CARE OF THE SURGICAL INTENSIVE CARE NURSERY GRADUATE

PEDIATRIC SURGERY FOR THE PRIMARY CARE PEDIATRICIAN, PART II

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CARE OF THE SURGICAL INTENSIVE CARE NURSERY GRADUATE The Primary Care Pediatrician’s Perspective Rebeccah L. Brown, MD, Michael S . Irish, MD, Henry E. Rice, MD, Michael G. Caty, MD, and Philip L. Glick, MD

Discharge from the intensive care nursery is a major step for patients, arents, and caregivers but may represent a beginning, rather than an ending, i d1 of new challenges and unexpected obstacles. Often, parents are discharged home with their newborns, inadequately prepared to deal with many of the problems that may arise. Neonatologists and primary care pediatricians may not be fully aware of the potential short- and long-term complications unique to surgical patients. The purpose of this article is to provide insight into the management of surgically related problems that may arise in neonatal patients after discharge from the intensive care nursery and when patients are being integrated into primary care pediatricians’ practices. NUTRITION Enteral Nutrition

Proper nutrition is critical for surgical patients. Obviously, the enteral route of nutrition is preferred, and the oral route should be used if possible. Infants with neurologic problems or prolonged NPO status without adequate oral stimu-

From the Department of Surgery, The State University of New York at Buffalo, School of Medicine and Biomedical Sciences; and the Division of Pediatric Surgery of The Children’s Hospital of Buffalo, Buffalo, New York PEDIATRIC CLINICS OF NORTH AMERICA VOLUME 45 * NUMBER 6 DECEMBER 1998

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lation may have poorly developed suck-swallow mechanisms and should be evaluated by a speech pathologist to determine the safety of oral feedings before their initiation. A cine esophagogram may be useful to evaluate the suck-swallow mechanism and assess risk for aspiration. After an infant has been deemed safe to take oral feedings, one must decide on which formula to use. If breast milk is available, it should be used. If breast milk is unavailable, and the infant has normal gastrointestinal function, standard cow’s milk-based formulas, such as Enfamil (Mead Johnson, Evansville, IN) or Similac (Ross Pediatrics, Columbus, OH), may be used. Very premature infants may benefit from formulas designed specifically for premature infants. Infants with short gut or gastroschisis may require elemental formulas, such as Pregestimil (MeadJohnson) or Nutramigen (Mead-Johnson),to maximize absorption. Infants recovering from duodenal atresia may benefit from the decreased curd formation associated with Nutramigen (Mead-Johnson). Infants with lactose intolerance may benefit from use of soy-based lactose-free formulas, such as Prosobee (Mead Johnson) or Isomil (Ross Pediatrics). Patients with resolving chylothorax or chylous ascites or infants with biliary atresia may benefit from formulas high in medium chain triglycerides, such as Portagen (Mead-Johnson). Many of the specialized elemental formulas are poorly palatable; however, newborns tolerate them as long as they are not given an alternative. Weaning or transition to standard formulas should be done cautiously because after standard formulas are introduced to newborns, it may be quite difficult, far taste reasons, to return to the use of specialized formulas if deemed necessary for nutritional support. In surgical patients with slow intestinal motility or absorptive problems, feeds should be advanced slowly, often with a gradual increase in the strength of the formula. The best indicator of adequate caloric support is weight gain, with a goal of approximately 1%per day, which is usually about 30 g/day or 210 g/week. Parents should be encouraged to have their infants weighed regularly (at least once a week initially) at their primary pediatrician’s office following discharge to ensure appropriate weight gain. For various reasons (i.e., neurologic impairment, Down’s syndrome with associated congenital heart disease, or tracheoesophageal fistula), some patients do not bottle enough to meet their nutritional needs and require supplementary orogastric or nasogastric tube feedings; however, this is a temporary solution. In patients who require supplementary feeds for a prolonged period of time, a more permanent type of feeding access is required. Most commonly, a gastrostomy tube is placed. Rarely, if delayed gastric emptying or gastroesophageal reflux is involved, patients may be fed via a jejunostomy. Parents must be taught proper care of these tubes and the stoma sites, as well as how to safely administer the feeds. Although often initially begun as continuous feeds, most gastrostomy feeds are eventually administered in a bolus fashion, which is easier and more convenient for the family. Jejunostomy feeds should always be administered continuously, usually for 16 to 20 h/day, rather than via bolus method. Parenteral Nutrition

