The current state of congenital tracheal stenosis.

Congenital tracheal stenosis (CTS) is an uncommon condition that has challenged pediatric surgeons for decades. Patients with CTS can present with a wide spectrum of symptoms and varying degrees of severity. In addition, a variety of techniques have been devised to repair this malformation. A review of these procedures and our suggestions for clinical standards and practice guidelines will be presented in this paper. A retrospective review of the literature on CTS from 1964 to 31 March, 2006. There is not one standard technique for the repair of CTS, as individualized approach to each patient and airway lesion is necessary to optimize patient management; nevertheless there is a consensus about segmental resection and anastomosis being best for short segment stenosis while slide tracheoplasty is most effective for the long-segment ones. Conservative management is also an option for select group of patients with careful and close follow up. Survival following surgery over the years has improved, but mortality remained high, particularly in a specific subset of patients presenting at the age less than 1 month with associated cardiac malformations. In conclusion, CTS remains a significant challenge for pediatric surgeons. Additional research is required to improve our understanding of the pathogenesis of CTS, and to develop evidence-based treatment protocols for the entire spectrum of presentation including conservative management.

Enterostomy tube placement in children with spinal muscular atrophy type 1.

OBJECTIVE: To determine the major complication rate in the first 30 days after enterostomy tube insertion in infants with spinal muscular atrophy (SMA) type 1. STUDY DESIGN: A retrospective case review of all children with SMA type 1 who had a gastrostomy or gastrojejunostomy tube placed by the image-guided technique at the Hospital for Sick Children from 1994-2004. Major complications were classified as peritonitis, aspiration pneumonia, respiratory failure, nonelective admission to the pediatric intensive care unit, and death. RESULTS: Twelve children were identified as having SMA type 1 with an enterostomy tube insertion. The median age at tube insertion was 6.1 months (range 2.2 to 15.8 months). Major complications in the first 30 days after the procedure included aspiration pneumonia (5/12 patients [41.6%]), respiratory failure requiring admission to the pediatric intensive care unit (4/12 [33%]), and death (2/12 [16.7%]). Children with development of aspiration pneumonia were significantly older at time of tube insertion (P < .05) than those with no aspiration. CONCLUSIONS: Major complications including death are seen in children with SMA type 1 in the first 30 days after enterostomy tube insertion.

Membranous duodenal stenosis: initial experience with balloon dilatation in four children.

INTRODUCTION: We present a novel approach to the treatment of membranous duodenal stenosis (MDS). To our knowledge this is the first paper to describe balloon dilatation for this entity. MATERIAL AND METHODS: Four children, 2 boys and 2 girls, aged between 8 and 28 days, underwent duodenal balloon dilatation. Balloon dilatation was performed under general anaesthesia using standard angiography balloons per os. Balloon diameters ranged from 6 to 14 mm. RESULTS: All balloon dilatations were successful. None of the procedures showed procedural or post-procedural complications. None of the patients subsequently required surgical intervention. To date all children are doing well. DISCUSSION: The initial experience with balloon dilation of MDS showed a 100% success rate, without procedural or post-procedural complications. The results obtained in this small group of patients suggest that the use of balloon dilatation in cases of MDS may be a safe technique that can be readily performed by an experienced interventional radiologist.

Endoscopic treatment of congenital H-Type and recurrent tracheoesophageal fistula with electrocautery and histoacryl glue.

