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.

Congenital lung malformations: an ongoing controversy.

INTRODUCTION: Congenital lung malformations are rare lesions that are most commonly diagnosed antenatally. Management of such lesions, particularly those that are asymptomatic, remains controversial. We undertook a survey to ascertain current practice of surgeons in the UK and Ireland. METHODS: All consultant members of the British Association of Paediatric Surgeons were asked to complete a survey on congenital lung malformations with respect to antenatal management, symptomatic and asymptomatic lesions, and operative techniques. RESULTS: Responses were received from 20 paediatric surgical centres and highlighted the ongoing variability in management of such lesions, particularly those that are asymptomatic. Twenty per cent of surgeons never resect an asymptomatic lesion and twenty-four per cent always do. The remainder intervene selectively, with size being the most commonly stated indication. Most resections are undertaken via thoracotomy although 35% of surgeons use thoracoscopy for some procedures. CONCLUSIONS: National data based on congenital anomaly registers are needed to determine the natural history of these malformations and to guide future management.

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.

Meconium obstruction of prematurity.

Meconium obstruction of prematurity is an entity primarily affecting very low birthweight or extremely low birthweight babies causing low intestinal obstruction. Its presence may at best delay establishment of enteral feeding and compromise nutrition and at worst lead to mechanical obstruction requiring surgery or to intestinal perforation. There are considerable challenges in the recognition, diagnosis and management of this condition. Awareness of the disease and understanding of its pathogenesis may lead to early detection of affected babies and allow proactive measures to decrease the associated morbidity and mortality.

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.

Neonatal spontaneous colonic perforation due to cystic fibrosis.

We report two cases of spontaneous colonic perforation associated with cystic fibrosis (CF) in the neonatal period. Both presented with an acute abdomen soon after birth and required laparotomy and stoma formation. Colonic perforation as an initial presentation of CF is reviewed and management options are discussed.

Recurrent neonatal gastro-intestinal problems after spontaneous intestinal perforation.

To identify intestinal complications during the neonatal period following spontaneous isolated intestinal perforation (SIP). A retrospective case notes review was undertaken of all patients with a diagnosis of SIP, confirmed at laparotomy or post-mortem, admitted between January 2000 and January 2005. Patients with confirmed gastric perforation were excluded as were patients with proven necrotising enterocolitis (NEC) or suspected, but not confirmed, SIP. Seventeen patients, median gestation 27 weeks and median birth weight 780 g, were treated by drain alone (1), drain and later laparotomy (4) or primary laparotomy (12). Eight patients required enterostomy formation at primary laparotomy (1 jejunostomy, 1 colostomy and 6 ileostomy). Five babies died in the neonatal period and three later in the first year. Nine patients (53%) had ten subsequent episodes of intestinal pathology requiring surgical intervention between 5 and 136 days later comprising early recurrent isolated perforation (2), NEC (3), milk curd obstruction with or without perforation (3) and adhesion obstruction (2). Secondary surgery involved laparotomy in eight patients, five of whom required formation or re-formation of a stoma, and palliative drain insertion in one patient. Recurrent intestinal pathology requiring surgical intervention during the neonatal period occurred in 53% of babies with SIP. Surgeons and neonatologists should be aware that this group of patients are prone to further intestinal pathology.

A technique to demonstrate external haemorrhoids.

Although uncommon in children, haemorrhoids are one of the causes of a protruding anal lesion and may be confused with rectal prolapse or prolapse of a rectal polyp. The lesions may not be obvious when the child is anaesthetised because of lack of straining. This may prevent accurate diagnosis and impede identification of the lesion if surgery is being attempted. The authors report 3 cases where a 20 F Foley catheter with 30 ml balloon was inserted rectally and gentle traction applied to reproduce the raised venous pressure generated during straining. On each occasion external haemorrhoids could be demonstrated as the underlying pathology.

The effect of antenatal diagnosis on the management of small bowel atresia.

The aim of this study was to determine whether babies with small bowel atresia (SBA) diagnosed antenatally followed a different course from those diagnosed postnatally. We reviewed the records of neonates admitted to a single institution between 1985 and 2000 with a diagnosis of SBA. Thirty-nine neonates presented with SBA, with antenatal diagnosis (AND) being made in 12 (31%). There was no difference between the AND and postnatal diagnosis (PND) groups in terms of gestational age at birth, but the AND group had a lower mean birth weight. The babies in the AND group were operated on more quickly than those in the PND group. Ten out of 12 (83%) AND patients required parenteral nutrition compared with 12 out of 27 (44%) in the PND group, and the AND group had a significantly longer mean length of stay and spent a longer time on parenteral nutrition than the PND group.

