Kokila Lakhoo

Comparison between open and thoracoscopic resection of congenital lung lesions.

PURPOSE This study compares the outcome between thoracoscopic and thoracotomy resection of congenital lung lesions. METHODS From November 2005 to August 2007, 14 consecutive cases of video-assisted thoracoscopic (VATS) lung resections have been performed in our institution. A retrospective review comparing these cases to the previous open thoracotomies for lung resection was performed. Intraoperative and early postoperative results were compared. RESULTS The mean age for VATS resection was 10 months compared with 7 months for thoracotomy. There were no major intraoperative complications. One case was converted from thoracoscopy to thoracotomy, and there was one anesthetic failed attempt of VATS resection, which was then performed open. Seven VATS resections and 6 thoracotomies were for congenital cystic adenomatous malformations. Intraoperative chest drains were used for all VATS resections but only 10 of the 14 thoracotomies, one of which developed a tension pneumothorax within hours of discharge. Perioperative outcomes including time to removal of chest drain, length of postoperative intravenous opioid requirement, and hospital stay were similar for both groups. Three had postoperative complications. Operative time was significantly lower in the thoracotomy group (124 minutes compared with 170 minutes, P < .05). The subgroup of congenital lobar emphysema had a particularly prolonged VATS resection time of 220 vs 155 minutes (P < .05). The thoracotomy group was more likely to receive adjuvant regional anesthesia (12 of 14 compared with 5 of 14). CONCLUSIONS Thoracoscopic resection of lung lesions results in longer operative time but is a safe and feasible alternative to open thoracotomy. Congenital lobar emphysema is a subgroup more challenging thoracoscopically, and it is recommended that these should be preselected for open surgery.

Management of congenital cystic adenomatous malformations of the lung

Congenital cystic adenomatous malformation of the lung (CCAM) is a rare lung lesion easily diagnosed on prenatal scan. The pathology of fetal lesions differs from postnatal lesion, hence the need for separate classifications during the different stages of development. Fetuses with CCAMs and hydrops have a poor prognosis and may be candidates for prenatal intervention where available. Most prenatally diagnosed CCAMs have a favourable outcome. Early surgery is required for symptomatic babies. Management of prenatally diagnosed asymptomatic lesions remains controversial, with the options of conservative management with CT scan surveillance or surgical excision. Surgical excision is favoured by many centres because of the risk of infection and malignant transformation if the CCAM remains in situ. Surgical outcomes are excellent.

Potency and stability of C terminal truncated human epidermal growth factor

INTRODUCTION Epidermal growth factor (EGF) is normally present as EGF1–53. A variety of C terminal truncated forms have been used in preliminary trials for treating gastrointestinal injury but their relative potency and stability when used in a clinical setting are unclear. Therefore, we compared the biological activity of recombinant EGF1–53, EGF1–52, EGF1–51, and the C terminal peptides EGF44–53 and EGF49–53. METHODS Purity of forms was confirmed by mass spectrometry. Bioactivity of the different EGF forms was determined using [methyl-3H] thymidine incorporation into primary rat hepatocytes and their ability to reduce indomethacin (20 mg/kg subcutaneously)/restraint induced gastric injury in rats. Stability of EGF peptides was determined by serial sampling from a syringe driver system containing EGF/4% albumin in saline. RESULTS Biological activity assays of EGF1–53, EGF1–52, and EGF1–51 gave almost identical thymidine uptake dose-response curves (maximal responses increasing baseline uptake from 4400 (600) cpm (mean (SEM)) to about 22 000 (2000) cpm when EGF was added at 1.6 nM). EGF44–53 and EGF49–53 did not stimulate 3H thymidine uptake. Control rats had 47 (4) mm2 damage/stomach, EGF1–51, EGF1–52, and EGF1–53 at 0.16 and 0.80 nmol/kg/h each reduced gastric injury by about 50% and 80%, respectively (both doses p<0.01 compared with control but no significant difference between the different forms). EGF was stable at room temperature for seven days but biological activity decreased by 35% and 40% at two and three weeks, respectively (both p<0.01). Exposure to light did not affect bioactivity. CONCLUSION EGF1–51and EGF1–52 are as biologically active as full length EGF1–53 but the C terminal penta- and decapeptides are ineffective. Clinical trials of EGF can probably use infusion systems for at least 48 hours at room temperature and with exposure to light, without reducing biological efficacy.