OBJECTIVE: Congenital H-Type tracheoesophageal fistulae (H-Type TEF) and recurrent fistulae after primary repair of esophageal atresia represent a difficult problem in diagnosis and management. The treatment traditionally involved an open technique via a cervical or thoracic route, approaches with high morbidity and mortality rates of up to 50%. Endoscopic closure of fistulae has been reported with various techniques such as tissue adhesives, electrocautery, sclerosants and laser. However, the published case series contain a small number of patients with usually short-term follow-up. The aim of this paper is to present the experience of a decade at Toronto's Hospital for Sick Children, using diathermy and histoacryl tissue adhesive and discuss the indications and limitations of this technique. METHODS: Since 1995, 192 patients have been managed in this institution with tracheoesophageal fistulae of which 10 patients have been treated endoscopically. The fistulae were both of H-Type and recurrent tracheoesophageal fistulae following surgery for esophageal atresia and fistula division. One fistula occurred following trauma. The procedure was undertaken under general anesthesia in the image guided therapy suite under fluoroscopic control. Flexible ball electrocautery and injection of histoacryl glue were used either on their own or in combination. RESULTS: Fistula closure was achieved in 9 out of 10 fistulae. Four patients had a second endoscopic procedure. No major respiratory or other complications were encountered in association with the procedure. Follow-up has been between 3 months and 9 years. CONCLUSION: We conclude, endoscopic treatment of tracheoesophageal fistulae with electrocautery and histoacryl glue has been a safe and successful technique of managing H-Type and recurrent tracheoesophageal fistulae. In this paper, we discuss the indications and the surgical steps of the procedure. We highlight that diathermy should be carefully controlled and applied preferably in the small non-patulous fistulae. A fistula that has not closed after two endoscopic attempts is not suitable for further endoscopic treatment and therefore an external approach should be recommended.

A prospective, longitudinal study of central venous catheter-related deep venous thrombosis in boys with hemophilia.

BACKGROUND: Central venous catheters (CVCs) are often inserted into boys with hemophilia to secure venous access for factor prophylaxis and immune tolerance induction therapy. Complications associated with CVCs include catheter-related infections, local hemorrhage, and mechanical failure. Less frequently reported is CVC-related deep venous thrombosis (DVT). We conducted a prospective study to determine the frequency and outcome of this complication. METHODS: All boys (n = 16) with congenital hemophilia A or B with a CVC in place who were registered in the pediatric comprehensive care program at the Hospital for Sick Children, Toronto, were included in the study. They were prospectively assessed by imaging studies and clinical examinations for CVC-related DVT at two time-points, 2 years apart. Each boy was evaluated for inherited hypercoagulability. RESULTS: Eleven (69%) of the 16 boys had radiological evidence of DVT at the first evaluation and 13/16 (81%) at the second evaluation. In two boys there was improvement in the venogram findings at the second evaluation. None of the CVC-related DVTs completely resolved. Median age at the time of initial insertion of a CVC was 1.0 years (range 0.02-6.7 years). Median duration of CVC placement was 6.4 years (range 3.3-15.5 years). Only 4/13 boys with DVTs had clinical evidence of upper venous system obstruction. Only one boy, who did not develop a DVT, had a low protein C level. CONCLUSIONS: CVC-related DVTs occur in the majority of boys with hemophilia who have CVCs inserted for a prolonged period of time. Annual screening with imaging is recommended for boys with CVCs in place for >/= 3 years. Consideration should be given to removing CVCs as soon as peripheral venous access is feasible.

Central venous catheter-related thrombosis presenting as superior vena cava syndrome in a haemophilic patient with inhibitors.

We report the case of a 10.5-year-old boy with severe haemophilia A (SHA) and inhibitors who presented with superior vena cava (SVC) obstruction while on immune tolerance induction (ITI) with daily recombinant factor VIII (rFVIII) and factor eight bypassing activity (FEIBA) (75 U kg(-1)) twice a week. The boy had a right-sided implanted central venous catheter. Imaging revealed a large occlusive thrombus in the SVC with all upper venous system drainage occurring through the azygos and collateral veins. Despite initial success with local thrombolytic therapy using recombinant tissue plasminogen activator, the thrombus persisted. Mechanical thrombolysis and angioplasty resulted in the successful removal of the thrombus and resolution of the SVC syndrome. Unfractionated heparin was used to prevent thrombus reformation/propagation. A work-up did not reveal any underlying genetic prothrombotic risk factors. The occurrence of such a profoundly symptomatic thromboembolism (TE) in a boy with SHA with inhibitors is unusual. A combination of risk factors, including the ongoing infusion of high doses of FVIII in the context of a disappearance of inhibitors together with the infusion of clotting factors known to be potentially thrombogenic, may place haemophilic patients on ITI (immune tolerance induction) at risk for this rare, life-threatening complication. The appropriate management of TEs in such a setting is unknown.

Renal pseudoaneurysm after blunt renal trauma in a pediatric patient: management by angiographic embolization.

