult for most governments. Altruistic, Non-Governmental Organizations (NGOs) are ideally placed to assist this goal but must introduce changes in practice to include onsite and off-site training, broad partnerships with other NGOs and academic bodies and greater advocacy with governments to ensure sustainability. NGOs should work in concert with local agencies, hospitals and governments to meet local needs., (Copyright © 2021 Royal College of Surgeons of Edinburgh (Scottish charity number SC005317) and Royal College of Surgeons in Ireland. Published by Elsevier Ltd. All rights reserved.)

Despite greater awareness of patient safety issues especially in the operating room and the widespread implementation of surgical time out World Health Organization (WHO), errors, especially wrong site surgery, continue. Most such errors are due to lapses in communication where decision makers fail to consult or confirm operative findings but worryingly where parental concerns over the planned procedure are ignored or not followed through. The WHO Surgical Pause/Time Out aims to capture these errors and prevent them, but the combination of human error and complex hospital environments can overwhelm even robust safety structures and simple common sense. Parents are the ultimate repository of information on their child's condition and planned surgery but are traditionally excluded from the process of Surgical Pause and Time Out, perhaps to avoid additional stress. In addition, surgeons, like pilots, are subject to the phenomenon of "plan-continue-fail" with potentially disastrous outcomes. If we wish to improve patient safety during surgery and avoid wrong site errors then we must include parents in the Surgical Pause/Time Out. A recent pilot study has shown that neither staff nor parents found it added to their stress, but, moreover, 100% of parents considered that it should be a mandatory component of the Surgical Pause nor does it add to the stress of surgery. Surgeons should be required to confirm that the planned procedure is in keeping with the operative findings especially in extirpative surgery and this "step back" should be incorporated into the standard Surgical Pause. It is clear that we must improve patient safety further and these simple measures should add to that potential.

We involved the parents of paediatric patients in the first part of the three-stage WHO Surgical Safety Checklist (SSC) process. Forty-two parents took part in the study. They came to the theatre suite with their child and into the induction room. Immediately before induction of anaesthesia they were present at, and took part in, the first stage of the three-stage SSC process, confirming with staff the identity of their child, the procedure to be performed, the operating site, and the consent being adequately obtained and recorded. We asked parents and theatre staff later whether they thought that parental involvement in the SSC was beneficial to patient safety. Both parents and staff welcomed parental involvement in the WHO Surgical Safety Checklist and felt that it improved patient safety.

Lesions at the thoracic inlet are difficult to access via a thoracic or cervical approach. The use of the anterior cervico-thoracic trapdoor incision has been reported to give good exposure to the anterior superior mediastinum in adults. We report our experience of four cases where a trapdoor incision was used to gain excellent access and exposure to thoracic inlet pathology in children., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background: Total or near total resection of high-risk, stage 4 abdominal neuroblastoma has been correlated with improved local control and overall survival but may be complicated by vascular injury. We describe our experience in the management of significant aortic injuries during this procedure., Methods: With the institutional review board waiver, medical records of children who had major abdominal aortic reconstruction during neuroblastoma resection from 1996 to 2006 were retrospectively reviewed., Results: There were 5 children with aortic grafting: 3 girls and 2 boys. Mean age at surgery was 7.2 years (range, 16 months to 17 years). Two children were operated on for recurrent retroperitoneal disease. Tumor encasement of the aorta was seen in all children. In 3 children, the injury occurred during dissection of paraaortic and interaortocaval lymph nodes below the level of the renal arteries. In the remaining 2 children, injury occurred early during mobilization of the tumor. Three polytetrafluoroethylene tube grafts and 1 on-lay patch graft were used to repair the 4 distal aortic injuries. One 4-year-old female with aortic and renal arterial injuries was managed with an aortic Dacron tube graft and a polytetrafluoroethylene tube graft for the renal artery. The mean period of follow-up is 28 months after aortic graft (range, 3 months to 10 years). Total colonic ischaemia, transient acute tubular necrosis, and duodenal perforation were seen in one child, who needed subtotal colectomy and ileostomy. Another child with an omental patch over the graft had a transient duodenal obstruction, which was managed conservatively. There were no other complications, and 4 of the 5 children are disease-free to date. One child at 10 years after his distal aortic tube graft remained asymptomatic with normal distal blood flow on magnetic resonance angiogram and with normal growth., Conclusion: The neuroblastoma surgeon should be prepared to perform aortic and vascular reconstruction. Aortic encasement, preoperative radiation therapy, and reoperative surgery were observed in these patients and may be risk factors.

