Contemporary management and outcomes for infants born with oesophageal atresia.

BACKGROUND: Reports on the management and outcome of rare conditions, such as oesophageal atresia, are frequently limited to case series reporting single-centre experience over many years. The aim of this study was to identify all infants born with oesophageal atresia in the UK and Ireland to describe current clinical practice and outcomes. METHODS: This was a prospective multicentre cohort study of all infants born with oesophageal atresia and/or tracheo-oesophageal fistula in 2008-2009 in the UK and Ireland to record current clinical management and early outcomes. RESULTS: A total of 151 infants admitted to 28 paediatric surgical units were identified. Some aspects of perioperative management were universal, including oesophageal decompression, operative technique and the use of transanastomotic tubes. However, there were a number of areas where clinical practice varied considerably, including the routine use of perioperative chest drains, postoperative contrast studies and antireflux medication, with each of these being employed in 30-50 per cent of patients. There was a trend towards routine postoperative ventilation. CONCLUSION: The prospective methodology used in this study can help identify practices that all surgeons employ and also those that few surgeons use. Areas of clinical equipoise can be recognized and avenues for further research identified.

Scope and feasibility of operating on the neonatal intensive care unit: 312 cases in 10 years.

PURPOSE: To report the scope, feasibility and learning experience of operating on neonates on the neonatal intensive care unit (NICU). METHODS: (1) Review of all NICU operations performed by general neonatal surgeons over 10 years; (2) 6-month prospective comparison of procedures performed in NICU or operating room; (3) structured interviews with five surgeons with 1-13 years experience of operating on NICU. RESULTS: 312 operations were performed in 249 infants. Median birth weight was 1,494 g (range 415-4,365), gestational age 29 weeks (22-42), and age at operation 25 days (0-163). Nearly half (147) were laparotomy for acute abdominal pathology in preterm, very low birth-weight infants There were no surgical adverse events related to location of surgery. Surgeon satisfaction with operating on NICU for this population was high (5/5). Several factors contribute to making this process a success. CONCLUSIONS: This is the largest reported series of general neonatal surgical procedures performed on NICU. Operating on NICU is feasible and safe, and a full range of neonatal operations can be performed. It removes risks associated with neonatal transfer and is likely to reduce physiological instability. We recommend this approach for all ventilated neonates and urge neonatal surgeons to operate at the cotside of unstable infants.

Trans-anastomotic tubes reduce the need for central venous access and parenteral nutrition in infants with congenital duodenal obstruction.

PURPOSE: To determine the effect of trans-anastomotic tube (TAT) feeding on outcome following repair of congenital duodenal obstruction (CDO). METHODS: Retrospective comparative study of all infants with CDO over 10 years. Data are median (range). Mann-Whitney U test and Fisher's exact test were used. RESULTS: Of 55 infants with CDO (48 atresia, 7 stenosis), 17 were managed with a TAT, 38 without. Enteral feeds were commenced earlier in infants with a TAT compared to those without (TAT 2 days post-repair [1-4] vs. no-TAT 3 days post-repair [1-7]; p = 0.006). Infants with a TAT achieved full enteral feeds significantly sooner than those without (TAT 6 days post-repair [2-12] vs. no-TAT 9 days post-repair [3-36]; p = 0.005). Significantly fewer infants in the TAT group required central venous catheter (CVC) placement and parenteral nutrition (PN) than in the no-TAT group (TAT 2/17 vs. no-TAT 28/38, p < 0.0001). There were six CVC-related complications (5 infections, 1 PN extravasation) and four TATs became displaced and were removed before achieving full enteral feeds. One infant with a TAT with trisomy 21 and undiagnosed Hirschsprung disease developed an anastomotic leak and jejunal perforation requiring re-operation. CONCLUSIONS: A TAT significantly shortens time to full enteral feeds in infants with CDO significantly reducing the need for central venous access and PN.

The management of bilious vomiting in the neonate.

Bilious vomiting is synonymous with intestinal obstruction, be it functional or anatomical. In the neonate it may be due to congenital malformations of the gastrointestinal tract or develop due to acquired conditions, particularly intestinal complications associated with prematurity. This review considers the congenital malformations that may present with bilious vomiting and explores the diagnostic dilemmas faced in the preterm infant. The difficult issue of the need to exclude malrotation in term infants with bilious vomiting and the consequences of time-critical transfer is discussed.