Renal pseudoaneurysm is a rare complication after blunt renal trauma. Only 18 cases have been reported in English-language published reports. We present a case of blunt renal trauma in an 11-year-old boy, complicated by delayed bleeding from a renal artery pseudoaneurysm. The patient was initially treated with conservative measures and was later treated with selective embolization of the pseudoaneurysm. The clinical presentation, management options, and clinical decisions are discussed.

Liver failure with marked hyperferritinemia: 'ironing out' the diagnosis.

Hemophagocytic lymphohistiocytosis (HLH) may manifest as neonatal liver failure characterized by hepatosplenomegaly, profound coagulopathy, ascites and hyperbilirubinemia. Marked hyperferritinemia may be present in these patients, mimicking perinatal hemochromatosis. Tissue specimens are critical in distinguishing these two diseases and in directing management. Clinical recognition and diagnosis of HLH can be difficult but are crucial for appropriate therapy and genetic counselling. Liver transplantation is absolutely contraindicated for patients with HLH but may be the only life-saving treatment modality for patients with perinatal hemochromatosis.

US-guided core biopsy of the spleen in children.

PURPOSE: To evaluate the safety, diagnostic yield, and clinical benefits of performing ultrasonography (US)-guided percutaneous splenic core biopsy in children. MATERIALS AND METHODS: US-guided splenic core biopsy was performed in 30 children aged 6 months to 15.3 years (mean, 7.0 years), with focal lesions in 27 patients and homogeneous splenomegaly in three. Four patients underwent repeat biopsy to identify changes in splenic disease. Four types of biopsy needles were used in this series. General anaesthesia was used in 21 patients and conscious sedation in nine. Medical records were reviewed to assess diagnostic accuracy, influence on treatment, and biopsy-related complications. RESULTS: All biopsies were performed without complication. Among the 30 biopsies, an accurate diagnosis was obtained in 25 (83%), a false-negative result was obtained in two (7%), and three (10%) were nondiagnostic. All conclusive results influenced treatment decisions. The mean number of needle passes was 2.7 per patient (range, 2-5 passes). Use of needles was 50%-100% successful in the acquisition of adequate tissue cores. Use of the 18-gauge needle was always successful in the safe acquisition of adequate tissue, with a maximum of three passes. CONCLUSION: US-guided splenic core biopsy is a minimally invasive, simple, and safe procedure for use in children. It provides relatively high diagnostic accuracy while minimizing complications when compared with alternative, more invasive procedures.

Intensive diabetes management decreases Na-Li countertransport in young subjects with type 1 diabetes and enlarged kidneys.

In type 1 diabetes, increases in sodium-lithium countertransport (Na-Li CT), kidney volume (KV), and albumin excretion rate (AER) may precede the development of persistent microalbuminuria. Limited data exist on reversibility of these factors early in the evolution of diabetic nephropathy. A crossover design was used to study the separate effects of enalapril and intensive diabetes management (IDM) on Na-Li CT, KV and AER in 17 children and adolescents with type 1 diabetes (5-10 years duration) with large kidneys (>275 ml/1. 73 m(2)) and predominantly normoalbuminuria. Subjects were randomized to receive 3 months of either enalapril (0.25 mg/kg/day) or IDM, a 3-month washout, followed by the alternate treatment for 3 months. During IDM, HbA1c decreased 2.5% (pre 9.5+/-0.3% (mean+/-SE), post 7.0+/-0.1%, p<0.0001), but was unchanged while on enalapril (pre 8.8+/-0.3%, post 8.5+/-0.3%, p=0.1). A significant decrease in Na-Li CT was seen with IDM (pre 0.43+/-0.05, post 0.36+/-0.04 mmol/l RBC/h, p=0.006) but not angiotensin converting enzyme inhibition (ACE-i) (pre 0.39+/-0.04, post 0.38+/-0.04 mmol/RBC/h, p=0.4). Neither ACE-i nor IDM affected KV or AER. It is concerning that kidney enlargement does not appear reversible at this early stage in the pathogenesis of diabetic nephropathy, although our conclusions are limited by the short duration of intervention and small sample size. The reduction in Na-Li CT with IDM suggests this may be a potentially modifiable risk factor for diabetic nephropathy.

