Escape of the yolk sac: a hypothesis to explain the embryogenesis of gastroschisis.

Gastroschisis is a significant birth defect that in many countries has shown an increased prevalence in recent decades, and the change has affected primarily younger mothers. Despite numerous epidemiological studies no other consistent associated risk factor has been identified. In this paper we review the five main theories related to the pathogenesis of this malformation and outline the reasons why we think none fully explains the embryogenesis of gastroschisis. We briefly present some clinical observations we have made that we consider germane to the pathogenesis and outline a hypothesis that we think can account for the origins of this malformation. Our proposal is that the determining defect in gastroschisis is failure of the yolk sac and related vitelline structures to be incorporated into the umbilical stalk. Otherwise, ventral closure of the lateral abdominal walls occurs normally, thus orphaning the vitelline duct and yolk sac outside both the main body stalk and the abdominal wall. Thus, in addition to the umbilicus, the abdominal wall has a separate perforation through which the midpoint of the gut is attached to the exteriorized vitelline structures. This connection through the ventral wall prevents normal egress of the gut into the umbilical cord during the second month of development and acts as the egress point for the gut resulting in gastroschisis.

Iatrogenic arteriovenous fistula after multiple arterial punctures.

Multiple arterial punctures in the neonate can result in iatrogenic arteriovenous fistula formation. In one case, a 3-month-old infant who had been born prematurely was noted to have physical findings consistent with arteriovenous malformation following approximately 90 arterial blood gas determinations over a 13-week period. The area was explored and the lesion was treated surgically with good results.

Direct central vein puncture for silicone rubber catheter insertion. An alternative technique for Broviac catheter placement.

The indications for placement of the Broviac catheter have been expanded. Although cephalic vein cutdown has become the accepted technique for placement of the catheter, cannulation of this vein is not always possible. A technique is described for direct placement of the Broviac catheter into the subclavian or internal jugular vein. Formal venous cutdown is not required, and the technique does not sacrifice a vein. We have performed 21 consecutive catheter insertions in 20 patients without complications. It is a safe alternative method for silicone rubber catheter placement.

A perinatal approach to the diagnosis and management of gastrointestinal malformations.

Ultrasonography now offers the opportunity to evaluate selected high-risk pregnancies for the presence of fetal malformations that are amenable to corrective measures as well as of those that remain incompatible with life. The authors observed major fetal malformations in 1% of 6050 cases that underwent antenatal ultrasonographic evaluation. A series of 9 prenatally diagnosed gastrointestinal tract anomalies, including the first documented instances of esophageal atresia diagnosed in utero, is presented in detail. Abdominal wall defects were those most commonly encountered in this series and were diagnosed as early as 16 weeks' gestation. The gastrointestinal system is not only one of the most common sites of birth defects, but it is also the one wherein lies the greatest hope for a successful neonatal outcome. The value of prenatal diagnosis in a comprehensive perinatal approach to congenital gastrointestinal lesions is emphasized. Forewarning the obstetrician and altering the pediatric-surgical team for resuscitation, immediate postnatal evaluation, and timely intervention decrease neonatal mortality and increase the chance of long-term infant survival.

Expanded applications of Broviac catheter.

Sixteen Broviac catheters were inserted in 15 patients ranging from 15 to 61 years of age for purposes other than parenteral nutrition. These patients required long-term intravenous (IV) antibiotic therapy for cystic fibrosis or chemotherapy for malignant neoplasm. There were no major complications. The Broviac catheter provides excellent long-term IV access for total parenteral nutrition. In addition, it may be used very effectively in patients requiring extended IV antibiotic therapy and/or chemotherapy.

Nodular hyperplasia, black thyroid, and chronic minocycline ingestion in a teenager.

An 18-year-old man with left-lobe thyroid hemiagenesis underwent isthmectomy for management of a nodule that failed to take up radioactive iodine during a nuclear scan. The resected tissue, which demonstrated nodular hyperplasia, and the remaining right lobe, were black. The association between deep staining and chronic minocycline ingestion was subsequently recognized. Twelve years later, the patient remained asymptomatic, suggesting that complete resection of tetracycline-stained thyroid tissue is unnecessary.