Magnetic Resonance Imaging of Intestinal Necrosis in Preterm Infants

Background and Objective. Noninvasive diagnosis of intestinal necrosis is important in planning surgery in preterm infants with necrotizing enterocolitis (NEC). We aimed to assess the potential of magnetic resonance imaging (MRI) for the diagnosis of intestinal necrosis. Study Participants and Methods. Abdominal MRI scans were performed in a group of preterm infants with suspected NEC and compared with surgical findings and to MRI results in a group of control infants. In addition, MRI was performed in 2 preterm infants with suspected NEC who did not require surgery. Results. Six infants with a median birth weight of 1220 g (range, 760-1770 g) and median gestational age at birth of 30 weeks (range, 28–34 weeks) were studied at a median postnatal age of 10 days (range, 4–19 days). Four infants had a bubble-like appearance in part of the intestinal wall, intramural gas, and an abnormal fluid level within bowel lumen. At surgery, NEC was found in 5 infants and sigmoid volvulus in 1. The site of the bubble-like appearance corresponded to the site of intestinal necrosis at surgery. Four control infants with a median birth weight of 1500 g (range, 730-2130 g) and a median gestational age of 31 weeks (range, 26–36 weeks) had abdominal MRI at a median postnatal age of 8 days (range, 4–70 days). None of the above findings were seen in any control infant. The bubble-like appearance was not seen in the 2 infants with suspected NEC who did not require surgery. Conclusion. Abdominal MRI allows the noninvasive diagnosis of bowel necrosis. This may aid the timing of surgical intervention in preterm infants with a clinical diagnosis of NEC. gangrene, ischemia, MRI, necrotizing enterocolitis.

Immunoreactive epidermal growth factor receptors are present in gastrointestinal epithelial cells of preterm infants with necrotising enterocolitis.

INTRODUCTION Epidermal growth factor (EGF) affects epithelial cell proliferation, differentiation and migration in the gastrointestinal tract of experimental animals, and increases proliferation when given intravenously to children with congenital microvillous atrophy or necrotising enteritis. The aim of the present study is to determine whether EGF receptors (EGFR) are present in the gut epithelium of preterm infants, and to discover whether neonatal necrotising enterocolitis (NEC) is associated with the absence of EGFR from mucosal cells. METHODS Tissues were taken from involved colon and small intestine of four preterm infants with NEC, and control tissues were taken from four other neonates who had laparatomies for congenital malformations. Sections of the tissues were examined histopathologically after treatment with a monoclonal antibody against the external domain of the EGFR (Zymed Laboratories, San Francisco, CA, USA). RESULTS Histopathological examination confirmed diagnosis of NEC in the involved bowel and controls showed appearance within normal limit. Immunoreactive EGFR were present on the epithelial cells of both the colon and small intestine, localised on the basolateral membrane of the cells of both subject and the controls. There was no apparent reduction in expression compared with controls. CONCLUSION NEC in preterm infants is not associated with absence of EGFR. The presence of EGFR in gut epithelial cells raises the possibility of using EGF for prophylaxis or treatment of NEC.

Staged reduction of gastroschisis using preformed silos: practicalities and problems

PURPOSE Previous single-center studies have reported favorable outcomes when preformed silos (PFS) are used for the staged reduction of gastroschisis. The aim of this study was to assess the frequency and nature of complications associated with PFS in a large population and provide an insight into the practicalities of their routine use. METHODS A retrospective review was carried out of all cases of gastroschisis managed with PFS in 4 UK neonatal surgical units for a 6-year period. RESULTS One hundred fifty infants were included, and 139 (92.7%) silos were applied at cot side (no sedation, n = 93). Median silo size was 4 cm, and time of application was 2.5 hours. Enlarging the defect by incision of fascia was required in 17 (11%). Defect closure was performed at a median of 4 days (0-47) with 93 (62%) being at cot side. Methods of closure were adhesive strips/dressings (n = 94), sutures (n = 48), and patch (n = 8). Discoloration of the viscera occurred in 16 (11%), managed successfully by simple methods (change of PFS, aspirating the stomach, or incision of the defect fascia) (n = 8), conversion to operative silo (n = 3), and operative reduction (n = 1). Four required bowel resection. Other complications included missed atresia (n = 5; 3.3%) and nectrotizing enterocolitis (n = 11; 7%). There were 5 deaths in the series (3.3%). CONCLUSIONS Staged reduction of gastroschisis with PFS is simple, convenient, and safe. The low rates of associated complications and mortality appear favorable when compared to infants managed with more traditional techniques. We recommend that PFS should be used for the routine management of gastroschisis.