Tympanic membrane displacement testing in regular assessment of intracranial pressure in eight children with shunted hydrocephalus.

OBJECT: The authors assessed the accuracy and repeatability of the tympanic membrane displacement (TMD) test, an audiometric technique that is used to evaluate changes in intracranial pressure (ICP) in children with shunted hydrocephalus. METHODS: A prospective comparative evaluation of 31 clinical episodes of shunt malfunction was made by using the serial TMD test and direct ICP measurement in eight children with shunted hydrocephalus between January 1995 and February 1996. The volume displacement of the tympanic membrane (Vm) on stapedial contraction was inward for raised ICP in 11 instances and ranged from -120 to -539 nl (mean -263.5 nl). This was confirmed by direct ICP monitoring, which showed values ranging from 20 to 30 mm Hg (mean 26 mm Hg). The TMD test measurement (Vm) in 18 instances of low ICP ranged from 263 to 717 nl (mean 431.3 nl); this was corroborated by direct ICP measurement, which ranged from 3 to 7 mm Hg (mean 4.2 mm Hg). The normal baseline Vm values obtained when patients were asymptomatic ranged from 98 to 197 nl (mean 110 nl). As a noninvasive diagnostic tool used in predicting changes in ICP, the TMD test had a sensitivity of 83% and specificity of 100%. The positive predictive value of the test was 100% and the negative predictive value was 29%. CONCLUSIONS: The TMD test can be used on a regular basis as a reproducible investigative tool in the assessment of ICP in children with shunted hydrocephalus, thereby reducing the need for invasive ICP monitoring. The equipment necessary to perform this testing is mobile. It will provide a useful serial guide to ICP abnormalities in children with shunted hydrocephalus.

Retrograde tunnel: a method for the fixation of long-term pediatric central venous catheters.

The fashioning of a subcutaneous tunnel has become an integral part of the placement of central venous catheters (CVC). Several methods have been described, but the long-term CVCs in pediatric practice pose special problems, particularly that of the patients continually testing the CVC's fixation. A polyurethane CVC is used that has not been previously described in pediatric usage and a retrograde tunneling technique has been developed which affords immediate and secure fixation.

Congenital diaphragmatic hernia: the prognostic significance of the site of the stomach.

Congenital diaphragmatic hernia is associated with a significant mortality, despite intensive treatment. The degree of pulmonary vascular hypoplasia is the main factor affecting mortality. Various features have been considered to determine the prognosis in these infants. In this study a series of 50 consecutive cases of left-sided diaphragmatic hernia has been reviewed. The site of the stomach (abdominal or intrathoracic), demonstrated radiologically and confirmed at operation, has been related to the final outcome (survival or death). An abdominal site is associated with an excellent prognosis (6.2% mortality), while an intrathoracic site is associated with a 58.8% mortality.

Renal agenesis: an acquired condition?

Renal agenesis is a relatively common congenital anomaly, although its etiology is unknown. It is clear that some solitary kidneys are the result of postnatal involution of multicystic dysplastic kidneys. The authors present a series of nine neonates with abnormal prenatal renal ultrasound findings; subsequent postnatal investigations showed an absent kidney. Five children had a prenatal diagnosis of multicystic dysplastic kidney (MDK) and two of hydronephrosis. In five cases (56%) there was a contralateral renal unit anomaly. It appears that MDK, and occasionally hydronephrosis, can involute prenatally, producing the postnatal appearance of renal agenesis.

Adverse outcome after prenatal diagnosis of gastroschisis: the role of fetal monitoring.

Fifty-seven fetuses with gastroschisis presented between 1982 and 1995 were studied by retrospective review of medical records. There were three late intrauterine deaths (IUD). Fetal distress, as determined by reduced fetal movements or abnormal cardiotopograph (CTG), was encountered in 23 of the 54 liveborn infants (43%), all of whom had delivery expedited either by emergency caesarean section (n = 19) or induction (n = 4). Six infants had abnormal neurological outcome: two died in the neonatal period of severe perinatal brain injury, neonatal fits were observed in four, two of whom developed cerebral palsy, and one died at the age of 7 years. All six of these infants had suffered fetal distress. If the three intrauterine deaths are included, 16% of all cases were associated with abnormal neurological outcome. The introduction of regular CTG monitoring from 32 weeks' gestation in 1990 increased the ability to detect fetal distress twofold. This resulted in a similar increase in obstetric intervention and an associated reduction in adverse neurological outcome. Pregnancies associated with gastroschisis should be considered at significant risk of fetal distress, which itself may culminate in late intrauterine death, neonatal death, or adverse neurological outcome. Careful, repeated fetal monitoring in the third trimester is indicated.

