Establishing benchmarks for the outcome of herniotomy in children.

BACKGROUND: Although complication rates following herniotomy in children are low, reported rates vary. The aim of this study was to propose desirable outcome measures that can be used by surgeons to assess the quality of their service. METHODS: A review of prospectively collected data on all hernias operated on by the Christchurch-based paediatric surgical service was undertaken. Complication rates were determined and compared with those reported in the literature. Rates for children aged 1 year or above were compared with those in children aged less than 1 year. RESULTS: There were 3128 herniotomies performed, with an overall recurrence rate of 0.6 per cent. Wound infections occurred in 0.9 per cent, but were confined to infants aged less than 12 months. The rate of acquired cryptorchidism was 0.4 per cent and testicular atrophy 0.8 per cent in boys. Injury to the vas deferens was recognized in 0.1 per cent. Children under 1 year of age were more likely to develop recurrence than older children (0.9 versus 0.2 per cent respectively; odds ratio 4.56, P = 0.013). CONCLUSION: Complication rates were relatively low compared with those reported previously, but were considered achievable in most paediatric surgical units. Specific attention should be paid to the youngest infants, as this group has the highest complication rate. Minimum standards can be proposed based on these and other published outcomes.

The degree to which the size and side of an inguinal hernia is predictive of a hernia on the other side.

BACKGROUND: The indication for contralateral exploration in patients with a unilateral inguinal hernia and the implications of a contralateral patent processus vaginalis have been the subject of much debate during recent decades. The aim of this study was to investigate whether the size and side of the hernial sac on the side of presentation are predictive for the operative appearance of the processus vaginalis/hernial sac on the contralateral side. METHODS: All primary herniotomies performed by the Christchurch-based paediatric surgical service were reviewed. Patients who developed a contralateral hernia after unilateral repair were identified. In a random subset of patients who had bilateral exploration for unilateral hernias, the size of the clinical hernial sac was plotted against the size of the contralateral patent processus vaginalis/hernial sac (when present) as assessed at the time of open surgery. RESULTS: Overall, 2,124 unique patients underwent herniotomies in the period reviewed, and 44 (3.8%) metachronous contralateral hernias were identified. Girls were not at greater risk than boys of developing a metachronous hernia. There was a positive correlation between the size of the clinical hernial sac and the size of the contralateral processus vaginalis/hernial sac. There was no significant relationship between the original presenting side and the development of a metachronous contralateral hernia. CONCLUSION: It is not possible to predict reliably which patients who present with a unilateral hernia would benefit from bilateral exploration, although the size of the hernia has a positive correlation with the size of the contralateral processus vaginalis/hernial sac. Those with "massive" hernias on the presenting side were more likely to have "large" contralateral hernial sacs, but how many of these would subsequently become symptomatic remains uncertain.

Rare pulmonary manifestations of tuberous sclerosis in children.

Tuberous sclerosis is one of the rare neurocutaneous syndromes and may involve organs other than skin and brain. Pulmonary involvement is uncommon and predominantly affects women of childbearing age. Those affected present with dyspnea or spontaneous pneumothorax from cystic or fibrous degeneration of the lung. Males are affected very rarely. This paper describes another manifestation of pulmonary involvement in tuberous sclerosis, namely, recurrent intrapleural cysts in a young boy.

A rare tumor masquerading as an empyema: pleuropulmonary blastoma.

Pleuropulmonary blastoma, although rare, should be considered in the differential diagnosis of children who present with a pleural effusion and symptoms of respiratory tract infection when the empyema fails to respond to medical management. A case of a 2-year-old boy with this rapidly growing tumor is reported. The treatment and outcome of pleuropulmonary blastoma in children is reviewed.

Long-term continence in patients with high and intermediate anorectal anomalies treated by sacroperineal (Stephens) rectoplasty.

Thirty-seven patients treated at the Royal Children's Hospital, Melbourne, by Stephens (sacroperineal or sacroabdominoperineal) rectoplasty were reviewed after 5 to 32 years. Continence was assessed using the Kelly, Templeton, Kiesewetter, and Wingspread criteria. Although approximately 20% of the patients were shown to have unsatisfactory control with each clinical scoring system, the remainder have a socially acceptable level of fecal control. Several patient groups had more favorable results than others: (1) females; (2) patients who did not have an abdominal component to their rectoplasty; and (3) patients reviewed more than 10 years after surgery. Twenty-eight patients reached their final level of continence in the first few years and nine improved gradually throughout childhood.

Preliminary evidence that cell death may contribute to separation of the trachea from the primitive foregut in the rat embryo.