Congenital lung lesions: classification and concordance of radiological appearance and surgical pathology

Congenital lung lesions are diagnosed antenatally in the majority of cases. Postnatal management includes chest radiography and CT-scanning, followed by either surgical resection or CT surveillance. Pre-operatively, lesions are often “labelled” as CCAM (and the Stocker classification incorrectly applied), sequestration, or lobar emphysema, and their frequent “hybrid” nature sometimes missed. The aim of our study was to correlate antenatal and postnatal radiological diagnoses and classification of congenital lung lesions with surgical and pathological outcome. Twenty-six consecutive cases of antenatally-diagnosed cystic lung lesions managed at our centre between January 2003 and April 2007 were reviewed. Diagnoses were: cystic adenomatoid malformations (CCAM) in 13 cases (50.0%), three bronchopulmonary sequestrations (11.5%), “hybrid” lesions in eight cases (30.8%), one isolated lobar emphysema (3.8%) and a normal lung segment with anomalous systemic supply (3.8%). Overall concordance rate between prenatal ultrasound and resection pathology was 61.5%, whereas CT findings correlated with pathology in 65.4% of cases. Incorrect radiological classification resulted in one morbidity, when an attempted thoracoscopic resection of a presumed CCAM had to be converted to an open procedure due to bleeding from an unsuspected anomalous vessel. The common embryological origin of these lesions, and the need for standardisation of reporting is discussed. We propose that lesions should be classified anatomically according to their nature, arterial supply and venous drainage in order to reduce discrepancies between radiological, intra-operative and pathological reporting.

Intravenous postoperative fluid prescriptions for children: A survey of practice

BackgroundPostoperative deaths and neurological injury have resulted from hyponatraemia associated with the use of hypotonic saline solutions following surgery. We aimed to determine the rates and types of intravenous fluids being prescribed postoperatively for children in the UK.MethodsA questionnaire was sent to members of the British Association of Paediatric Surgeons (BAPS) and Association of Paediatric Anaesthetists of Great Britain and Ireland (APAGBI) based at UK paediatric centres. Respondents were asked to prescribe postoperative fluids for scenarios involving children of different ages. The study period was between May 2006 and November 2006.ResultsThe most frequently used solution was sodium chloride 0.45% with glucose 5% although one quarter of respondents still used sodium chloride 0.18% with glucose 4%. Isotonic fluids were used by 41% of anaesthetists and 9.8% of surgeons for the older child, but fewer for infants. Standard maintenance rates or greater were prescribed by over 80% of respondents.ConclusionMost doctors said they would prescribe hypotonic fluids at volumes equal to or greater than traditional maintenance rates at the time of the survey. A survey to describe practice since publication of National Patient Safety Agency (NPSA) recommendations is required.

The Global Paediatric Surgery Network: A Model of Subspecialty Collaboration Within Global Surgery

Attention to surgical conditions in lowand middle-income countries (LMICs) has increased in recent years. Because half of the population in the world’s poorest countries are children [1], paediatric surgical conditions compose a significant proportion of the global burden of disease (BoD), and there are critical shortages in workforce and skills to treat these diseases in LMICs. Several populationbased studies have highlighted the magnitude of the need for paediatric surgery and the limited capacity, both in human resources and in infrastructure, to tackle the problem [2, 3]. Africa, in particular, has a grave shortage of paediatric surgeons. The number of fully trained paediatric surgeons ranges from 1 in Malawi (population 13 million) to 120 in Egypt (population of 80 million). In more than

A novel mutation in the endothelin B receptor gene in a patient with Shah-Waardenburg syndrome and Down syndrome

Editor—A case of Down syndrome, total gut Hirschsprung disease (HSCR), and segmental hypopigmentation is described in a neonate presenting with bowel obstruction. In addition to having trisomy 21, this patient was homozygous for a novel mutation in the endothelin B receptor ( EDNRB ) gene. A term female infant with karyotype 47,XX,+21 presented on day 3 of life with bowel obstruction. She was of Somali origin and had large areas of segmental hypopigmentation affecting the left side of the face and trunk, the left upper limb, including the hair follicles, and had white scalp hair. At laparotomy she had an annular pancreas, duodenal web, and inspissated meconium in the ileum and colon, for which she underwent a duodenoduodenostomy. Histology of the rectal biopsy and appendix was inconclusive at this stage. Intestinal obstruction persisted and on day 20 she underwent a further laparotomy, which showed breakdown of the original anastomosis. Intraoperative frozen sections showed complete aganglionosis throughout the entire large and small …