Factors associated with mortality in neonates with gastroschisis.

PURPOSE: The ongoing epidemic of gastroschisis has created multiple challenges and continues to raise questions concerning the optimal management of these high-risk patients. Although the overall survival rate has increased over the past 3 decades, morbidity and mortality remain significant. The purpose of this study was to analyze the main factors associated with mortality in neonates admitted to an intensive care unit for the management of this abdominal wall defect. METHODS: This study is a retrospective review of a large de-identified neonatal intensive care dataset encompassing 284 institutions in 32 states and Puerto Rico, from 1/1/1997 to 1/1/2010. Of the 629 440 neonates in the dataset, a total of 3 456 newborns were diagnosed with gastroschisis (5.5/1 000 hospital discharges). Of these, 685 were transferred to other centers and data was missing on 22, leaving 2 749 infants available for analysis. RESULTS: Out of these 2 749 infants of whom we knew the outcome, 115 (4.2%) died. Multivariate logistic regression showed that the factors independently associated with an increased risk of death were male gender, immature gestational age, low birth weight, low 5 min Apgar Score, the need for vasopressors during the first week after birth and the need for high levels of oxygen support. The presence of associated anomalies, vaginal delivery, treatment with surfactant and the need for ventilator support on the day of birth were not independent risk factors associated with an increased mortality. CONCLUSION: Premature delivery and low birth weight are the most important factors associated with an increased risk of mortality. Cesarean section does not appear to reduce the risk.

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.

Imaging for endoscopic surgery: new developments applicable to pediatric surgical interventions.

To introduce pediatric surgeons to new developments in imaging that are already or soon to become available that permit "in-line" work and three-dimensionality, five new approaches to imaging were evaluated in 419 children over a 3-year period: (1) Image projection onto the abdomen (n = 4). As opposed to conventional video camera positions, with this modality the line of view and the line of work are aligned. (2) Image projection onto a plate mounted just above the abdomen (n = 280). As in the first approach, screen arrangement position obviates looking up at the monitor. (3) A touch screen mounted above the abdomen, enabling the surgeon to not only monitor the procedure, but also to control all the equipment (n = 128). As with the first two modalities, the line of view is aligned with the direction of the work. (4) A three-dimensional (3-D) head display (n = 6). With this approach the use of a headset is required. (5) A 3-D screen (n = 1) for which, no goggles or headsets are required. The 3-D picture can be appreciated from a wide angle of view, and thus can be used by the surgeon and assisting team. Direct imaging projection onto the abdominal surface is a visual process that at present is too complicated for routine surgery. Projection onto a flat plate or touch screen is a major improvement; the surgeon looks and works in the same direction. Headsets for 3-D imaging remain heavy and the image is not as sharp as that provided by two-dimensional monitors. The most significant practical progress was felt to be with the flat 3-D monitor because with this equipment, depth perception and ergonomic positioning were both rated as very good. New imaging modalities are exciting, albeit still in their early developmental stages. The novel imaging provided by a 3-D monitor is most promising, because it combines good depth perception with physiologic in-line visual-manual coordination. These developments should further facilitate the transition from conventional, open techniques to videoscopic approaches.

Acute appendicitis in children: the importance of family history.

BACKGROUND/PURPOSE: Acute appendicitis is common, frequently atypical, challenging, and still associated with significant morbidity. Despite major technologic advances, appendicitis remains a primarily clinical diagnosis. Therefore, no relevant anamnestic information should be overlooked. Surprisingly, the relationship between heredity and appendicitis is seldom considered. Because of the potential clinical importance of the family history, the authors addressed this question prospectively over a 52-month period in a practice that includes the majority of pediatric patients with appendicitis in the region. METHODS: Family histories were obtained in a standardized manner, focusing on first-degree relatives. Children with incomplete family information were excluded. Patients (ages 2(1/2) to 19 years) were divided into 3 groups: group A, children who underwent an appendectomy (n = 166); group B (first control), children who presented with an acute abdomen and suspected appendicitis but did not undergo an appendectomy (n = 117); group C (second control), children who were seen in the practice for unrelated conditions (n = 141). RESULTS: A positive parental history was obtained from 59 patients (36%) in group A, 24 patients (21%) in group B, and 20 patients (14%) in group C, and the odds ratios (ORs) were 2.0 (P =.035) and 2.9 (P <.001) for groups A versus B and A versus C, respectively. Of the 13 patients whose sibling had had acute appendicitis, 9 were in group A versus 2 each in groups B and C, and the OR for any family history (siblings, parents) in groups A versus B was 1.9 (P =.028) and for groups A versus C was 2.9 (P < 0.001). Appendicitis was histologically confirmed in 93% of children in group A. CONCLUSIONS: Heredity is a significant factor in pediatric patients who have appendicitis. Children who have appendicitis are twice as likely to have a positive family history than are those with right lower quadrant pain (but no appendicitis) and almost 3 times as likely to have a positive family history than are surgical controls (without abdominal pain). Because of its potential value in changing the threshold for intervention, a careful family history should be obtained for every child in whom acute appendicitis is suspected.

