The multi-disciplinary management of complex congenital and acquired tracheo-oesophageal fistulae.

AIM OF THE STUDY: Complex tracheo-oesophageal fistulae (TOF) are rare congenital or acquired conditions in children. We discuss here a multidisciplinary (MDT) approach adopted over the past 5 years. METHODS: We retrospectively collected data on all patients with recurrent or acquired TOF managed at a single institution. All cases were investigated with neck and thorax CT scan. Other investigations included flexible bronchoscopy and bronchogram (B&B), microlaryngobronchoscopy (MLB) and oesophagoscopy. All cases were subsequently discussed in an MDT meeting on an emergent basis if necessary. MAIN RESULTS: 14 patients were referred during this study period of which half had a congenital aetiology and the other half were acquired. The latter included button battery ingestions (5/7) and iatrogenic injuries during oesophageal atresia (OA) repair. Surgical repair was performed on cardiac bypass in 3/7 cases of recurrent congenital fistulae and all cases of acquired fistulae. Post-operatively, 9/14 (64%) patients suffered complications including anastomotic leak (1), bilateral vocal cord paresis (1), further recurrence (1), and mortality (1). Ten patients continue to receive surgical input encompassing tracheal/oesophageal stents and dilatations. CONCLUSIONS: MDT approach to complex cases is becoming increasingly common across all specialties and is important in making decisions in these difficult cases. The benefits include shared experience of rare cases and full access to multidisciplinary expertise.

Double-blind randomized clinical trial of percutaneous endoscopic gastrostomy versus radiologically inserted gastrostomy in children.

BACKGROUND: The aim of this RCT was to determine whether radiologically inserted gastrostomy (RIG) in children is associated with more complications than percutaneous endoscopic gastrostomy (PEG). METHODS: Children at a single tertiary children's hospital requiring a primary gastrostomy were randomized to PEG or RIG. Patients were followed by assessors blinded to the insertion method. Complications were recorded, assigned a severity score, and analysed by zero-inflated Poisson regression analysis on an intention-to-treat basis, adjusting for length of follow-up. RESULTS: Over a 3-year period, 214 children were randomized (PEG, 107; RIG, 107), of whom 100 received PEG and 96 RIG. There was no significant difference in the number of complications between PEG and RIG groups (P = 0·875), or in the complication score: patients undergoing RIG had a 1·04 (95 per cent c.i. 0·89 to 1·21) times higher complication score than those who underwent PEG (P = 0·597). Only age had an independent significant effect on complication score, with older patients having a 0·97 (0·95 to 1·00) times lower complication score per year. CONCLUSION: PEG and RIG are both safe methods of gastrostomy insertion with a low rate of major complications. Registration number: NCT01920438 ( http://www.clinicaltrials.gov).

Tissue-Engineered Tracheal Replacement in a Child: A 4-Year Follow-Up Study.

In 2010, a tissue-engineered trachea was transplanted into a 10-year-old child using a decellularized deceased donor trachea repopulated with the recipient's respiratory epithelium and mesenchymal stromal cells. We report the child's clinical progress, tracheal epithelialization and costs over the 4 years. A chronology of events was derived from clinical notes and costs determined using reference costs per procedure. Serial tracheoscopy images, lung function tests and anti-HLA blood samples were compared. Epithelial morphology and T cell, Ki67 and cleaved caspase 3 activity were examined. Computational fluid dynamic simulations determined flow, velocity and airway pressure drops. After the first year following transplantation, the number of interventions fell and the child is currently clinically well and continues in education. Endoscopy demonstrated a complete mucosal lining at 15 months, despite retention of a stent. Histocytology indicates a differentiated respiratory layer and no abnormal immune activity. Computational fluid dynamic analysis demonstrated increased velocity and pressure drops around a distal tracheal narrowing. Cross-sectional area analysis showed restriction of growth within an area of in-stent stenosis. This report demonstrates the long-term viability of a decellularized tissue-engineered trachea within a child. Further research is needed to develop bioengineered pediatric tracheal replacements with lower morbidity, better biomechanics and lower costs.