Gastric tube interposition as an esophageal substitute: comparative evaluation with gastric tube in continuity and gastric transposition.

BACKGROUND/PURPOSE: The aim of this study was to evaluate growth, hematologic and biochemical parameters, and histopathology after gastric tube interposition, gastric tube in continuity with or without posterior fundoplication, and gastric transposition in an experimental model. METHODS: Twenty-two postweaned, 28-day-old piglets were divided randomly into four groups: group 1 (n = 9) gastric tube interposition, group 2 (n = 4) gastric tube in continuity, group 3 (n = 5) gastric tube in continuity with posterior fundoplication, and group (n = 4) gastric transposition. The postoperative assessment included weekly measurement of weight, documentation of clinical symptoms, and deglutition difficulties. Hemoglobin, serum ferritin, albumin, globulins, total proteins, and red cell folate were measured. The pigs were killed and histopathologic assessment was made following a maximum observation period of 149 days. RESULTS: The four pigs with gastric transposition died within 96 hours postoperatively of respiratory embarrassment. The salient clinical features and histology are summarised. The salient clinical complications observed in the four groups were as follows. Group 1: vomiting (11%), minor leak (22%), and stricture (11%). There was no impairment of deglutition and the growth was normal. Group 2: vomiting (100%), excessive salivation (100%), and episodes of cyanosis (100%). Growth was impaired but there was no impairment of deglutition. Group 3: vomiting (80%), excessive salivation (80%), and episodes of cyanosis (20%). There was no impairment in deglutition or growth. Group 4: vomiting (100%), episodes of cyanosis (100%), and respiratory embarrassment (100%). Pigs in group 4 had to be sacrificed on day 3 or 4 postoperatively because of severe respiratory embarrassment, cyanosis, and presumed gastroesophageal reflux. At autopsy the anastomoses were intact, with no evidence of leak. The stomach and esophagus had good vascularity. Histopathology demonstrated esophagitis in 11% of the specimens in group 1, 100% of those in group 2, and 60% of those in group 3. Submucosal fibrosis was seen in 56% of group 1, 100% of group 2, and 80% of group 3. Hyperkeratosis was observed in 75% of group 2 and 40% of group 3 specimens. CONCLUSIONS: Gastric tube interposition in this animal model was associated with improved growth, fewer clinical complications, and fewer histopathologic changes than gastric tube in continuity with or without posterior fundoplication or gastric transposition.

Reflux nephropathy secondary to intrauterine vesicoureteric reflux.

In 107 infants with 182 antenatally diagnosed urinary tract anomalies, 24 had either unilateral (12) or bilateral (12) vesicoureteric reflux (VUR). The VUR was more common in boys (male to female ratio, 16:8) and usually severe (grades IV [16], III [10], II [4], and I [6]). Intravenous pyelography showed the changes of atrophic pyelonephritis in 10 refluxing units, and in another two with an associated pelviureteric junctional hydronephrosis. Lateral ectopia of the ureteric orifices was noted in six of these 10 refluxing renal units. Isotopic renography showed a reduction in function in nine of the 14 patients examined, ranging between 9% and 41%. (45% and above was considered within the normal range). Only two patients developed a urinary infection before intravenous pyelography or isotopic renography was performed, suggesting that renal changes noted were primary rather than secondary. Findings support the hypothesis that foetal VUR may be a contributing factor in the causation of atrophic pyelonephritis (foetal reflux nephropathy) observed in these patients.

Gastroschisis bowel in an intact exomphalos: implications for etiology and possible prevention.

The etiology of changes in the bowel in gastroschisis is unknown. A case of exomphalos is described, in which the bowel had a gastroschisis-like appearance. The possibility of a vascular mechanism as the cause is discussed.

Could the stomach site help predict outcome in babies with left sided congenital diaphragmatic hernia diagnosed antenatally?