Antenatal detection of anterior abdominal wall defects (gastroschisis and exomphalos) enables detailed prenatal planning and counselling with appropriate intrauterine transfer, delivery in a tertiary referral centre with prompt access to paediatric surgery and early surgical intervention. The authors believed that there was a relatively low rate of antenatal detection in Ireland and that an improved antenatal screening program would allow increased detection thus avoiding emergency retrievals from peripheral obstetric centres. Our hypothesis was that there was a significant difference in management and outcome in children with anterior abdominal wall defects detected antenatally and those detected at birth. All anterior abdominal wall defects in The Republic of Ireland are assessed and closed in two surgical centres, Our Lady's Hospital for Sick Children, and the Children's University Hospital, Dublin. A retrospective review of all admissions in both centres revealed 96 neonates with anterior wall defects (excluding bladder exstrophy and cloaca) over a 7 year period from 1998 to 2004 inclusive. Medical records, operative reports, neonatal databases and admission details were reviewed. The antenatal data search included anomaly detection, prenatal management plan and intrauterine transfer. Perinatal data included place of birth, weight, gestational age, mode of delivery, time to surgery and type of surgery, the time to establish full enteral feeding and the length of hospital stay were used as overall markers of outcome. Fifty-three patients had gastroschisis and 43 exomphalos with an antenatal detection rate of 53% (n = 28) and 34% (n = 15), respectively, with an overall detection rate of 44%. There was no significant difference in the median birth weight (2.83 vs. 2.85 kg), gestational age at birth (37 vs. 37 weeks), time to full feeding (12 vs.14 days) and length of stay (20 vs. 17 days) between those detected antenatally or postnatally, respectively. About 77 had a primary closure performed of which 63 infants had surgery within the first 24 h of life. There was no difference in the time to surgery, frequency of complications or the surgical outcome in either group. Intrauterine transfer did not affect any outcome measure assessed. The demographics and the presence of associated anomalies did not differ between the groups. The hypothesis that antenatal diagnosis in anterior abdominal wall defects improves outcome has been demonstrated to be false. Despite this result, the importance of antenatal screening and prenatal management of complex foetal conditions with consultation with experienced paediatric and neonatal staff is without doubt.

Aim: Scarcity of paediatric cholelithiasis limits the obtainable experience in paediatric laparoscopic cholecystectomy (LPC). We report minilaparotomy cholecystectomy (MLC) in children which may be a useful alternative to LPC avoiding the added cost of disposable laparoscopic equipment., Methods: Nineteen cases underwent a retrograde MLC using a 2-4 cm transverse right upper quadrant incision. Various parameters were assessed., Results: Mean age at presentation was 10.87 years, mean length of the incision 2.8 cm, mean operative time 36.5 min, mean analgesic requirement 2 days, mean time to feed/ mobilize 1 day each, and mean duration of hospital stay was 2.7 days. All had satisfactory cosmetic result without postoperative complications., Conclusions: MLC in children involves a small incision, excellent cosmetic scar and rapid postoperative recovery at relatively low cost. It is a good alternative to LPC.

Chest wall reconstruction following major tumour resection remains a challenging problem in the paediatric population due to the need for growth and development following the repair. Synthetic non-biodegradable grafts and materials have led to asymmetrical growth and significant functional and cosmetic compromise. The ideal graft material should be biological non-immunogenic which enables natural healing and facilitates further growth. Ewing's sarcoma is an uncommon primitive neuroectodermal tumour which rarely occurs in the ribs. However, recent improvements in management have produced a significantly improved survival; increasing the importance of chest wall reconstruction. We prospectively assessed the utilisation of Surgisis (an acellular collagen matrix) as a replacement graft in two children undergoing primary chest wall reconstruction following tumour resection. Surgisis is derived from porcine small intestinal submucosa (SIS) that serves as a template for tissue reconstruction and remodeling. The graft was well tolerated; there was no herniation and it allowed normal symmetrical chest movement and growth to occur. The properties of SIS allows natural collagen growth and compares favourably with marlex mesh and other forms of chest wall reconstruction. We believe its unique properties allow it to act as a suitable framework for chest wall reconstruction allowing chest wall growth and development.