Fluoroscopic landmark for SVC-RA junction for central venous catheter placement in children.

BACKGROUND: Vascular access devices are commonly placed under image guidance. The usual aim is to place the tip at the superior vena cava-right atrial junction (SVC-RA). OBJECTIVE: To identify a radiographic landmark for the SVC-RA junction that would be useful for accurate central venous catheter tip placement in children. MATERIALS AND METHODS: Images from 56 children undergoing contrast studies of their upper limb venous systems were examined for location of the SVC-RA in relation to a radiographic landmark. RESULTS: Most patients (92.5%) showed the SVC-RA junction to lie at the sixth thoracic vertebral level or the interspace above or below. The SVC-RA junction lay lower than the right main bronchus and the notch on the right cardiomediastinal contour. CONCLUSION: The vertebral body provides a useful and radiographically visible landmark for accurate central catheter tip placement.

Further report of small-bowel intussusceptions related to gastrojejunostomy tubes.

BACKGROUND: The use of gastrojejunostomy (GJ) tubes for feeding pediatric patients who have inadequate oral intake, cannot tolerate gastric feeding, or have significant gastroesophageal reflux may present problems. OBJECTIVE: To report our experience of intussusception associated with GJ tubes, with emphasis on risk factors. MATERIALS AND METHODS: Clinical histories and imaging studies were reviewed for all patients with GJ tube-associated intussusceptions at our institution from January 1995 to March 1999. RESULTS: Of 251 GJ tubes inserted, 40 (16%) intussusceptions occurred in 30 patients (20 males). They ranged in age from 3 months to 17 years (mean 2.6 years) and in weight from 3 to 90 kg (mean 12.5 kg). The underlying diagnosis varied. The main symptom was bilious vomiting. The initial diagnosis was made by sonography in 19 cases and fluoroscopy in 21. Intussusceptions recurred eight times in patients with a distal pigtail (n = 17) but only twice in patients without the distal pigtail (n = 18). CONCLUSIONS: GJ-tube related intussusception is a common, easily diagnosed problem. Predisposing factors appear to be male sex, young children, and presence of a distal pigtail on the tube. Awareness of intussusception is imperative for prompt diagnosis to achieve feeding tolerance.

Efficacy and safety of radiologically placed gastrostomy tubes in paediatric haematology/oncology patients.

BACKGROUND: The treatment of malnutrition, which is of great concern in paediatric haematology/oncology patients, is fraught with problems. The goals of our study were to document the complications and assess the weight gain with gastrostomy tubes (G-tubes) in this population. PROCEDURE: Patient data were acquired by retrospective review of all haematology, oncology, and bone marrow transplant (BMT) patients (n = 44) who received radiologically placed G-tubes at our institution over a 4-year period. RESULTS: Forty-four G-tubes were placed (59% peri-BMT). At tube placement, 55% of patients were malnourished and 45% were nourished. Seventy-five percent of patients had the procedure without general anaesthetic. Localized G-tube-site infection was the most common complication (41%). Major complications occurred in 3 patients; 2 patients experienced localized peritonitis, and 1 patient developed a localized collection of pus in the abdominal wall. There were no G-tube-related deaths. At 1 month after the tube insertion, 39% of patients had gained, 54% maintained, and 7% lost weight. At 3 months, 69% had gained, 28% maintained, and 3% lost weight. There was a statistically significant weight gain from the time of the G-tube placement to both 1 month (P < 0.018) and 3 months (P < 0.0001) after G-tube placement. Patients in all diagnosis categories showed improvement from 1 to 3 months. CONCLUSIONS: We conclude that retrograde tube placement is safe and can frequently be done without general anaesthetic and that G-tube feeding effectively reverses malnutrition and prevents weight loss in this patient population.

CT-guided percutaneous needle biopsy of small lung nodules in children.

Malignant pulmonary nodules in a patient with neoplasia alters clinical staging and therapy. Although it may be difficult, it is imperative to obtain a tissue diagnosis of such nodules. We describe a coaxial technique using controlled respirations, which we have found useful for biopsy of small pulmonary nodules in children. Clinical records, imaging and pathology (histology and cytology) were reviewed for 17 children who had 18 nodules biopsied in the method described. A diagnosis of malignant or benign tissue was made in 15 nodules. One biopsy was inadequate and there was one false-negative. Adequate cores were obtained in 15 nodules, cytology in 2 and inadequate material in 1. There was no significant morbidity.