Infants with short gut, gastroschisis, and motility disorders may be unable to tolerate enteral feedings and require total or supplementary parenteral nutrition for a given period of time. Peripheral hyperalimentation with low concentrations of glucose and calcium may be used for short periods of time; however, if prolonged parenteral nutrition is required, central venous access must be obtained. During the stay in the intensive care nursery, a peripherally inserted

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central catheter may be placed for initiation and maintenance of hyperalimentation; however, for more prolonged courses, insertion of a central venous catheter is indicated. It is critical that parents be taught proper central venous catheter care, including routine flushing and aseptic dressing changes. They should also be made aware of the symptoms and signs of a central venous catheter infection, including fever, lethargy, or local erythema, warmth, tenderness, or purulent discharge along the subcutaneous tract or at the exit site. Parents must be informed that they should seek medical attention immediately if this occurs. Central venous catheters may also become dislodged, especially shortly after insertion before the subcutaneous cuff has had time to become fully incorporated. The nonabsorbable sutures securing the catheter in place should be left in for at least 1 month to ensure adequate cuff incorporation. It is important for parents to take appropriate precautionary measures to prevent unintentional dislodgement of the central venous catheter before the cuff becomes fully incorporated. In the event that the central venous catheter is lacerated or transected, parents should have an atraumatic clamp available to occlude the catheter and prevent a potentially fatal air embolus. If the central venous catheter is lacerated or transected several centimeters from the skin exit site, it may be repaired by the pediatric surgeon using a commercially available central venous catheter repair kit. Central venous catheters may also become occluded, either by thrombus or precipitate. If a catheter does not flush or aspirate, a chest radiograph should be obtained initially to confirm appropriate placement of catheter. In the event of thrombosis of the catheter, urokinase may be used to unclog the catheter. Urokinase may be infused as a single bolus of 0.5 mL to 1.5 mL (5000 U/mL) and allowed to sit within the catheter for 5 to 10 minutes or until the thrombus is dissolved. Occasionally, a 24-hour continuous urokinase infusion at 100 U/kg may be necessary. In patients receiving hyperalimentation or antibiotic infusions, precipitate may form within the lumen of the catheter, occluding it. This may occasionally be remedied with a bolus infusion of 0.5 mL to 1.5 mL of 0.1 sodium hydrochloric acid. The primary complications of prolonged parenteral nutrition are infection, electrolyte abnormalities, and cholestatic jaundice. When an infant receiving hyperalimentation via a central line develops fever or signs of systemic sepsis, one should suspect line sepsis, obtain appropriate cultures, and have a low threshold for initiation of intravenous antibiotics or pulling the central line if grossly infected. Electrolyte abnormalities are common but may be recognized and corrected by weekly to bi-weekly monitoring of appropriate laboratory tests. Cholestatic jaundice is a potentially severe complication of hyperalimentation and may lead to irreversible hepatic failure. Avoidance of this complication is a strong incentive to use hyperalimentation judiciously and to stop it as soon as it is no longer necessary. Liver function tests should be monitored closely weekly or biweekly in patients requiring hyperalimentation. Essential fatty acid deficiency that is manifest by an eczema-like rash with neutropenia and thrombocytopenia may occur if lipids are not provided. Trace metal deficiencies have also been reported but are rare with the newer hyperalimentation formulations. STOMAS Cervical Esophagostorny

Occasionally, patients with long gap esophageal atresia, complications of tracheoesophageal fistula repair, or perforation of the esophagus may require a