Aim: The aim of this report was to assess the effects of radiotherapy (RT) in children with abdominopelvic tumours in whom a biodegradable (Polyglactin 910) mesh had been inserted prior to commencement of radiotherapy., Methods: Retrospective analysis was carried out of four patients with abdominopelvic tumours who underwent radiotherapy between 2000 and 2002 as part of their management., Results: All children tolerated radiotherapy well with no evidence of acute or chronic radiation enteritis. One child developed prolonged postoperative ileus and a second child developed infective diarrhoea and fever, not related to radiation., Conclusion: We have highlighted a good tolerance of radiotherapy in children following the insertion of a Polyglactin 910 mesh prior to starting radiation and would recommend further larger studies with longer follow-up to support this.

Background: The aim of the study was to assess the utility of alpha glutathione S-transferase (alphaGST) as a potential marker of intestinal ischemia-reperfusion injury in children after cardiac surgery., Methods: Twenty-six patients undergoing cardiac surgery were enrolled in this longitudinal experimental study. Blood samples were drawn for analysis at specified time points during surgery and analyzed for alphaGST levels. Clinical indices of splanchnic morbidity were assessed up to discharge from hospital. Results were analyzed using Mann-Whitney tests and linear mixed effects models., Results: Two groups were identified. Group 1 (n = 16) showed no intestinal morbidity and group 2 (n = 10) had signs of intestinal morbidity. Statistical differences were shown between the 2 groups with respect to time with aortic cross-clamp (ACC) in situ, time on cardiac bypass, duration of operation, time to enteral feeding and full feeding, time on mechanical ventilation, and time in the intensive care unit postoperatively. The serum concentration of alphaGST was significantly higher for group 2 and this rise was greatest after removal of the ACC., Conclusions: AlphaGST showed significant elevation in patients with prolonged bypass times and ACC times. These patients also displayed signs of intestinal morbidity, suggesting that this marker may be useful in screening patients at risk for intestinal pathology. This rise in alphaGST was associated with a prolonged ischemia time, and was greatest after the cross-clamp was released, suggesting that it is a postischemic reperfusion phenomenon leading to its elevation. A low alphaGST level appears to exclude significant intestinal ischemia.

There is no ideal bio-tensile material for tissue replacement in paediatric surgical conditions. A variety of materials have been described to replace or reinforce tissue deficits in congenital diaphragmatic hernia (CDH). The problems with these materials range from extensive surgery to body wall deformity and hernia recurrence. The ideal graft would be safe, strong, have the potential to grow and not require replacement, not affect chest wall development or produce deformity, and have minimal risk of recurrence. Surgisis is a biomaterial composed of porcine intestinal submucosa that provides inherent tensile strength as a collagen lattice and is ultimately replaced by native collagen tissue. Our objective was to prospectively assess the utility of Surgisis as a replacement graft in a cohort of 10 children undergoing primary or recurrent CDH repair. The graft was well tolerated, and there was no recurrence of herniation. We conclude that Surgisis collagen mesh may be a useful alternative for tissue replacement or reinforcement in difficult cases of CDH and diaphragmatic agenesis. The biological framework of Surgisis may also prove useful in other situations of complex tissue loss in children.

Infantile myofibromatosis (IM) is a condition characterized by the formation of spindle cell tumors of skin, soft tissue, and viscera. Although small vessel involvement by the process is a frequently identified and indeed diagnostically useful histological finding, involvement of large vessels is not widely reported. Fibromuscular dysplasia (FMD) is a noninflammatory arteriopathy characterized by intimal, medial, and/or adventitial fibroplasias leading to luminal compromise and aneurysm formation. Although venous disease has been reported, involvement of arterioles and viscera has not been identified. We report a patient in whom IM was diagnosed, on the basis of multiple soft tissue tumors present from birth, who subsequently developed generalized and ultimately fatal FMD. These two conditions exhibit overlapping pathologic features, including pronounced intimal fibroplasia. Their occurrence in a single individual may provide insights into the pathogenesis of both conditions, suggesting that they represent part of the same spectrum of vascular myofibroblastic proliferations., (Copyright 2004 Society for Pediatric Pathology)

Gastrointestinal stromal tumors (GIST) in children are rare and their behavior has been regarded as difficult to predict on pathological criteria. We report our experience with two gastric GISTs in children aged 10 and 11 years. Both remain alive and free of disease at 5 years and 2 years respectively. Comparison of the pathological features in the resected specimens with a recently proposed guidelines for predicting outcome in this group of tumors is reported., (Copyright 2003 Wiley-Liss, Inc.)