Exclusion of androgen insensitivity syndrome in girls with inguinal hernias: current surgical practice.

To review the current approach of paediatric surgeons to the exclusion of androgen insensitivity syndrome (CAIS) in girls with inguinal hernias (IH), a questionnaire was sent to all specialist paediatric surgeons in the United Kingdom and Ireland asking if they exclude CAIS, how they exclude it, and what they say to parents preoperatively. In all, 32 surgeons responded (29%); 41% made no attempt to exclude CAIS because they thought the incidence was too low to justify exclusion; 19(59%) excluded CAIS at the time of surgery by assessment of the internal genitalia. Only 1 performed karyotyping primarily, and then only for bilateral IH. Although most would proceed to karyotyping if the primary assessment suggested CAIS, some would not. Of those who exclude CAIS, only 1 mentions CAIS preoperatively, 6 others mention gonadal inspection, and 12/19 (63%) make no comment. Thirty-one surgeons agreed to take part in a prospective study to define the incidence of CAIS in girls with IH. It is concluded that surgeons who exclude CAIS in girls with IH adopt different assessment methods, some of which may be unreliable. However, many do not attempt to exclude CAIS, believing the incidence to be too low. As the health and medicolegal consequences of failing to exclude CAIS may be considerable, surgeons should consider changing their practice. A prospective study should be undertaken to determine the incidence of CAIS in girls with IH.

The ascending testis: is late orchidopexy due to failure of screening or late ascent?

It is widely recommended that infant boys with undescended testes (UDT) should be referred for surgical opinion before the age of 18 months. To attempt to identify the reasons why the mean age at orchidopexy in our institution was as high as 5.5 years, a retrospective review of the screening history, examinations, and management of boys over the age of 3 years at the time of orchidopexy was undertaken by reference to community, general practitioner, and hospital records. In 36 children where hospital and community records giving information prior to referral were available, the UDT had previously been documented on at least one occasion as descended in 24 children or retractile in 10. In 1 child there was delayed referral, and in another, there was operative delay. It is concluded that there is either frequent failure of the Child Health Surveillance screening programme, or that late ascent of a testis previously sited in the scrotum is a common occurrence. We recommend further prospective studies to clarify this latter phenomenon.

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.

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.

Management of antenatally diagnosed pulmonary sequestration associated with congenital cystic adenomatoid malformation.

BACKGROUND: Sequestration with associated cystic adenomatoid malformation is rare. A study was undertaken to determine whether pulmonary sequestration associated with congenital cystic adenomatoid malformation has a more favourable natural history than that of sequestration without associated cystic adenomatoid malformation. METHODS: An outline of the postnatal work up leading to the management of extralobar or intralobar pulmonary sequestration with congenital cystic adenomatoid malformation diagnosed antenatally as pulmonary malformation is presented and the indications for surgical intervention are discussed. RESULTS: In five infants in whom an antenatal ultrasound scan had detected a congenital lung malformation at 18-19 weeks gestation a final diagnosis of extralobar or intralobar pulmonary sequestration with congenital cystic adenomatoid malformation was made postnatally. Postnatal ultrasound and computerised axial tomographic scans confirmed the diagnosis of sequestration by delineating anomalous vascular supply. Cystic changes were also observed in the basal area of the sequestration in all patients. Four children remained asymptomatic and one infant presented at 10 months of age with pneumonia. The mean age at surgical resection was 6.8 months (range 2-10). Histopathological examination confirmed intralobar pulmonary sequestration with associated Stocker type 2 congenital cystic adenomatoid malformation in two patients and extralobar pulmonary sequestration with associated Stocker type 2 congenital cystic adenomatoid malformation in three patients. The mean period of follow up was four years (range 1-8). The children remain well and are developing normally. CONCLUSIONS: The importance of seeking an anomalous blood supply in children with congenital lung lesions is emphasised. Pulmonary sequestration and congenital cystic adenomatoid malformation probably share a common embryogenesis despite diverse morphology. The natural history of antenatally diagnosed lung masses is variable. Early postnatal surgical resection of pulmonary sequestration with cystic adenomatoid malformation is recommended. Surgical excision should be conservative, sparing the normal lung parenchyma.

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.

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.

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.

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.