BACKGROUND/PURPOSE: The embryogenesis of many congenital tracheoesophageal abnormalities has been elucidated poorly, mainly because of an incomplete understanding of the normal mechanism of separation of the primitive foregut into the trachea and esophagus. There has been controversy about the existence and significance of the so-called tracheoesophageal septum. This study investigates the normal mechanism of tracheoesophageal separation in the rat. METHODS: Timed-pregnant Sprague-Dawley rats were killed on gestational days 11 to 16, respectively (the day on which the vaginal smear showed sperm was considered day 0 of gestation). Thirty-six embryos (six embryos from each age group) were fixed in 10% formalin. Fixed embryos from gestational day 13, 12, and 11 were sandwiched in melted agar to facilitate proper orientation before being processed. Half of the fetuses from each age group were sectioned serially in a transverse plane and the other half in a sagittal plane. The histological sections were stained with H&E. RESULTS: On gestational day 11, the foregut appears as a ventrodorsal slit in transverse section. Below the primitive pharynx, the epithelial cells on the ventral part of the foregut are actively proliferating, producing two lateral epithelial bulges (primitive bronchial buds). The epithelial cells of the dorsal aspect of the foregut show no sign of active growth. On day 12, the proximal trachea still shares a common lumen with the foregut. The distal trachea, the tracheal bifurcation, and both primary bronchi are recognized easily. At the point of tracheoesophageal separation, there is obvious debris of dead epithelial cells and condensed nuclei. These appearances were specifically located around the so-called tracheoesophageal septum and nearby grooves, resulting in enfolding cristae. There was no cell proliferation or inflammatory response in this region. On gestational day 13, the separation of the trachea and esophagus was almost complete. CONCLUSION: The present study demonstrates that a specific and consistent pattern of cell death in the region of the "tracheoesophageal septum" produced enfolding cristae that may be part of the mechanism of separation of the trachea and esophagus.

The leadpoint in intussusception.

Fifty-six patients with a pathological lesion at the leadpoint of their intussusception were encountered at the Royal Children's Hospital, Melbourne, over a 16-year period. The most common lesions were Meckel's diverticula, followed by small bowel polyps, lymphosarcomas, and duplication cysts. Age at presentation was the only factor discriminating these patients from patients with idiopathic intussusception. Unlike previous studies, duration of symptoms was not useful in identifying a pathological leadpoint. Hydrostatic reduction was successful in reducing the leadpoint in three of 21 attempts. Neither morbidity nor mortality was encountered in a policy of initial enema reduction of intussusception, regardless of age.

Progression of intussusception.

The position of the apex in intussusception indirectly reflects the length of the intussusceptum in the majority of cases in which the intussusception begins in the region of the terminal ileum. The relationship of the apex to the duration of symptoms and the ease of enema reduction suggests that progression of intussusception occurs rapidly after the onset of symptoms and is normally complete by the time the patient presents to hospital. Further increase in length of the intussusception is probably limited by progressive edema of the intussusceptum and by the length of small bowel mesentery available to it. The position of the apex per se is not a contraindication to attempted enema reduction, although once the apex has reached the rectosigmoid region successful enema reduction is less likely.

Relationship of the notochord to foregut development in the fetal rat model of esophageal atresia.

BACKGROUND/PURPOSE: The notochord (Nt) is thought to act as a primary organizer for adjacent axial embryonic organs. The current study used the Adriamycin-induced fetal rat model of esophageal atresia and tracheoesophageal fistula (EA-TEF) to determine whether anomalies of the foregut (FG) were associated with an abnormal Nt. METHODS: Eight experimental female Sprague-Dawley rats received intraperitoneal injection of Adriamycin (1.75 mg/kg) on gestational days 6 to 9 inclusive, and 4 control rats received saline injection only. Their embryos were harvested on gestational days 11, 12, 13, and 14. Embryos from each age subgroup were serially sectioned and stained with H&E. The FG and Nt were traced from the primitive pharynx to the level of the stomach. RESULTS: By day 11, the Nt of control embryos had completely separated from the FG and was located immediately ventral to the neural tube. On gestational day 12, the Nt detached from the neural tube, and the trachea and esophagus were separating. On day 11, in the Adriamycin-treated embryos, the Nt was still attached to an FG that was narrowed or occluded. On day 12, the Nt remained adherent to the FG from the primitive pharynx to the level above the primitive respiratory buds, at which point it became thicker and branched sagittally, with the anterior branch contacting or merging with the FG. The FG usually loses its lumen or continuity when in contact with the Nt. CONCLUSIONS: Exposure of rat embryos to Adriamycin leads to abnormal development of the Nt, including prolonged attachment to or fusion with the FG, and abnormal branching. Traction on the FG by the Nt produces occlusion of its lumen and may result in its complete interruption. Separation of the Nt from the FG would appear to be a prerequisite for the normal development of the FG into its derivatives: the esophagus and trachea.