Background/purpose: The accurate and early diagnosis of intestinal ischemia remains difficult chiefly because of a lack of a suitable marker that is noninvasive and easy to use. The glutathione S-transferases (GST) are a family of cytosolic enzymes involved in detoxification and released from a variety of cells when the cell membrane is damaged. The enzymes are distributed widely in the intestine and show isoform specificity in their distribution throughout the intestinal tract. Several previous reports have shown the utility of these enzymes in the diagnosis of liver and renal graft damage during and after organ transplantation. The object of this study was to determine if GST levels correlated with histologic changes of intestinal ischemia in a controlled animal model of mesenteric intestinal ischemia., Methods: Control and experimental male Sprague-Dawley rats underwent laparotomy and ligation of the Superior Mesenteric Artery (SMA) and both control and experimental animals were studied at 30, 60, 90, 120, and 240 minutes. Blood taken from the Inferior Vena Cava (IVC) and Portal Vein (PV) and jejunal and ileal perfusates were assayed for alpha and mu isoforms of GST using a commercially available enzyme immunoassay. In addition, jejunal and ileal segments were sampled and reviewed by a histopathologist blinded to the group being studied., Results: A reproducible pattern of intestinal ischemia was noted with worsening grades of injury observed with greater ligation times. Luminal alpha and mu GST release (as measured by the appearance in luminal perfusate) increased with increasing ischemia times. Increased ischemia times resulted in increased levels of alpha and mu GST in both portal and systemic venous samples but lagged behind the appearance of raised luminal GST values., Conclusions: The results suggest that GST may be an interesting and useful marker in the early detection of intestinal ischemia. Its detection in peripheral blood has implications for a more detailed study design to determine the sensitivity and specificity of this marker in more diverse clinical conditions such as necrotizing enterocolitis and superior mesenteric artery occlusion., (Copyright 2002, Elsevier Science (USA). All rights reserved.)

A 5-year-old boy presented with an infected left-sided branchial fistula. Despite antibiotic treatment and repeated excision of the fistula, purulent discharge from the wound persisted. Three-dimensional computed tomography (3D CT) reconstruction greatly facilitated the diagnosis and management of this case by showing the course of the fistulous tract. The complexity of the tract suggests that this represents a variant arch anomaly because it contains features of first, second, third, and fourth arch remnants., (Copyright 2001 by W.B. Saunders Company.)

Sigmoid volvulus is an exceptionally rare cause of intestinal obstruction in neonates. Only 7 cases have been reported in the English and French literature. The authors report a recent case of sigmoid volvulus in a neonate secondary to anal stenosis and review the diagnosis and management of this serious condition. The authors believe that carefully performed radiologic reduction is the preferable alternative to surgical intervention., (Copyright 2001 by W.B. Saunders Company.)

Acute febrile neutrophilic dermatosis, or Sweet syndrome, is a cutaneous eruption characterized clinically by the appearance of painful red plaques and nodules and histologically by an intense dermal neutrophilic infiltrate. Extracutaneous manifestations are rare. We report a patient in whom otherwise typical cutaneous Sweet syndrome was accompanied by an extracutaneous manifestation in the ileum.