Real-time x-ray abnormality alerts for emergency departments using a radiographer comment model - a multisite pilot study.

INTRODUCTION: The timely communication of clinically significant image appearances to Emergency Department (ED) referrers is necessary for optimum patient care. Australian reliance on verbal communication only is time-limited, open to misinterpretation and lacks transparency. A combined radiographer alert and comment model was designed to reliably communicate image abnormalities to ED referrers in real-time. METHODS: A multidisciplinary steering group designed the model for all ED general imaging. Protocols were developed to document radiographer comments (critical, urgent and clinically significant) in patients' medical records. Critical findings were communicated directly to ED. Five NSW hospitals varying in size, complexity and population demographics piloted the model between three to twelve months during 2021-2022. Site auditors compared comments with the radiology report and designated each as True Positive (TP), False Positive (FP), indeterminate and clinically significant. Indeterminate cases were analysed by an external radiologist. Inter-observer consensus was obtained for all classifications via two independent auditors. The Positive Predictive Value (PPV), or precision of the comment, was calculated for each site. RESULTS: Radiographers (n = 69) provided comments for 1102 cases. The pooled average PPV for TP was 0.96; (0.947-0.971; 95% CI). The weighted mean error (FP comments) was 3.9%; (2.9% - 5.3%.; 95% CI). CONCLUSION: The Radiographer Comment model provided consistent levels of commenting precision and reproducibility across a range of sites with a pooled average PPV (0.96). The False Positive rate or weighted mean error (FP) of 3.9% (2.9% - 5.3%.; 95% CI) was low. IMPLICATIONS FOR FUTURE PRACTICE: A strategic, interprofessional approach in the implementation of an image alert combined with a Radiographer Comment can be adapted across a variety of hospital settings for ED and other departments.

MR imaging of the brain in patients cured of acute lymphoblastic leukemia--the value of gradient echo imaging.

BACKGROUND AND PURPOSE: Hemosiderin and white matter lesions are 2 of the most common neurologic complications found on MR imaging that may be related to cranial irradiation and intrathecal methotrexate (MTX) therapy in childhood acute lymphoblastic leukemia (ALL). We evaluated the brains of patients previously treated for ALL with cranial irradiation and intrathecal MTX with MR imaging and tested the hypothesis that these patients have more MR evidence of central nervous system (CNS) injury than control patients who are in complete remission following systemic chemotherapy without cranial irradiation. METHODS AND MATERIALS: ALL patients recruited from a pediatric cancer center data base were examined at 1.5T by using T1-weighted, T2-weighted, gradient echo (GE), and fluid-attenuated inversion recovery sequences. Patients treated in childhood for solid extracranial neoplasms were used as controls. RESULTS: Lesions consistent with old hemorrhage were detected in 23 (55%) of the ALL patients and in none of the control patients (P <.001). Of the 62 hemorrhages detected on the GE images, only 9 (15%) were shown on T2-weighted images. White matter abnormalities were found in 2 ALL patients (5%) and one control patient (5%). Old infarcts were seen in 2 ALL patients (10%). CONCLUSION: Cerebral hemorrhages related to radiation-induced vascular malformations, most of which are asymptomatic, are much more common in survivors of childhood ALL than previously thought. GE sequencing is more sensitive in detecting hemorrhagic lesions than T2-weighted sequencing and is to be included in imaging protocols for follow-up study of patients after cerebral radiation therapy.

Successful resection of a congenital superior mesenteric arteriovenous malformation.

A 15-month-old male presented with severe gastrointestinal bleeding and heart failure. Imaging revealed a superior mesenteric artery arteriovenous malformation, associated with a congenital portosystemic shunt. The heart failure was cured by resection of the arteriovenous malformation.

Computed tomography versus bronchography in the diagnosis and management of tracheobronchomalacia in ventilator dependent infants.