The diagnosis of congenital tracheoesophageal fistula.

Congenital tracheoesophageal fistula presents with clinical features of variable types and severity, including choking, coughing, and cyanosis with feeds, pneumonia, and abdominal distension. The rarity of the condition, its nonspecific symptomatology, and limitations in its demonstration by contrast radiology and endoscopy contribute to delays between first presentation and confirmation of the diagnosis. Recognition of symptom complexes and a high index of clinical suspicion should assist early diagnosis. In our experience with 30 infants with "H" fistula, an initial barium swallow demonstrated the fistula in 73%, and in all patients by the third attempt. Refinements in technique have shown contrast radiology to be satisfactory and safe in the initial investigation of infants with suspected congenital tracheoesophageal fistula and enables differentiation from other causes of aspiration.

Intussusception: prediction of outcome of gas enema.

Gas has replaced barium as the preferred medium for enema reduction of intussusception in tertiary pediatric institutions. Previously, adverse clinical features that in combination predicted a low likelihood of successful reduction using barium had been identified and used to select patients suitable for attempted enema reduction. This study examines whether these adverse features are equally applicable to gas reduction of intussusception. For any given number of adverse features, gas enema reduction was found more likely to be successful than barium reduction and, in the absence of clinical evidence of peritonitis, multiple adverse features probably do not represent a contraindication to attempted gas enema reduction.

Intussusception: a repeat delayed gas enema increases the nonoperative reduction rate.

The operative rate in intussusception has been significantly reduced by (1) expansion of the indications for attempted enema reduction of intussusception to include all children except those with peritonitis or septicaemia and (2) use of the gas (oxygen) enema rather than barium. This prospective study looked at the value of performing a delayed repeat gas enema in children in whom only partial reduction was achieved during the first enema, and who remained clinically stable. Twenty-one patients (of 156 with proven intussusception) had a repeat delayed gas enema, 30 minutes or more after the first attempt at reduction. This was successful in over 50%, reducing the number of patients requiring surgery at our institution by 24% and saving approximately five children a year from surgery.

How to avoid complications during percutaneous endoscopic gastrostomy.

Percutaneous endoscopic gastrostomy (PEG) has advantages over open gastrostomy, and is an accepted technique for children. However, a number of technical problems may be encountered during insertion. This report identifies those problems and proposes precautions that can be taken to reduce their likelihood. Recognized major complications include esophageal injury, colonic perforation, wound infection, gastric erosion by the gastrostomy tube, and later symptomatic gastroesophageal reflux requiring correction by fundoplication.

Secondary esophageal surgery following repair of esophageal atresia with distal tracheoesophageal fistula.

During the period 1948 through 1988, 498 patients with esophageal atresia and distal tracheoesophageal fistula were admitted to the Royal Children's Hospital, Melbourne. Fifty patients had a second operative procedure on the esophagus, for anastomotic stricture (30), recurrent fistula (15), both (4), and a postmyotomy diverticulum (1). During the same period, nine patients underwent esophageal replacement and 33 patients a Nissen fundoplication. Improvements in the technique of esophageal anastomosis, and in recent years the use of fundoplication to correct gastroesophageal reflux have led to a marked reduction in the need for secondary surgery to the esophagus after repair of esophageal atresia. Esophageal replacement is rarely required in esophageal atresia and distal tracheoesophageal fistula. One-layer end-to-end esophageal anastomosis using interrupted sutures resulted in the lowest rate of recurrent fistula and anastomotic stricture.

Cryptorchidism in spina bifida and spinal cord transection: a clue to the mechanism of transinguinal descent of the testis.

In spina bifida there is a high incidence of cryptorchidism, particularly where the lesion is at or above L2. This article describes the location of the testis in 32 spina bifida patients and the effect of spinal cord transection in neonatal rats: both studies suggest that the spinal cord influences testicular descent. It is already established that androgens, an intact genitofemoral nerve, and the gubernaculum are essential for the transinguinal phase of testicular descent to occur. Yet the mechanism by which testosterone exerts its effect on the gubernaculum remains obscure. To account for all the observed phenomena we postulate that a spinal cord nucleus may exist that is sensitive to androgens and mediates impulses through the genitofemoral nerve to the gubernaculum. Neuronal modulation of the gubernaculum may induce testicular descent.

Management of the premature infant with esophageal atresia and hyaline membrane disease.