Background: Xanthogranulomatous pyelonephritis is a severe, atypical form of chronic renal parenchymal infection accounting for 6/1000 surgically proved cases of chronic pyelonephritis. Its manifestations mimic those of neoplastic and other inflammatory renal parenchymal diseases and, consequently, it is often misdiagnosed preoperatively., Aim: To examine the relation between clinical history and the results of renal investigations performed in children with xanthogranulomatous pyelonephritis., Method: A retrospective review of 31 cases presenting with the histopathological diagnosis of xanthogranulomatous pyelonephritis between 1963 and 1999., Results: The mean follow up was 8.2 years. The male:female ratio was 1:1.1. The left kidney was affected in 26 of the 31 patients. The positive findings on examination and investigation at presentation were: fever, 16 children; pyuria, 26 children; positive urine culture, 16 children. A haemoglobin of < 100 g/l was measured in 27 of 31 patients and 15 of 18 patients tested had a raised erythrocyte sedimentation rate of > 20 mm in the first hour. Twenty six children had renal calculi, with a large reduction in the function of the affected kidney on isotope scintigraphy in 27 of the 29 patients tested. Hypertrophy of the contralateral kidney, shown on imaging, was present in 17 of 31 patients., Conclusions: Increasing awareness of this condition should lead to the diagnosis being suspected preoperatively.

The role of surgery in the intensive care unit (ICU) remains unclear. Although previously shown not to increase morbidity for patent ductus arteriosus ligation, Broviac catheter insertion, and recently, general neonatal and paediatric surgery, there remains a reluctance to operate on sick patients in the ICU (in-situ surgery, ISS). A retrospective study of 25 critically ill children and neonates who underwent ISS was performed. Surgery was aided by operating loupes and a high-intensity headlight. ISS was not associated with any morbidity, and although a 36% mortality occurred in this small series, in no case was this due to ISS. ISS avoids the risks of transfer to the operating theatre and the potential delays in theatre access. Our results suggest that ISS in a tertiary-level paediatric surgical hospital is safe and does not impact adversely on clinical outcome.

A 7-week-old infant with a locally invasive, orbital, congenital hemangiopericytoma underwent emergency external carotid artery (ECA) ligation for exsanguinating hemorrhage from an intraoral biopsy site. ECA ligation was successful in controlling the life-threatening hemorrhage and in reducing tumor size and vascularity. The location and extensive nature of the tumor prevented primary excision. Preoperative adjuvant chemotherapy was unsuccessful in controlling tumor growth. After ECA ligation, with reduction in tumor bulk and blood supply, the tumor was radically excised. This technique has important implications in the management of patients with extensive hemangiopericytomas or sino-facial tumors previously regarded as unresectable and which present with life-threatening hemorrhage.

The determination of small bowel perfusion after an ischemic insult is difficult. Regional perfusion was determined in an animal model of neonatal intestinal ischemia using the techniques of laser Doppler flowmetry and the clearance of locally generated hydrogen. Both methods reliably measured tissue perfusion in the areas of maximal ischemic injury. However, considerable variability, perhaps owing to motion artifact, was seen in areas of patchy necrosis. The results suggest that the laser Doppler flowmeter is a suitable technique to measure tissue perfusion in areas of maximal ischemia. However, efforts to reduce motion artifact will be necessary if the laser Doppler is to be used in tissue sites where blood flow is critical for safe anastomosis. This will be a subject of future study.

Intraoperative positioning of the pediatric patient during venous access procedures is crucial. Currently such patients are positioned with a sandbag beneath the shoulders and in a head-down position. This has significant limitations because the bag must be removed for subsequent on-table radiography. The authors have devised a simple inexpensive technique that obviates this need and facilitates the procedure. They have used the device in over 80 patients undergoing venous access. No problems relating to the device have been noted, and on-table x-rays are readily facilitated.

Three patients with stable liver function after Kasai portoenterostomy developed acute liver failure secondary to liver necrosis. Doppler ultrasound at presentation revealed reversed diastolic hepatic arterial blood flow. Two patients survived after urgent liver transplantation. Liver necrosis should be suspected in children with chronic liver disease presenting with fever and rapidly deteriorating liver function.

Thirty children received 35 liver transplants for fulminant or late-onset liver failure between March 1988 and May 1993. Aetiology included non-A non-B hepatitis in 12, Wilson's disease in 8, drug-induced hepatic failure in 6, hepatitis B in 1, hepatitis A in 1, tyrosinaemia in 1 and congenital haemochromatosis in 1. Three patients were retransplanted, one each for hepatic artery thrombosis, non-A non-B graft reinfection, and chronic rejection. Two of these three patients received a third transplant for chronic rejection and hepatic artery thrombosis. One patient in the retransplant group survived. Overall, graft and patient survival at a mean follow-up of 17 months were 49% and 57%, respectively. Mortality was related to vascular complications in three patients (hepatic venous obstruction, portal vein thrombosis and hepatic artery thrombosis). Two patients died of primary sepsis (cerebral aspergillosis and cytomegalovirus (CMV) pneumonitis in association with graft-versus-host disease). Systemic sepsis and multiorgan failure was documented as a cause of death in four children and sepsis in association with chronic rejection in a further three patients. One child died of respiratory failure 4 weeks after transplantation. Mortality in eight children less than 2 years was 75% and this was significantly greater than for older children (P < 0.003, Mantel Cox). Earlier referral, even in the absence of a definitive diagnosis and particularly in children under 2 years is advisable and may improve survival.