AIM: To assess the relative accuracy of dynamic spiral computed tomography (CT) compared with tracheobronchography, in a population of ventilator dependent infants with suspected tracheobroncho-malacia (TBM). SETTING: Paediatric intensive care unit in a tertiary teaching hospital. PATIENTS AND METHODS: Infants referred for investigation and management of ventilator dependence and suspected of having TBM were recruited into the study. Tracheobronchography and CT were performed during the same admission by different investigators who were blinded to the results of the other investigation. The study was approved by the hospital research ethics committee, and signed parental consent was obtained. RESULTS: Sixteen infants were recruited into the study. Fifteen had been born prematurely, and five had cardiovascular malformations. In 10 patients there was good or partial correlation between the two investigations, but in six patients there was poor or no correlation. Bronchography consistently showed more dynamic abnormalities, although CT picked up an unsuspected double aortic arch. Radiation doses were 0.27-2.47 mSv with bronchography and 0.86-10.67 mSv with CT. CONCLUSIONS: Bronchography was a better investigation for diagnosing TBM and in determining opening pressures. Spiral CT is unreliable in the assessment of TBM in ventilator dependent infants. In addition, radiation doses were considerably higher with CT.

Long-term cerebral metabolite changes on proton magnetic resonance spectroscopy in patients cured of acute lymphoblastic leukemia with previous intrathecal methotrexate and cranial irradiation prophylaxis.

PURPOSE: To evaluate the long-term brain metabolite changes on (1)H-MRS in acute lymphoblastic leukemia (ALL) patients who had intrathecal methotrexate (ITMTX) and cranial irradiation (CRT) for central nervous system (CNS) prophylaxis against CNS relapse. METHODS AND MATERIALS: Thirty-seven ALL patients (12 females, 25 males) with history of ITMTX and CRT for CNS prophylaxis were studied. Age ranges at the time of diagnosis and at magnetic resonance examination were 0.8-13 years and 12-27 years, respectively. The interval since diagnosis was 5.6-19 years. T2-weighted and gradient-recalled echo (GRE) magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy ((1)H-MRS) were performed to assess brain injury. RESULTS: On MRI, 3 leukoencephalopathy (LEP) and 1 infarct were detected. Twenty-two patients had evidence of hemosiderin. On (1)H-MRS no statistically significant difference in choline (Cho)/creatine (Cr) and N-acetylaspartate (NAA)/Cr was associated with LEP. A lower Cho/Cr (p = 0.006) and NAA/Cr (p = 0.078) was observed in brains with hemosiderin. Linear-regression analysis showed no statistically significant relationship between NAA/Cr or Cho/Cr with age at diagnosis, but there was a statistically significant decreasing trend of NAA/Cr and Cho/Cr with the interval since diagnosis. CONCLUSION: Long-term brain injury in ALL survivors after CNS prophylaxis with ITMTX and CRT was reflected by decreasing NAA/Cr and Cho/Cr with the interval since diagnosis. The lower Cho/Cr associated with hemosiderin but not LEP suggested a different pathophysiology for these brain lesions.

Burton skeletal dysplasia: the second case report.

We describe a 2-year-old girl with clinical and radiological findings of Burton skeletal dysplasia. This rare disorder shows some similarities to Kniest dysplasia. Short stature, joint stiffness, microstomia, and pursed lips are characteristic clinical findings. Platyspondyly with cervical kyphosis, but no coronal clefts, and bowing of the long bones are distinctive radiographic findings.

Cat-scratch disease with an extraaxial mass.

CT and MR imaging of the brain and gallium-67 scintigraphy showed an enhancing, gallium-avid mass in the left middle cranial fossa of a 10-year-old girl. Craniotomy revealed an inflammatory mass related to the left trigeminal nerve. The lesion contained rodlike bacteria, and serologic tests were positive for cat-scratch disease. Neurologic involvement in cat-scratch disease is uncommon, and the presence of organisms in neural tissue has not been reported.

Hemangioendothelioma of the parotid gland in infants: sonography and correlative MR imaging.