Diagnosis and management of stenotic aorto-arteriopathy in childhood.

Stenotic aorto-arteriopathy is an uncommon vascular lesion characterized by segmental arterial stenoses. We reviewed the experience with several management algorithms to define the most effective management course. The clinical records of 14 pediatric patients with acquired SAA who presented over a 16-year period were reviewed. Most patients presented with a mid-thoracoabdominal coarctation and were diagnosed with Takayasu arteritis. Differentiating between Takayasu arteritis and fibromuscular dysplasia was difficult on clinical grounds or by angiography. Medical management of the end-organ disease and renovascular hypertension was only palliative. Selective percutaneous transluminal balloon angioplasty of the stenotic renal arteries had only transient benefits; renal autotransplantation had slightly better success. Dilation of stenosed aortic segments with balloon-expandable endovascular stents and subsequent renal autotransplantation proved useful. Distinguishing SAA resulting from fibromuscular dysplasia caused by Takayasu arteritis in the chronic vaso-occlusive phase may be unnecessary for effective treatment. Therapy should focus on interventions to minimize the end-organ damage caused by the vaso-occlusive manifestations of the disorders.

Tracheobronchial stenting for the treatment of airway obstruction.

PURPOSE: The authors report a 5-year experience of inserting the Palmaz stent into infants and children who had a variety of major airway obstructions. METHODS: From 1992 to 1997, 30 balloon expandable stents (Palmaz) were inserted in the trachea (n = 18) and bronchi (n = 12) of 16 infants, ages 1 week to 26 months (median, 9 months), suffering from three types of serious airway obstruction. In group 1, 10 stents were placed in eight children for tracheal or bronchomalacia. In group II, 11 stents were inserted in four infants for stricture at the site of surgical repair of stenosis. In group III, nine stents were placed to relieve airway compression from enlarged pulmonary arteries associated with severe congenital heart disease in three children and mediastinal lymphangioma in one. Tracheal stents were 30 mm long and were expanded to 8 to 10 mm at placement. Bronchial stents were 12 to 15 mm long and were expanded to 7 to 9 mm. The nonexpanded stents were placed on an inflatable balloon catheter and were inserted into the desired position in the airway through a bronchoscope or endotracheal tube using x-ray control. They were expanded and fixed in place by inflating the balloon to its rated diameter. RESULTS: In group I, granulation tissue developed over the stents in five of eight cases. Obstructing granulations were removed by scraping or balloon compression in three and resulted in earlier than the planned removal in two. Stents have now been removed in six of eight cases. Major airway obstruction has not recurred. In group II, stents have been in place in all cases for 13 to 56 months after insertion, but in one child with three stents, two were removed for obstructive granulations 44 months after insertion. All are well. All group III patients could be extubated after stenting, but two with heart disease died after 3 and 12 months of palliation. During the course of follow-up, stents in the bronchi of two had migrated, and an additional stent was required. Autopsy in one showed full-thickness bronchial erosion but no perforation by the stent. A total 11 of 30 stents have been removed bronchoscopically in seven children without complications. Another child referred here for tracheal stent removal after laser resection of granulations died at attempted removal because the stent was "welded" into the tracheal wall by the inflammatory reaction. Manipulation of the stent completely occluded the airway. CONCLUSIONS: Airway stents can be inserted easily and safely and left in-situ for prolonged periods to relieve major airway obstruction from a variety of causes. Tissue reaction may necessitate bronchoscopic manipulation and early stent removal, and adds to the difficulty of removal.

Nonoperative management of pancreatic injuries in children.