A simple technique for preventing bar displacement with the Nuss repair of pectus excavatum.

BACKGROUND/PURPOSE: The most common complication of the minimally invasive technique for repair of pectus excavatum (MIRPE) is bar displacement, which has been reported to occur in 9.5% of all cases, particularly in teenaged patients. The use of a lateral stabilizing bar has improved stability but has not eliminated the occurrence of this problem. The authors report a new technique added to the standard MIRPE that creates an additional third point of fixation of the pectus bar to prevent displacement. METHODS: The technique requires the simple placement, via a spinal needle, of a nonabsorbable suture next to the sternum, encircling a rib and the bar, using a single 3-mm stab wound and thoracoscopic guidance. The suture simply is buried under the skin. Since 1998, this technique has been applied to 20 patients who underwent MIRPE. RESULTS: The average age was 14 years; 80% were boys. Average operating time was 75 minutes, and all patients had thoracoscopy with the MIRPE. A lateral stabilizing bar also was used in 14 patients. Four patients had 2 struts placed. Average length of stay was 5.5 days. There were no early complications. Mean follow-up was 12 months. Bar displacement occurred in 1 patient early in the series in which an absorbable suture was used for fixation. One patient had a prolonged hospital stay of 7 days because of postoperative pain. CONCLUSIONS: This modification to the original technique of MIRPE creates a 3-point fixation system that minimizes the risk of bar shifting even in teenaged patients. It does not add any significant time or cost to the operation, and it is fairly simple to perform. The authors believe that this technique decreases the occurrence of bar displacement, and they recommend its use for all patients with pectus excavatum considered candidates for the Nuss repair.

Pediatric surgical logoscript.

Pediatric surgical logoscript (PSL) was developed as a graphico-pictorial approach to the terminology of the specialty. It requires no more than paper, pencil, and imagination. The creation of two alphabetical samplers is shown to illustrate the technique. PSL is effective, widely applicable, and most promising.

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.

Abdominal compartment syndrome in children: experience with three cases.

BACKGROUND/PURPOSE: Abdominal compartment syndrome (ACS) is defined as cardiopulmonary or renal dysfunction caused by an acute increase in intraabdominal pressure. Although the condition is well described in adults, particularly trauma patients, little is known about ACS in children. METHODS: Three girls, ages 4, 5, and 5 years, were treated for ACS by silo decompression. Each child presented in profound shock, required massive fluid resuscitation, and had tremendous abdominal distension. The first child sustained a thoracoabdominal crush injury, underwent immediate celiotomy for splenic avulsion and a liver laceration, and required decompression 5 hours postoperatively. The second underwent ligation of her bluntly transected inferior vena cava; because of massive edema, her abdominal wall could not be closed, and prophylactic decompression had to be performed. The third presented with shock of unknown etiology, and ACS developed acutely with a bladder pressure of 26 mm Hg. RESULTS: Respiratory, renal, and hemodynamic function improved immediately in all 3 patients after decompression. Subsequently, each child underwent abdominal wall reconstruction and recovered uneventfully. CONCLUSIONS: ACS is a potentially lethal complication of severe trauma and shock in children. To prevent the development of renal or cardiopulmonary failure in these patients, decompression should be considered for acute, tense abdominal distension.

Life-threatening air rifle injuries to the heart in three boys.

Air rifles, or BB guns, are generally thought of as childhood toys. Although most injuries are not serious, life-threatening events have been reported. Within a 1-year period, 3 boys presented after BB gun shots to the chest, all requiring surgical intervention for penetrating injuries to the heart. A 15-year-old underwent window pericardiotomy for hemopericardium with thrombus 24 hours after admission. Another, 5 years of age, underwent emergent exclusion of the cardiac apex for a traumatic ventricular septal defect. The third, 8 years old, had a right ventricular injury requiring an urgent subxiphoid pericardial window for tamponade. All recovered uneventfully. Increased public awareness, adult supervision, safety training, and appropriate legislation are needed to decrease the risks of these potentially lethal weapons.

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.

Surgery is safe in very low birthweight infants with necrotizing enterocolitis.