BACKGROUND AND PURPOSE: Hemangioendothelioma is the most common parotid gland tumor of childhood, and is diagnosed on clinical grounds, supported by imaging findings. Previous work has suggested that MR is the best imaging technique for assessment of parotid hemangioendothelioma. Demonstration of a reliable sonographic appearance would reduce the need for MR imaging in infants with this lesion. METHODS: We performed high-frequency sonography (including color Doppler and power Doppler imaging) in three patients, each with a diagnosis of parotid hemangioendothelioma confirmed by clinical follow-up. Two patients were also examined with MR imaging and labeled red cell scintigraphy. RESULTS: All sonographic studies showed a homogeneous mass enlarging and replacing most or all of the visualized parotid gland, with a lobular internal structure, fine echogenic internal septations, and a mildly lobulated contour. Color Doppler and power Doppler imaging showed extremely high vascularity within the mass. Correlative MR images in two infants showed a well-defined lesion with uniform intense contrast enhancement. Labeled red cell scintigraphy showed a well-defined area of intense activity. CONCLUSION: In the presence of a typical clinical history, sonography and clinical follow-up alone may prove to be sufficient for safe management of parotid hemangioendothelioma. MR imaging or labeled red cell scintigraphy may only be required if the sonographic features are atypical. These findings require confirmation in a larger series of patients.

How sensitive is ultrasound in the detection of renal scars?

The English language medical literature was reviewed to determine the strength of the published evidence for the assertion that dimercaptosuccinic acid scintigraphy (DMSA) is superior to ultrasound (US) in the detection of established renal scarring in children. The MEDLINE database was used to identify papers published between 1985 and 1997 that claimed to be concerned with the detection of scars, and contained sufficient information to permit calculation of the sensitivity of US relative to DMSA. Only 10 studies were identified. The sensitivity of US for scarring, using DMSA as a gold standard, ranged from 37% to 100%, and its specificity from 65% to 99%. These wide ranges mean that evaluation of the role of US in the detection of scarring remains controversial. All papers contained methodological flaws. Allowing for these, the sensitivity of US appears to be acceptable. Further research that avoids these methodological problems is required.

Vesicoureteric reflux and renal scarring in Chinese children.

Vesicoureteric reflux (VUR) and renal scarring are commonly found in children with urinary tract infection (UTI). The prevalence of VUR and scarring may vary between racial groups. There are no published data on the prevalence of VUR and scarring in Chinese children with UTI. A retrospective, single-institution study was made of Hong Kong Chinese children aged less than 5 years with a documented UTI investigated by both micturating cystourethrography and dimercaptosuccinic acid scintigraphy. VUR was identified in 39% of 93 Chinese children with UTI. Renal scarring was present in 28% of boys, which is comparable with published data on Western children. Scarring appears to be less common in Chinese girls with UTI (11%) than in Western girls (30-38% from published data), and its severity is poorly related to VUR grade. There is a significant dependency relationship between grade of VUR and degree of scarring in Chinese boys (p < 0.05). In conclusion, renal scarring appears to be relatively uncommon in Chinese girls. The correlation between grade of VUR and degree of scarring in Chinese boys suggests a relationship, but provides no evidence about the direction of causation.

The use of ultrasound in the venous assessment of lower limb Klippel-Trenaunay syndrome.

Colour Doppler ultrasound was used to study the affected lower limbs of a group of patients with the Klippel-Trenaunay syndrome. The abnormal lateral venous channel characteristic of this syndrome was examined, together with its deep venous connections and deep venous patency established if present. Seventeen patients (18 limbs) were studied. Ultrasound demonstrated calf vein patency in 15 out of 17 limbs and deep venous patency from the popliteal vein to the inferior vena cava in 17 out of 17 limbs. Ultrasound was also effective in demarcation of the abnormal lateral venous channel, together with its deep connections (13 out of 16 limbs). We conclude that ultrasound is superior to the more established venographic techniques in the investigations of patients with the Klippel-Trenaunay syndrome. From this study, the deep venous aplasia said to occur in the condition would appear to be less common than previously thought.