Cytotoxic therapy for paediatric malignancy continues to be associated with significant mortality and morbidity. However, survival figures continue to improve despite increasingly toxic treatment protocols. Aggressive supportive care, more than any other modality, has produced a dramatic reduction in mortality during the past 30 years, and includes the appropriate management of infection, provision of adequate nutrition, and proper use of blood and blood products. The paediatric surgeon plays an integral role in the multidisciplinary team of cancer management and together with other team specialists is responsible for the planning of surgical intervention and also ensuring the safe passage of immunologically impaired patients through the additional stress of surgery. This paper details essential aspects of supportive care as commonly practiced in paediatric patients undergoing therapy and surgery.

Forty-eight patients with repaired congenital oesophageal anomaly underwent aortopexy for significant tracheomalacia between 1980 and 1990. Indications for aortopexy included recurrent apnoea/cyanosis in 31, near fatal episodes in 16, recurrent respiratory distress and infection in 20 and worsening stridor in 15. Gastro-oesophageal reflux was noted in 30 patients, recurrent fistula in 6 and oesophageal stricture in 14. Aortopexy cured near fatal episodes in all patients and resulted in improvement of airway obstruction in 95%. The procedure failed in 2 patients due to unrecognised bronchomalacia and phrenic nerve palsy respectively. Aortopexy is the primary procedure of choice for significant tracheomalacia when associated with near fatal episodes and significant airway obstruction.

The pathophysiology of skeletal muscle loss in cancer cachexia is poorly understood. Immature, male, New Zealand White rabbits (TBs; n = 11) were implanted with VX-2 carcinoma and various indices of systemic and limb metabolism were examined in comparison with pair-fed controls (PFCs; n = 9) and normal controls (NCs; n = 22) fed ad lib. The TBs became hypophagic and experienced reduced growth relative to both control groups (P << 0.001). At 7 weeks (tumor burden 3-6% of body weight; no metastasis) the TBs had the following statistically significant differences from NCs: anemia, neutrophilic granulocytosis and thrombocytosis, hypercalcemia, hypoinsulinemia, elevated plasma triglycerides and altered plasma amino acids, increased hind limb effluxes of lactate and most amino acids. These alterations were not caused by hypophagia, since the PFCs were normal at 7 weeks with regard to all measured parameters except body weight and limb flow, both of which were reduced. The decrease in flow (P < 0.05) apparently contributed to conservation of skeletal muscle amino acids in the PFCs. Young New Zealand White rabbits implanted with VX-2 carcinoma manifest tumor burden, wasting, and metabolic alterations qualitatively similar to those seen with many human cancers.

Testicular growth was measured in 33 males who had undergone 2 orchiopexies on the developing testis. Multiple surgery on the abdominal or canalicular testis was associated with a high rate of testicular atrophy (40%) and a 46% decrease in testicular volume. A similar decrease (43%) in testicular volume was noted in those patients with an infracanalicular testis but no testis atrophied in this group. The results suggest that primary orchiectomy be considered in the high testis provided the contralateral testis is normal, or alternatively a Fowler-Stephens or microvascular transplant if it is not. Patients with an infracanalicular testis may undergo a careful second-stage orchiopexy. All patients with retarded testicular growth should perform routine self-examination.

Abdominal cystic masses in childhood are rare and despite improved imaging techniques continue to present diagnostic confusion. Typically such cystic lesions are mesenteric or intestinal in origin. However, rarer causes include cystic dilatation of a blind ending ureter. To date over 100 cases of blind ending ureters have been reported and their management described. We report on a case of non-communicating ureteric cyst associated with a blind ending ureter that recurred following percutaneous drainage and required operative resection. Although percutaneous drainage techniques have been advocated for a variety of abdominal cystic lesions, the experience gained from this case suggests that operative excision is the treatment of choice.