The site of the stomach in 36 babies presenting postnatally with left sided congenital diaphragmatic hernia (CDH) was assessed as a predictor of outcome. Babies with a thoracic stomach had a higher mortality (P less than .0005), and more frequently developed significant persistent foetal circulation (PFC) (P less than .001), than babies in whom the stomach was normally sited. Normal stomach site was associated with 100% survival and only a 20% incidence of significant PFC. It is possible that stomach site may be the most accurate predictor of outcome in left-sided CDH diagnosed antenatally, and may thus help in planning perinatal and postnatal management. It may also open the door for prenatal surgical correction of CDH by predicting a poor prognostic group or, perhaps more importantly, by predicting those babies with a good prognosis in whom antenatal surgery should not be attempted.

Noninvasive assessment of portosystemic shunting in extrahepatic portal hypertension in rats.

A simple reproducible animal model of extrahepatic portal hypertension (EHPHT) has been developed in weanling Wistar rats using a two-stage ligation of the portal vein. This model consistently produces substantial collaterals, both portosystemic (hepatofugal) and portoportal (hepatopetal). Using dynamic hepatic scintigraphy (DHS) with 99mTechnetium sulphurcolloid, hepatopetal collateral flow was measured as the mesenteric fraction (MF) of total hepatic blood flow and compared with measurement of hepatofugal collateral flow (portosystemic shunting) following intraportal injection of radiolabeled microspheres. Strong and significant correlation between the two assessments was found with reduction in MF denoting increased portosystemic shunting (PSS). The technique of DHS has been used successfully in adults to assess compromised portal venous flow and is a simple noninvasive test to aid diagnosis, assessment, and follow-up of children with EHPHT.

Vitamin K coagulation status in surgical newborns and the risk of bleeding.

The vitamin K coagulation status in surgical newborns, who may be at increased risk of developing hypocoagulability and hemorrhage, has not previously been studied. Therefore, we measured the combined activity of the plasma vitamin K-dependent coagulation factors (Thrombotest), total prothrombin, PIVKA II, plasma vitamin K1, fibrinogen, D-Dimer, and platelets in 49 newborns admitted to a neonatal surgical intensive care unit. All infants had significant pathology, and treatment involved surgery in all but two. Twenty-three infants (47%) underwent surgery on two or more occasions. Intravenous or oral antibiotics were used in all patients and many received more than one course. All infants had vitamin K1 prophylaxis at birth. At day 0 (date of birth), the mean Thrombotest and total prothrombin levels were 51% (range, 20% to 100%) and 40% (range, 24% to 59%), respectively. Coagulation activity decreased on day 1 (P > .1) and was followed by a graduate increase in clotting activity, reaching normal adult levels (> 60%) at day 5 for Thrombotest and day 24 for total prothrombin. Only three infants had a Thrombotest less than 20%. PIVKA II was detected in 20 cases (41%). However, levels were within normal limits (< 0.9%) in 17 of these, and between 1.0 and 4.8% in the remaining three infants. There was no relationship between elevated PIVKA levels and coagulation activity in these patients. Plasma vitamin K1 was very high, particularly in the first days of life.(ABSTRACT TRUNCATED AT 250 WORDS)

Gastric tube graft interposition as an oesophageal substitute.

BACKGROUND: Use and techniques of oesophageal replacement for long gap oesophageal atresia are still evolving. Gastric tube graft interposition as an oesophageal substitute was evaluated on an animal model. METHODS: Twenty-three postweaned, 28-day-old-piglets were used as the experimental animals. Isoperistaltic gastric tube interposition based on the right gastroepiploic vessels was performed at 28 days of life. Postoperative evaluation included weekly measurement of weight, clinical assessment for gastrointestinal and respiratory complications and deglutition difficulties. Haemoglobin, serum ferritin, albumin, globulins, total proteins and red cell folate were assayed. Pigs were killed and analysed histopathologically following a maximum observation period of 149 days. RESULTS: Growth of the pigs was normal. Deglutition was not impaired in 16 pigs (89%). Minor leak was diagnosed in three pigs (17%), which was successfully managed conservatively. Anastomotic stricture was seen in two pigs (11%). Graft necrosis was not seen. Gross histology showed the absence of hypertrophy, redundancy and kinking of the interposed gastric tube graft. Microscopically non-erosive oesophagitis was seen in three pigs (17%), ulcerative oesophagitis was seen in two pigs (11%) and submucosal fibrosis was seen in seven pigs (39%). Dysplasia or Barrett's oesophagitis was not observed at the end of animal growth. CONCLUSIONS: Gastric tube graft interposition is an immediate ideal oesophageal substitute due to fewer complications, probable absence of gastro-oesophageal reflux in the majority by histology, and absence of dilatation and redundancy of the interpose tube. The oesophageal substitute adequately met the nutritional needs for growth and development in the animal model.