A technique for enhancing union of allograft to host bone.

The aim of limb-salvage surgery in malignant bone tumours in children is to restore function and eradicate local disease with as little morbidity as possible. Allografts are associated with a high rate of complications, particularly malunion at the allograft-host junction. We describe a simple technique which enhances union of allograft to host bone taking advantage of the discrepancy in size between the adult allograft and the child's bone. This involves lifting a flap of periosteum before resection from the host bone, which is then telescoped into the allograft medullary canal, which may require internal burring or splitting, for a distance of 1.5 to 2 cm and covering the bone junction with the periosteal flap. This is more stable than conventional end-to-end opposition. For each centimetre of telescoping the surface area available for bony union is increased more than three times. The periosteal flap also augments union. Additional surface fixation with a plate and screws is not necessary. We have used this technique in nine children, in eight of whom there was complete union at a mean of 16 weeks. Delayed union, associated with generalised limb osteoporosis, occurred in one. Early mobilisation, with weight-bearing by three weeks, was possible. There was only one fracture of the allograft.

Immediate ambulation after amputation.

It has long been realized that many patients who have their lower limbs amputated have a difficult and prolonged period of rehabilitation consequent upon stump difficulties. We would like to describe a scheme which eliminates, to a large degree, many of these troubles. The "immediate" walking of a patient who has had his lower limb amputated was first suggested by Professor Marian Weiss of Poland during the Ninth World Congress of the International Society for Rehabilitation of the Disabled held in Copenhagen in 1963.

Inferior vena cava filters--percutaneous insertion?

The incidence of deep venous thrombosis (DVT) and pulmonary embolism (PE) among Chinese is much lower than in Caucasians. The total number of inferior vena cava (IVC) filters inserted in regional hospitals in Canada (about 700 beds in Toronto General Hospital) and Hong Kong (about 1,250 beds in Pamela Youde Nethersole Eastern Hospital) also reflects this. Thirty-six IVC filters were deployed in Toronto General Hospital, compared to 8 IVC filters inserted in Pamela Youde Nethersole Eastern Hospital from August 1997 to September 1998. Despite this, the physician may encounter patients with thromboembolic disease who require inferior vena cava interruption. The usual indication will be pulmonary embolism with contraindications to, or failure or complications of, anticoagulation therapy. It is important for angiographers to be familiar with the technique of percutaneous insertion of IVC filters. The types of IVC filters, techniques of insertion and guidelines relating to the choice of a filter would be discussed.

Embolisation of a renal artery pseudoaneurysm in a patient with renal malrotation and chronic aortic dissection.

INTRODUCTION: Renal artery pseudoaneurysms may arise as a complication of percutaneous nephrolithotomy (PCNL). Prompt recognition and treatment is essential to arrest haemorrhage which may be life threatening. CLINICAL PICTURE: A patient with chronic aortic dissection and malrotated right kidney underwent PCNL for right renal calculus. He developed delayed gross haematuria. TREATMENT: Angiography showed a pseudoaneurysm arising from one of two right renal arteries, which in turn arose from the false lumen of the aortic dissection. The supplying artery was successfully embolised. CONCLUSION: Renal artery pseudoaneurysms can be successfully treated with prompt angiography and embolisation, even in the presence of renal malrotation and aortic dissection.

Bronchomegaly as a complication of fetal endoscopic tracheal occlusion. A caution and a possible solution.

Fetal medicine is developing rapidly and aims to improve the outcome for fetuses with congenital anomalies. Fetal endoscopic tracheal occlusion (FETO) has been developed for fetuses with congenital diaphragmatic hernia to counterbalance the compression of the lung by the abdominal viscera, preserving the pulmonary maturation. Because the perinatal morbidity and mortality of patients treated with FETO have decreased, new complications are emerging in the older survivors. Tracheomegaly has been reported to be a late complication of FETO, sometimes requiring tracheostomy. We report a case of bronchial dilatation after FETO and suggest an alternative surgical treatment.