Congenital laryngeal clefts are rare. This paper reports on the management of 3 patients with Type 2 laryngo-tracheo-oesophageal cleft. One patient died following tracheopexy after successful cleft closure. Prognosis is related to the presence of other major anomalies, the early diagnosis of the lesion and institution of appropriate respiratory and nutritional care prior to correction of the defect. A greater awareness of the condition combined with aggressive diagnostic endoscopy should result in early diagnosis and improved survival.

Problems with loss of correct orientation of the pull-through rectal pouch during posterior sagittal anorectoplasty (PSARP) for high imperforate anus can occur during laparotomy after perineal closure is complete. A simple modification of the de Vries-Peña technique is described that facilitates preservation of orientation and precise anatomical neomuscle construction. A slotted rubber tube preserves correct anatomical orientation of the neorectal pouch and allows accurate construction of the muscle complex around the tube.

A total of four patients with a major pancreatic pseudocyst were treated by percutaneous pigtail catheter drainage. All patients recovered with complete resolution of the pseudocyst. There were no complications in relation to catheter insertion. Mean(s.d.) hospital stay was 56(15) days with a mean(s.d.) duration of catheter drainage and total parenteral nutrition of 21(14) and 35(9) days respectively. Percutaneous drainage is an appropriate method of first line therapy for non-resolving or enlarging pancreatic pseudocyst in children.

A 12 year old boy with a splenic pseudocyst is reported. Percutaneous drainage resulted in complete emptying but rapid reaccumulation. Cyst decapsulation with splenic preservation was curative and is recommended for definitive therapy of splenic pseudocysts.

Although extracorporeal shockwave lithotripsy (ESWL) has revolutionised the management of urinary calculous disease, the long-term effects of ESWL on renal function are not known. This study of 18 children demonstrated a mild, statistically insignificant decrease of 15% in DTPA measured glomerular filtration rate following ESWL. In view of this, we recommend that pretherapy and posttherapy estimates of renal function be obtained in all patients undergoing ESWL. Long-term follow-up is necessary to further quantify the effects of ESWL on the developing kidney.

Two management patterns were identified in 36 patients with exomphalos--primary surgical closure and initial topical therapy with delayed surgical closure. Primary surgical closure of minor exomphalos was well tolerated in 15 patients, but was associated with a high local and systemic morbidity rate in 14 patients with major defects. In contrast, initial topical therapy with silver sulphadiazine and delayed closure in seven matched patients with a major defect were well tolerated and did not prolong duration of hospitalization. Enteral feeding was more readily established and subsequent fascial closure facilitated in the conservatively treated group. It was suggested that this method should be more often considered in the management of all instances of major exomphalos.

Regional blood flow in man is ideally measured by techniques that are noninvasive, accurate, and can measure flow repetitively with comparative ease. Although numerous noninvasive techniques are available, no single method records blood flow accurately in every location. The neophyte investigator is often faced with a confusing array of methods and can spend considerable time searching for the ideal one. This paper presents current methods available to the clinical or metabolic researcher and comments on the strengths and limitations of each method. It is hoped that this will allow more rapid selection of a flow measurement method that is tailored to each individual's need.

Quantitative radionuclide scintigraphy often requires empirical calibration factors derived from phantoms which simulate the radioactivity distribution, tissue geometry and tissue composition of the region of interest. This paper describes a method in which casts made with fiberglass tape of the region of interest. This paper describes a method in which casts made with fiberglass tape are used to form realistic, water-fillable phantoms of the limbs. Phantoms were constructed for the hind legs of the dog and rabbit, species frequently used in developing new radioscintigraphic techniques. Leg bones removed from euthanized animals were mounted anatomically within the casts. The dimensions of the phantom cavities were determined by x-ray computed tomography. A procedure was developed for orienting the phantoms to match the hind leg geometry of a given experimental setup. Use of the phantoms for image activity calibration is illustrated for a geometric-mean counting technique used to determine 99mTc activity densities in soft-tissue regions of the dog thigh. Generalization of the calibration technique to planar and tomographic imaging is straightforward. In situ measurements of 99mTc activity density obtained by external counting were compared with in vitro radioassays of excised tissue. For 22 tissue samples obtained from four dogs, the in situ and in vitro data were linearly correlated (r = 0.98, p much less than or equal to 0.001) over a 50-fold range of activity density. The mean and standard deviation of the observed percent discrepancies [% discrepancy = 100 (in situ - in vitro)/in vitro] were (7.8 +/- 2.9) and (13.7 +/- 2.1), respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