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cervical esophagostomy. The major problem associated with cervical esophagostomy is local skin irritation and breakdown caused by chronic contact with salivary secretions. Parents should be instructed to keep the ostomy site as clean and dry as possible, protecting the skin surrounding the ostomy with constant application of a barrier cream or use of a properly fitted ostomy bag to collect secretions. It is particularly important to continue to feed these infants orally, despite the mess, to maintain the normal oropharyngeal swallowing mechanism until the cervical esophagostomy can be taken down. Gastrostomy

Gastrostomy is generally indicated for prolonged decompression of the gastrointestinal tract or to feed patients who are unable to take adequate nutrition orally. Typically, a long Malecot (Bard, Covington, GA) or Pezzer (Bard) gastrostomy tube is placed at the initial operation. A long gastrostomy tube facilitates better early wound care, provides more effective decompression in the early postoperative period, and is more comfortable for infants in the early postoperative period. A skin-level gastrostomy device may be placed 6 to 8 weeks postoperatively after a well-developed tract has been established. The advantages of a skin-level gastrostomy device, such as the Mic-Key (MIC, Draper, UT) device or Bard (Bard) button, include improved cosmesis, less risk for dislodgement, and diminished formation of troublesome granulation tissue around the tube site. The skin-level gastrostomy device must be appropriately sized for the patient. If it is too short and tight agGinst the skin, chronic ulceration of the skin occurs. If it is too long, increased mobility of the tube leads to formation of excessive granulation tissue at the ostomy site. If it is too small, leakage of gastric contents around the tube occurs, causing local skin irritation and breakdown. The Bard button tends to be more secure and lasts longer (i.e., 9-18 mo versus 3 mo), but the Mic-Key device is less traumatic to replace. The major indications for replacement of these skin-level gastrostomy devices include dislodgement; disruption or deflation of the balloon; or a broken, leaking valve. Several types of the commonly used gastrostomy devices are demonstrated in Figure 1. Parents must be instructed in meticulous care of the gastrostomy site. The gastrostomy site must be gently cleansed once or twice a day with soap and water or half-strength hydrogen peroxide. A gentle barrier cream applied to the skin surrounding the ostomy site may prevent local skin irritation from gastric juices. A candidal skin rash may develop around the gastrostomy site, which may be treated with the application of antifungal powder. It is not unusual for a gastrostomy site to look mildly inflamed and erythematous around the edges. This is a normal reactive inflammatory response caused by the presence of a chronic foreign body. It is important, however, to differentiate this from a true infection of the gastrostomy site in which marked erythema, tenderness, and warmth, often with purulent drainage from the site, are present. Associated systemic manifestations, including fever, loss of appetite, and lethargy, also may be present. Infection of the gastrostomy site requires treatment with appropriate antibiotics and rarely may require incision and drainage if localized abscess formation is present. Excessive granulation tissue formation at the gastrostomy site is a commonly encountered problem. An appropriately sized gastrostomy tube, securely fixed and relatively immobile, is probably the best insurance against this complication; however, if this complication does occur, it can usually be treated success-

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Figure 1. Different types of gastrostomy devices. A, Pezzer (Bard, Covington, GA) long gastrostomy tube. 6,Malecot (Bard, Covington, GA) long gastrostomy tube. C, Mic-Key (MIC, Draper, UT) skin level gastrostomy device. 0,Bard (Bard, Billerica, MA) gastros-

tomy button.