The aim of this study was to determine how the operative event itself affects very low birthweight (VLBW) infants (< 1500 g) with necrotizing enterocolitis (NEC) undergoing surgery, and to try to identify preventable factors leading to perioperative morbidity and mortality. Eighty-five VLBW infants developed NEC during a 6-year period; 34 of those required emergent celiotomies. Data were collected retrospectively from hospital charts available on 33 infants. Birthweight ranged from 566 g to 1415 g (mean +/- SD: 961+/-262 g) and gestational age from 24 to 34 wk (28+/-3.2 wk). Thirty infants had been fed premature formula (first feed at 5+/-3.6 d) prior to the onset of symptoms and three had not been fed at all. Age at NEC symptoms was 19+/-15 d. Infants < 1000 g developed NEC much longer after the first feed compared to infants > 1000 g (p < 0.002; t-test). In 42% of the children, intraoperative blood pressure fell at least 20% from the preoperative value. Body temperature dropped from a preoperative 36.5+/-0.340 degrees C to 35.5+/-1.20 degrees C (p < 0.005), although in all children two or more heating devices were employed in the operating room. All infants survived the procedure. Six infants with pannecrosis died within 72 h of the operative event. In an appropriate setting, operative intervention under general anesthesia is well tolerated by VLBW infants with NEC. Since hypothermia was a major problem, the authors have modified their approach and now no longer transport these infants to the operating room. Instead, these infants are operated upon in the neonatal intensive care unit, directly on an infant radiant warmer system.

The 'penny pincher': a new technique for fast and safe removal of esophageal coins.

BACKGROUND/PURPOSE: Considerable debate surrounds the choice of technique for the removal of esophageal coins: endoscopic extraction versus dislodgement with a Foley balloon versus dislodgement using bougienage. The "penny-pincher" (PP) technique was developed as an alternative, incorporating the main advantages of these various approaches. METHOD: The PP technique is based on the insertion of a fluoroscopically guided device that consists of a grasping endoscopic forceps covered by a soft rubber catheter. The forceps provides a firm hold on the coin. The catheter protects the oropharynx and aligns the device with the coin. Once the tip of the catheter is close to the upper edge of the coin, the previously retracted radiopaque prongs of the grasping forceps are deployed and the edge of the coin firmly grasped and extracted. The procedure is done without anesthesia or sedation. RESULTS: Twenty coins were removed from 19 consecutive children with a mean age of 34 months. Average lip-to-lip removal (including fluoroscopy) time was 41 seconds. There were no complications, and all patients were discharged shortly after coin removal. CONCLUSION: The penny-pincher method for the removal of upper esophageal coins combines the simplicity, speed, and cost effectiveness of balloon catheter or bougie coin dislodgement with the safety and secure grasping of endoscopic or forceps removal.

Seat-belt transection of the pararenal vena cava in a 5-year-old child: survival with caval ligation.

Blunt traumatic disruption of the inferior vena cava is associated with high mortality and is rare in children. A seat-belted 5-year-old girl sustained, in a motor vehicle accident, pararenal caval transection, right renal vein transection, laceration of the right kidney, duodenal injury, and a second lumbar vertebral fracture. Damage-control surgery consisted of inferior vena caval and right renal vein ligation and temporary abdominal wall silo closure. She is alive and well 10 months after the accident, with no sequelae of caval ligation and with normal right renal function.

Iontophoresis: a needle-free, electrical system of local anesthesia delivery for pediatric surgical office procedures.

BACKGROUND/PURPOSE: Delivery of local anesthesia for surgical office procedures for pediatric patients can be difficult. Injections are painful and often lead to patient anxiety, and topical anesthetics frequently provide incomplete anesthesia. The authors prospectively studied the efficacy of iontophoresis, a needle-free technique in which positively charged lidocaine and epinephrine molecules are drawn into the tissue by an electrical current as an anesthetic for pediatric surgical office procedures. METHODS: Children undergoing an office procedure were offered local anesthesia via iontophoresis. Prospectively collected data included patient characteristics, procedure, iontophoresis dose and time, need for additional injected anesthetic, pain during the procedure as determined by a 0 to 5 faces scale, and complications. A satisfaction questionnaire was completed at the follow-up visit or by telephone. RESULTS: Over an 8-month period, 34 patients with a mean age of 6.8 years (range, 3 months to 15 years) underwent 38 office procedures with anesthesia supplied through iontophoresis. Skin lesion excision (n = 14) and abscess drainage (n = 12) were the most common procedures. Seven patients required unplanned injected anesthetic. A small, superficial burn was the only complication. Sixty percent of patients and 84% of parents rated pain as 0 to 2 (zero to mild). Overall, 88% were satisfied with the anesthetic. CONCLUSION: Iontophoresis appears to be an effective and safe alternative method of local anesthesia delivery for pediatric surgical office procedures.

Gastrostomy button conversion into a combined gastric and jejunal access device.

PURPOSE: The aim of this report is to describe the conversion of a gastrostomy button into a combined gastric decompression and jejunal feeding device. METHODS: In this technique two 9.6F single-lumen central venous silicone rubber catheters are threaded through the shaft of a 24F gastrostomy button. The longer limb is guided into the jejunum, and the shorter one rests in the stomach. The remaining space in the shaft is filled with silicone rubber paste. RESULTS: The approach was successfully used in seven neurologically impaired children. CONCLUSION: This arrangement provides effective palliative gastric and jejunal access.

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.