Extra-lobar intra-abdominal pulmonary sequestration.

An ultrasonography scan detected a left upper quadrant abdominal mass in a fetus of 20 weeks gestation. The mass was confirmed by a postnatal ultrasonography scan, computerized tomography and magnetic resonance imaging prior to surgery. The sonographic characteristic of this lesion was that of a homogenous echogenic mass embedded in the left diaphragmatic crus, which moved with respiration. Histopathological evaluation of the mass demonstrated alveolar and bronchial tissue, with its own visceral pleural covering corroborating the diagnosis of extra-lobar intra-abdominal pulmonary sequestration. Surgical excision is a valid recommendation because of the uncertainty of preoperative diagnosis of this uncommon malformation.

Quantitative assessment of intracranial pressure by the tympanic membrane displacement audiometric technique in children with shunted hydrocephalus.

The objective of this prospective study was to compare the clinical features at presentation, tympanic membrane displacement test results and direct intracranial pressure measurements in children with shunted hydrocephalus to procure a quantitative measure of the intracranial pressure by tympanic membrane displacement test. A prospective comparative evaluation of 61 clinical episodes of shunt malfunction was assessed by volume displacement of the tympanic membrane and direct intracranial pressure measurements in 40 patients with shunted hydrocephalus between January 1995 and June 1996. The volume displacement of the tympanic membrane (Vm) on stapedial contraction was inward for raised intracranial pressure in 27 episodes and ranged from -120 nl to -506 nl (mean = -250 nl). This was confirmed by direct intracranial pressure monitoring, which ranged from 23 to 40 mm Hg (mean = 29 mm Hg). The tympanic membrane displacement test measurement in 30 episodes of low intracranial pressure ranged from +263 nl to +810 nl (mean = +530 nl), and this was corroborated by direct intracranial pressure measurement ranging from 1 to 6 mm Hg (mean = 3.8 mm Hg). The normal baseline Vm values obtained when the subjects were asymptomatic ranged from +58 nl to +175 nl (mean = +115 nl). The tympanic membrane displacement test as a non-invasive diagnostic tool in predicting changes in intracranial pressure had a sensitivity of 93% and specificity of 100%. The predictive value of the test was 100%, and the negative predictive value was 73%. The kappa statistical analysis was used to measure the agreements between the groups. The strength of the agreement was very good, kappa = 0.88 and the P value was < 0.001. The objective measure of intracranial pressure by tympanic membrane displacement test with the Vm value of -200 nl and more negative was indicative of raised intracranial pressure and a Vm value of +200 nl and greater, for low intracranial pressure. The intracranial pressure measurements made on an individual subject basis were reliable and accurate. The test can therefore be used for regular assessment of shunted hydrocephalics to enable correlation of intracranial pressure with symptoms in individual patients.

Thoracic wall lipoblastoma: a case report and review of histopathology and cytogenetics.

A rare case of a successfully excised intra- and extrathoracic lipoblastoma of the anterior chest wall in a 13-month-old female infant is reported. Histopathology and cytogenetical analysis established the diagnosis of a lipoblastoma. The differential diagnosis, histology and cytogenetical evaluation of lipomatous neoplasms are discussed. Karyotypic analysis may be of use in diagnostically difficult cases owing to the characteristic alterations in 18q11-13. A complete resection of lipoblastomas is feasible and advantageous with no need for a mutilating radical excision.

Long-term assessment of intracranial pressure using the tympanic membrane displacement measurement technique.

The tympanic intracranial pressure (ICP) measurement technique was used to assess intracranial pressure over several months in two patient populations. In the first study, 43 shunted hydrocephalic children, aged 4-17 years, were tested over a period of 18 months when clinically well. Of these 11 (26%) were later admitted with symptoms suggesting acute shunt blockage. The tympanic ICP measurement correlated with clinical and/or operative findings in 10 cases. In the second study, illustrated by a case report, repeated testing over a period of 5 months in children with chronic symptoms suggestive of periods of increased ICP, demonstrated a correlation between symptoms and ICP and helped influence management decisions. Tympanic ICP measurement in shunted children is a valuable tool in the assessment of acute and chronic shunt malfunction. Serial testing was shown to be clinically useful in the long-term management of these patients as a diagnostic indicator of pressure variation and shunt dysfunction.