fully with several local applications of silver nitrate. Rarely, with extensive granulation tissue formation refractory to silver nitrate, surgical excision and cauterization may be required. One of the major problems associated with gastrostomy devices is inadvertent dislodgement by the patient or caregivers. Parents must take precautions to prevent unintentional dislodgement of the gastrostomy tube, making it as inaccessible to the patient as possible. Constant supervision, meticulous securing of the tube, and multiple layers of clothing may be helpful in preventing this complication. A "onesy," or crotch-buttoning tee-shirt, often provides additional security. It is essential that a dislodged gastrostomy tube be promptly replaced because the tract may close within several hours. Parents must be instructed to seek medical attention by their pediatric surgeon immediately if this occurs. Replacement may be difficult if the tube has been out for a prolonged period of time and may require gentle, serial dilatation with lubricated Hegar dilators. If replacement of the gastrostomy tube is traumatic or requires dilatation of the tract, a contrast radiograph should be obtained to confirm appropriate placement of the tube in the stomach and rule out perforation. With recently constructed gastrostomies (i.e., less than 4 wk), a contrast radiograph is mandatory to confirm appropriate replacement of a dislodged gastrostomy tube. With older gastrostomies with well-established tracts, confirmation of appropriate tube placement may be established by return of gastric contents upon insertion and ability to flush the catheter with ease. After a gastrostomy tube has been replaced, parents must be given explicit instructions to call immediately if their infants develop fever, abdominal pain, distension, or feeding intolerance. Surgeons are often called upon to evaluate "leaking" gastrostomy tubes.

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Causes for leakage around the gastrostomy tube include a ruptured balloon, a broken valve, insufficient tube diameter, and distal migration of the balloon or tip of the gastrostomy tube, causing pyloric or duodenal obstruction. Bilious leakage from the gastrostomy site may signal underlying bowel obstruction. The natural tendency to place progressively larger gastrostomy tubes should be avoided if possible because this only serves to serially enlarge the stoma1 opening, thus perpetuating the problem. With chronic leakage and inflammation, it may be best to place a smaller gastrostomy tube, hold feedings, and place the tube to gravity drainage to allow healing and concentric contraction of the gastrostomy site. After the gastrostomy tube is no longer deemed necessary, it may be removed. With a temporary gastrostomy, the site generally closes spontaneously 24 to 48 hours following removal of the tube; however, when a gastrostomy has been in place for 6 months or longer, delayed closure or failure of the site to close may occur, resulting in a persistent gastrocutaneous fistula, in which case operative closure may be required.

lleostomy Ileostomy may be required in infants with meconium ileus, complicated intestinal atresias, malrotation with midgut volvulus, and necrotizing enterocolitis. High-volume ostomy output and malabsorption may result in significant dehydration, electrolyte abnormalities, and nutritional failure. Elemental formulas may be required and should be advanced only gradually as tolerated. Supplemental hyperalimentation may be required until bowel adaptation occurs and goal enteral feeds are tolerated. Electrolytes should be monitored closely and aggressively repleted. Newborn patients with ileostomies may develop severe sodium depletion and metabolic acidosis from bicarbonate loss?” Supplementation with sodium chloride and sodium bicarbonate may be necessary to correct this deficit. Close attention to maintenance of adequate hydration is critical. Large volume losses may require the addition of antidiarrheal agents to slow intestinal transit but must be used with caution and are absolutely contraindicated if a mechanical obstruction is suspected. Detection of reducing substances in the stool indicates malabsorption. However, the best solution for patients with high-volume ostomy losses, electrolyte abnormalities, or malabsorption is ileostomy takedown and restoration of bowel continuity as soon as feasible. In contrast to a colostomy, which may have intermittent stool output, ileostomy output should be rather constant. An abrupt decrease in, or cessation of, ileostomy output suggests bowel obstruction. Bilious emesis may occur. The abdomen may become distended and tympanitic. An abdominal radiograph should be obtained, which may demonstrate multiple distended small bowel loops with air-fluid levels. The site of obstruction in infants with ileostomies is frequently at the fascia1 level, where the ostomy exits the peritoneal cavity. The stoma may be very gently probed and serially dilated with lubricated Hegar dilators to exclude this possibility. This should only be done by a pediatric surgeon, however, because neonatal bowel is quite fragile and highly susceptible to perforation even with the gentlest manipulations. Meticulous ostomy care is essential to prevent the significant complications of skin inflammation, excoriation, and ulceration caused by chronic contact with intestinal contents. Parents should be instructed in routine ostomy care and be provided with effective skin barrier creams and properly fitting ostomy appli-