PURPOSE: The safety and efficacy of nonoperative management of pancreatic contusions and transections was examined by reviewing the case histories of 35 consecutive children with pancreatic injuries treated over the past 10 years. METHODS/RESULTS: Surgical exploration was performed for the management of associated injuries only. The diagnosis of pancreatic trauma was suspected in children with abdominal pain, tenderness, elevated serum amylase levels and findings consistent with pancreatic injury on abdominal ultrasound scan or computerized tomography (CT) examination. After children were diagnosed with pancreatic injury, enteral feedings were withheld and total parenteral nutrition administered until abdominal pain resolved and serum amylase levels and radiographic findings improved. Twenty-three children received diagnosis within 24 hours of injury, and in 12, the diagnosis was delayed 2 to 14 days. Hyperamylasemia was found in 27 of 35 children. Twenty-eight children sustaining pancreatic injuries were treated nonoperatively. Abdominal imaging in these children demonstrated pancreatic contusion in 14, transection in 11, and pseudocyst in three. Enteral feeding resumed an average of 15 days after injury. The average hospital stay was 21 days. Pseudocysts formed in 10 children (2 of 14 with contusion; 5 of 11 with transection; three children presented late, and the type of pancreatic injury could not be determined), whose average hospital stay was 25 days. All pseudocysts were successfully managed nonoperatively, although percutaneous aspiration or drainage was required in six children. Children underwent follow-up for an average of 10 months after injury (range, 1 to 144 months). Abdominal pain and radiological abnormalities resolved in all children before discharge from the clinic. CONCLUSIONS: Nonoperative management of pancreatic contusion and transection diagnosed radiologically is effective and safe. Pseudocysts may form after pancreatic injury, and if large or symptomatic, can be managed successfully by percutaneous drainage.

Ureteral opening pressure: a novel parameter for the evaluation of pediatric hydronephrosis.

PURPOSE: The antegrade nephrostogram is an important tool in the evaluation of the upper urinary tract. However, the information currently provided by a nephrostogram is largely limited to anatomical details. To establish a meaningful pressure-flow parameter that may be incorporated into a routine nephrostogram, we evaluated the ureteral opening pressure (defined as the pressure at which contrast material is first seen beyond the suspected site of obstruction) and correlated these findings with the results of pressure-flow studies performed with an external infusion and/or furosemide induced diuresis. MATERIALS AND METHODS: A total of 52 renal units were studied under a prospective pressure-flow study protocol. All patients had grade 3 or 4 hydronephrosis (Society of Fetal Urology classification) and patient age range was 0.2 to 12 years (median 1.1). The suspected sites of obstruction were the ureteropelvic and ureterovesical junctions in 42 and 10 renal units, respectively. With the patient under general anesthesia 22 gauge percutaneous nephrostomy needles were inserted. Pressure-flow studies with an external infusion and/or furosemide induced diuresis were then performed. As the renal pelvic pressure progressively increased during the course of the pressure-flow studies, the renal pelvic pressure at which contrast material was first seen to appear distal to the suspected site of obstruction was recorded as the ureteral opening pressure. Ureteral opening pressures were compared to the results of the pressure-flow studies. RESULTS: With a positive test defined as renal pelvic pressure greater than 14 cm. water, positive ureteral opening pressures were associated with positive pressure-flow study results in 100% of the cases, regardless of which form of pressure-flow study was used or where the suspected site of obstruction was located. In contrast, negative ureteral opening pressures had specificities and negative predictive values of only 19 to 57%, depending on the form of the pressure-flow study and the suspected site of obstruction. CONCLUSIONS: An elevated ureteral opening pressure was 100% predictive of obstruction and may obviate the need for more elaborate pressure-flow analyses. However, if the ureteral pelvic pressure remained low, the possibility of a potentially significant obstruction could not be definitively eliminated and further evaluation was required.

Non-operative management of traumatic pancreatic pseudocysts associated with pancreatic duct laceration in children.

Objective. To assess the successful non-operative management in traumatic pancreatic pseudocysts (TPP) associated with duct laceration in children. Surgical therapy (cystogastrostomy or distal pancreatectomy with splenic salvage) has been classically considered the treatment of choice for those pseudocysts. Materials and methods. This report presents the clinical and imaging findings in two children with TPP and pancreatic duct disruption observed either on endoscopic retrograde cholangiopancreatography or injection via catheter drainage. Results. Both children responded to long-term cyst drainage. Conclusion. Although the experience is limited, the authors suggest that pancreatic injury associated with duct laceration can respond to non-operative management.