Percutaneous endoscopic gastrostomy. Review of 150 cases.

Percutaneous endoscopic gastrostomy was performed on 50 children and 100 adults from June 1979 to May 1982 at Case Western Reserve University Hospitals and the Mt Sinai Medical Center in Cleveland. Morbidity was low (10%), and there were no procedure-related deaths. Complications included minor wound infections in seven patients early in the series, extrusion of the tube in three, unnecessary laparotomy in two suspected of having problems with the tube early in the series, a partial separation of the gastrostomy from the abdominal wall in one adult, and gastrocolic fistula in one adult and one child. The last condition disappeared after removal of the gastrostomy tube in both patients. No leakage around the catheter, hemorrhage, peritonitis, or gastric outlet obstruction was encountered. This procedure provided a rapid, safe, and effective method for creating a feeding gastrostomy and did not require general anesthesia and laparotomy. Percutaneous endoscopic gastrostomy should become the method of choice for the creation of a feeding gastrostomy.

Moxalactam vs tobramycin-clindamycin. A randomized trial in secondary peritonitis.

One hundred five patients with peritonitis were randomized to receive either tobramycin sulfate plus clindamycin phosphate or moxalactam alone before surgical intervention. Fifty-nine patients were evaluable. A mean of 3.1 (moxalactam) and 3.5 (tobramycin-clindamycin) pathogens per patient were identified. Overall success rate was 85% (tobramycin-clindamycin, 24/30; moxalactam, 26/29). When patients with appendicitis were excluded, there was an observed but not statistically significant advantage of moxalactam over tobramycin-clindamycin (85% vs 67%). There were five deaths (tobramycin-clindamycin, four; moxalactam, one). Other complications included hypoprothrombinemia (tobramycin-clindamycin, five; moxalactam, five), renal dysfunction (tobramycin-clindamycin, three; moxalactam, one), and superinfection (tobramycin-clindamycin, nine; moxalactam, six). More wound infections were noted in the group given tobramycin-clindamycin. These data suggest that moxalactam is as safe and efficacious as tobramycin plus clindamycin. The observed benefits of this agent warrant study in a larger sample to verify advantages of moxalactam over combination therapy.

Small bowel obstruction secondary to incomplete removal of a ventriculoperitoneal shunt: case report.

Small bowel obstruction and partial enteral loss secondary to entrapment by an incompletely removed ventriculoperitoneal shunt is a previously unreported hazard.

Percutaneous approaches to enteral alimentation.

Feeding gastrostomy and jejunostomy provide effective access for long-term enteral nutrition. Traditional operative techniques for the performance of these procedures requires laparotomy and often, general anesthesia. This report describes our experience with two relatively new methods, percutaneous endoscopic gastrostomy and percutaneous endoscopic jejunostomy. Results of percutaneous gastrostomy and jejunostomy to date in 323 cases include a morbidity of 5.9 percent and a 0.3 percent operative mortality. Percutaneous endoscopic gastrostomy and jejunostomy should become the procedures of choice for the establishment of enteral access in patients requiring long-term enteral alimentation.

Vascular access techniques and devices in the pediatric patient.

Vascular access is a sine qua non in the management of pediatric surgical patients. The indications, as well as the number of available access routes, types of devices, and their use, have expanded over the last two decades. This article is an overview intended to allow the surgeon to match the safest and most effective access to the child's therapeutic needs. It also contains descriptions of sites for percutaneous and cut-down vascular access in children, as well as the author's personal approach to central venous access. Vascular access in children requires skill, time, patience, and the appropriate equipment. Fortunately, with attention to detail, most complications can be avoided.

Gastrostomy techniques and devices.