Prematurity of 34 weeks' gestation or less occurs in 12% of infants born with esophageal atresia and distal tracheoesophageal fistula, and is likely to result in moderate or severe hyaline membrane disease. In the past, these infants have often had their surgical treatment delayed or staged. Based on our experience of 24 such patients since 1977, we propose that optimal treatment involves early division of the distal tracheoesophageal fistula via a thoracotomy and simultaneous repair of the esophagus where the patient's condition permits. This should be in the first 12 hours of life, before the hyaline membrane disease becomes fully established. Gastrostomy is not required and may be contraindicated.

Apoptosis during regression of the tailgut and septation of the cloaca.

PURPOSE: Apoptosis is involved in the embryonic morphogenesis of many organs. The current study was undertaken to ascertain the role of apoptosis during cloacal development in the rat. METHODS: One hundred five rat embryos, ranging from gestational days 11 to 16, were sectioned serially in the transverse or sagittal planes and stained with H&E. The cloaca, urorectal septum, rectum, urogenital sinus, Wolffian ducts, and tailgut (TG) were examined consecutively in temporospatial sequence. RESULTS: The tailgut immediately distal to the hindgut starts to regress by apoptosis on day 12 of gestation in a craniocaudal direction and has regressed completely by day 13.5. A large number of apoptotic cells and debris can be identified in the urorectal septum during cloacal septation. Vacuoles are formed by coalescence of apoptotic cells at the tip of urogenital sinus from day 15 to 16, and, at the same time, sporadic apoptotic bodies in the anal membrane contribute to its thinning. CONCLUSION: Results of the current study confirm that apoptosis occurs in a specific temporo-spatial sequence in the hindgut and cloaca and appears to be an important mechanism in TG regression, uro-rectal separation, urethral opening, and rupture of the anal membrane.

Temporospatial aberrations of apoptosis in the rat embryo developing esophageal atresia.

BACKGROUND/PURPOSE: Recent work has shown that apoptosis is a key component of the normal development of the foregut. This study was designed to compare the patterns of apoptosis in the normal foregut with those in the fetus developing esophageal atresia and tracheoesophageal fistula (EA-TEF) using 3-dimensional reconstructive techniques. METHODS: Timed pregnant rats that received no treatment (control group) or received Adriamycin intraperitoneally (experimental group) had their embryos removed between days 11 and 14 of gestation. The embryos were sectioned serially and stained with H&E. Three-dimensional reconstructions were made of the foregut and areas of apoptosis were marked on them to facilitate analysis of apoptotic patterns. RESULTS: Apoptosis was evident in control embryos in the region in which tracheoesophageal separation occurs from days 12 and 12.5. Experimental embryos showed no apoptosis until day 13 when apoptosis was observed immediately posterior to the foregut within the esophageal mesenchyme and in the laryngeal mesenchyme ventral to the foregut. CONCLUSIONS: The pattern, timing and location of apoptosis in rats developing EA-TEF is abnormal. Our work indicates that it is actually a complete lack of apoptosis at the crucial stage of development that leads to this abnormality rather than an alteration in the patterning of apoptosis at this crucial time. The observation of apoptosis only within the mesenchyme raises the possibility that apoptosis in the foregut developing EA-TEF may be a reaction to that abnormal development rather than its cause.

The relationship between cerebral palsy and cryptorchidism.

This study examined the reported association between cerebral palsy and cryptorchidism. A comparison was made among 25 boys with cerebral palsy under the age of 2 years and 6 months, 25 boys with cerebral palsy aged between 5 and 10 years, and age-matched controls. The testes remained in the same position with age in boys with cerebral palsy, whereas in normal children the testes were slightly lower initially (P less than .005) and became lower still with growth (P less than .001). This result, taken in conjunction with previous studies, casts doubts on the theories of early damage to the hypothalamic-pituitary-gonadal axis as the cause of maldescent in cerebral palsy. It is postulated that any apparent increase in cryptorchidism in older patients with cerebral palsy may be caused by spasticity of the cremaster muscle leading to pathologic retraction of the testis out of the scrotum.

Chest wall deformity in patients with repaired esophageal atresia.

Chest wall deformities developed after thoracotomy for esophageal atresia, in 77 of 232 patients (33%) who did not have a congenital vertebral anomaly. Anterior chest wall asymmetry was present in 47, scoliosis in 18 and a combination of both in 12 patients. Scoliosis was convex away from the incision in two thirds of those affected. Anterior chest wall deformity was more common in patients greater than 25 years of age, and scoliosis was more common in patients who had had multiple thoracotomies. Breast surgery to minimize inequality was required in three female patients, and spinal surgery in one patient. Twenty-two of 53 patients with a congenital vertebral anomaly developed scoliosis, eight of whom required surgery. The scoliosis was probably the result of the vertebral anomaly in these patients, who are particularly at risk for progressive deformity.