The management of differentiated thyroid cancer in childhood is controversial. In particular, the role of aggressive surgical treatment has been questioned. This study was performed to identify those factors that are predictive of recurrence and morbidity following treatment through use of a multivariate model. The records of all patients 17 years of age or less admitted in the last 35 years with histologically confirmed differentiated thyroid carcinoma were reviewed. Data were sufficient for multivariate analysis in 93. The mean age at diagnosis was 13.3 years, and the median period of follow-up was 20 years. Seventy-one percent of the patients had nodal metastases. There were no deaths from thyroid carcinoma in this series, and the overall recurrence rate after initial treatment was 34%. Multivariate analysis demonstrated that only age (p less than or equal to 0.07) and histologic subtype (p less than or equal to 0.01) were predictive of time to recurrence. Major morbidity was a function of age (p less than or equal to 0.007) and extent of thyroid surgery (p less than or equal to 0.01). Probability of minor complications was predicted by use of radical neck dissection (p less than or equal to 0.02). Use of total or subtotal thyroidectomy or of radical neck dissection in children does not prevent recurrence and is associated with an increased risk of complications. We conclude that these procedures should be avoided in pediatric patients.

Over 200 cases of colonic hemangioma have been reported, with more than 50% occurring in the rectosigmoid. These benign vascular neoplasms usually present in childhood, often with life-threatening hemorrhage. Lack of awareness of the condition continues to lead to errors in diagnosis and management, and may contribute to previously reported mortality rates of 50%. We report a case of diffuse cavernous hemangioma of the rectosigmoid in a 10-year-old boy and its treatment by low anterior resection using the end-to-end autostapler.

A retrospective study of 19 patients with pancreatic cystadenoma included 15 patients with microcystic and 4 with mucinous cystadenomas. The typical clinical presentation was that of an elderly woman with an upper abdominal mass. An association with diabetes mellitus and extrapancreatic malignant disease was noted. Total tumor resection provided the best chance of cure and removed the risk of compression of adjacent organs and, in mucinous cystadenomas, the risk of malignant transformation.

We are studying the transport of C-11 and N-13 labeled amino acids in tumor-bearing rabbits to determine the role of amino acid transport in the pathogenesis of muscle wasting in cancer. To validate a new, in vivo, method for measuring transport in skeletal muscle with these compounds, an isolated hindlimb model was developed in rabbits. The limb was perfused with a non-recirculating, normothermic, constant pressure system and a cell-free perfusate. Hemodynamic and metabolic parameters were measured during the first 75 min. of perfusion and found to remain normal and stable. Flow varied directly with perfusion pressure over the normal range of resting flows in the intact rabbit hindlimb. Time-activity curves (TAC's) were recorded from the medial thigh following bolus co-injection of L-[amide N-13] glutamine or N-13 L-glutamate with Tc-99m human serum albumin (HSA) into the femoral artery. Regional plasma flow was determined from the Tc-99m data. The N-13 TAC's consistently manifested a three-phased washout with half times of approximately 30 sec., 5 min. and 2 hr. Capillary and cellular transport parameters were computed from the N-13 data using a double barrier, single capillary model of capillary and cellular transport and assuming that the three washout components result, respectively, from tracer throughput, extraction into the interstitial space and extraction into the intracellular space. This interpretation was validated and the sensitivity of the technique to transport processes demonstrated by examining the effects on the N-13 TAC's and computed transport parameters of several factors known to influence cellular transport of amino acids, viz., the insulin concentration, amino acid concentration and pH of the perfusate. Time-activity curves and transport parameters for N-13 L-glutamine in the isolated limb were very similar to those observed in the intact rabbit hindlimb, suggesting that studies in the perfused model are indicative of amino acid transport in vivo. The methodology described here is especially well suited for studying the specific effects on transport of factors which influence amino acid metabolism in skeletal muscle (e.g., hormones and monokines).