Gastrostomies play an important role in the management of a wide variety of surgical and nonsurgical conditions of childhood. Many techniques and gastrostomy devices are available. In our experience, percutaneous endoscopic gastrostomy has proved safe and effective, and the gastrostomy button has eliminated most of the catheter-related problems. Candidates for gastrostomy, particularly children with foregut dysmotility, must be carefully selected, undergo preoperative studies aimed at determining the degree of gastroesophageal reflux, and have appropriate long-term follow-up. Attention to technical detail is essential to avoid operative complications. A good working relationship between the surgeon, gastroenterologist, nurse, and patient's family is essential to minimize long-term morbidity, particularly stoma-related problems.

Acute abdomen. When to operate immediately and when to observe.

When to operate immediately, when to observe, and when not to operate at all represent major challenges in the management of a child with an acute abdomen. This article is an overview of the subject from symptom to diagnosis, evaluation, and preparation for the surgical intervention. Tables provide examples of conditions requiring prompt surgical intervention and relative surgical urgency; pathologies suitable for (initial) nonsurgical management; and clinical pictures where surgical intervention is not indicated. Factors that influence the timing of operation are provided, as is the differential diagnosis between intestinal strangulation and obstruction. Brief notes highlight four important causes of acute abdomen in children acute appendicitis, malrotation with volvulus, Meckel's diverticulum, and intussusception. These as well as other intraabdominal pathologies are illustrated by means of surgical photographs. The acute abdomen is a clinical diagnosis. Other diagnostic modalities have merely supporting roles. The decision to operate is based primarily on the results of a good history and thorough physical examination(s).

Inguinal hernia in infants with very low birth weight.

Inguinal hernias (IH) are among the most commonly encountered surgical problems in infants with very low birth weight (VLBW, <1,500 g) with a reported incidence of 16%. A trend toward earlier operation has emerged in recent years, with most now being repaired before discharge from the neonatal intensive care unit. The authors review the many special concerns regarding the management of IH in this patient population, including the timing of repair, the risk of incarceration, anesthetic management, the frequency of bilaterality, the high incidence of undescended testes, and the technical aspects and complications associated with IH repair in the VLBW infant.

Percutaneous endoscopic gastrostomy in the cancer patient.

A retrospective analysis of all patients with malignancy undergoing percutaneous endoscopic gastrostomy (PEG) was undertaken. PEG was attempted in 42 cancer patients from a total of 142 patients and successful in 38 (90.5%). The success rate in completing this procedure in cancer patients was significantly less than in noncancer patients (P = 0.04). The most common malignancy was oral-pharyngeal carcinoma (29%). Survival data was available in 35 of 38 patients (92%). Three patients died in less than one month. Forty six per cent of the patients survived three months or longer and 20 per cent 1 year or longer. The majority of the patients (68%) were able to return home after the PEG. There was no mortality related to the procedure. Local anesthesia was used in 95 per cent of the procedures. The major concerns derived from the study are the short-term survival in a small number of patients and aspiration in selective patients. Attempts should be made to identify these patients and avoid PEG since its benefits will be limited. When used selectively, PEG can improve the quality of life of the cancer patient.

Is thoracoscopically aided pleural debridement advantageous in children?

Thoracic empyema encompasses a spectrum of inflammatory manifestations ranging from thin parapneumonic pleural effusion to the formation of a thick, constricting rind. The aim of this study is to determine the applicability of thoracoscopically aided pleural debridement (TAPD) in children with complicated empyema and to assess its possible advantages. In the last 6 years, 26 children (ages 2 months-16 years; median, 7 years; mean, 7 years) were diagnosed with empyema (right, n = 15; left, n = 11). Their charts, radiographs, and follow-up courses were reviewed. All children had typical clinical and radiological findings of empyema; one also had necrotizing pneumonitis. Treatment modalities included antibiotics only (n = 3), antibiotics with tube thoracostomy (n = 11), open thoracotomy (n = 5), and TAPD (n = 7). Children treated with antibiotics alone had an average (avg) length of stay (LOS) of 31 days. Those managed with tube thoracostomy had an avg LOS of 13 days, and those who underwent thoracotomy had an avg LOS of 16 days. The seven children treated with TAPD had an avg LOS of 12 days, and their avg postoperative chest tube use was 6 days. Children with TAPD had considerable less pain and recovered faster. TAPD of empyema is promising for children whose lungs do not expand promptly after tube thoracostomy or who have a persistent loculated empyema.

A rationale for routine use of transverse abdominal incisions in infants and children.

Although transverse abdominal incisions (TAI) are valued for their excellent exposure in the newborn, infant, and child, measurements to support this clinical impression have been lacking. The abdomens of 80 infants and children and 11 premature infants were measured and evaluated and correlated with growth. It was demonstrated that the younger the child, the relatively larger the abdominal cavity and wall. Because of the anatomical differences between the abdomen of adults and small children, the cavity of the latter resembles a horizontally oriented ellipsoid; accordingly, TAI give greater exposure. The younger the child, the larger proportionately was the costoiliac space, allowing easier lateral extension of the incision, if necessary. The umbilicus is low in infants and small children and the transverse supraumbilical incision may be placed over the anatomical center of the abdominal cavity. The conclusion drawn was that the younger the child, the greater the rationale for routinely employing transverse abdominal incisions.

Gastroschisis and extraabdominal ectopic testis: simultaneous repair.

During a 10-year span, three males with gastroschisis were seen in whom one of the testes exited through the paraumbilical opening with the exteriorized intestine. In each case, prior to correction of the abdominal wall defect, the testes (left side in 2, right side in 1) were placed into the ipsilateral scrotum and anchored. In the first child, the undescended gonad, very small at birth, continued hypoplastic at 1 year. The second patient (8 years) required a second stage orchidopexy at age 2 years. The third child, a 1.3-kg premature infant, has good testicular size and position at 6 1/2 years. Undoubtedly because of the magnitude of the main pathology, recommendations on how to handle the gonad in this association are not available. This report focuses on the simplicity of concomitant repair. A similar approach was employed in two children with omphalocele and undescended testes.

Percutaneous endoscopic gastrostomy: a 10-year experience with 220 children.

This series analyzes 224 percutaneous endoscopic gastrostomies placed in 220 children. Our experience indicates that the percutaneous endoscopic gastrostomy has a wide range of indications, is advantageous in high-risk patients, and, if properly performed, compares favorably with traditional gastrostomies.

Percutaneous endoscopic gastrostomy-20 years later: a historical perspective.

BACKGROUND/PURPOSE: Percutaneous endoscopic gastrostomy (PEG), initially developed for children with inability to swallow, had its first presentation at the annual meeting of the American Pediatric Surgical Association in Florida in 1980. Based on the novel concept of the sutureless approximation of a hollow viscus to the abdominal wall, this minimally invasive procedure has become the standard for direct gastric access worldwide. This report is a brief retrospective about the evolution of PEG and the expanded applications of the surgical concept on which it is based. METHODS: Information related to PEG was obtained from personal records, a focused literature search, and data from various registries and the industry. RESULTS: The search identified 836 peer-reviewed publications directly related to PEG. The original Journal of Pediatric Surgery article has received 483 bibliographic citations. The procedure has had a profound impact on nutritional management, particularly among adult patients. Over 216,000 PEGs are performed annually in the United States. Twelve major manufacturers produce PEG or PEG-related enteral access devices. Select expanded applications of PEG and its principle include indications beyond feeding, use in high-risk patients, percutaneous jejunostomy, percutaneous cecostomy, correction of gastrostomy leakage and gastric volvulus, multiple PEG portals for intragastric interventions, and laparoscopically assisted gastrostomies. CONCLUSIONS: Over 20 years, percutaneous endoscopic gastrostomy has experienced exponential growth. Improved guidelines and technical refinements have added to its safety. The concept on which it is based has created a ripple effect and led to numerous applications beyond gastric access for feeding. In an era when so many of our procedures are adopted from "adult" general surgery, it is worthwhile to have an historical perspective on PEG, a technique that originated in pediatric surgery.