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ances and bags. An experienced enterostomal therapist should be available for teaching routine ostomy care and ”troubleshooting” any problems that may arise. Colostomy

Colostomy may be required in infants with complications of necrotizing enterocolitis, colonic atresia, Hirschsprung’s disease, and high anorectal malformations. The site of colostomy depends on the pathologic process for which the colostomy is being performed. For example, in patients with Hirschsprung’s disease, a leveling colostomy is performed, in which the colostomy is brought out just above the transition zone from ganglionated to aganglionated bowel (i.e., the level at which ganglion cells are present). In general, stool from a rightsided colostomy is more liquid in consistency and more frequent, whereas stool from a left-sided colostomy is more formed and intermittent. Therefore, a rightsided colostomy should be more elevated from the skin surface to prevent skin excoriation caused by chronic contact with liquid intestinal contents, whereas a left-sided colostomy may be constructed more flush with the skin. Depending on the amount of diversion required, a colostomy may be either partially diverting, as in a loop colostomy, or totally diverting, as in an end colostomy. Stricture or obstruction of the colostomy may be manifest by passage of pencil-thin stools or cessation of bowel movements associated with bilious emesis and abdominal distention. Parents must be taught to recognize the complications associated with colostomy, including prolapse, fistula, and stricture or obstruction and to seek medical attention promptly should these problems arise. Meticulous skin and ostomy care is essential to prevent pToblems with skin excoriation, inflammation, and ulceration from contact with fecal materials, and the assistance of an experienced enterostomal therapist should be enlisted. GASTOESOPHAGEAL REFLUX DISEASE

Gastroesophageal reflux disease (GERD) occurs commonly in newborn infants, especially premature infants, in whom the lower esophageal sphincter (LES) is not fully mature. GERD is also observed frequently in neurologically impaired infants, as well as in infants who have undergone repair of esophageal atresia and tracheoesophageal fistula (EA/TEF) and congenital diaphragmatic hernia. In most series, neurologically impaired infants constitute the largest population requiring surgical intervention for GERD. Although the mechanism that predisposes these infants to GERD is not clear, it is theorized that central nervous system dysfunction has an adverse effect on foregut m0tility.4~Infants with tracheoesophageal fistulae often have abnormal esophageal peristalsis, promoting pathologic reflux. Mobilization of the distal esophageal segment during tracheoesophageal fistula repair may also alter the anatomy of the gastroesophageal junction and contribute to the development of GERD. The incidence of GERD in infants with congenital diaphragmatic hernia approaches 35%.30,5o Closure of the diaphragmatic defect places tension on the esophageal crura, displacing the left crura laterally, thus significantly distorting the anatomy of the gastroesophageal junction. Although hiatal hernia is identified in only approximately 3% to 6% of infants with pathologic GERD, its presence generally necessitates surgical intervention.I6 Placement of a gastrostomy tube may also predispose infants to GERD because of opening of the angle of His, lowering of

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LES pressure, and reducing LES length." Because of this complication, some surgeons have a low threshold for performing antireflux procedures concomitantly in patients requiring gastrostomies. Most premature and neurologically intact infants "outgrow" their GERD with time and appropriate medical therapy. Infants with severe neurologic deficits and those with anatomic reasons for GERD tend to be more recalcitrant to medical therapy and eventually require surgical intervention. The evaluation of infants with suspected GERD begins with a detailed history and physical examination. GERD may be manifest by frequent emesis, recurrent upper respiratory tract infections, or failure to thrive. Apnea and bradycardic episodes may predominate in the premature infant. Physical examination may reveal the presence of Sandifer's syndrome, in which infants arch their backs and turn their heads to the side to avoid the reflux. GERD may be confused with pyloric stenosis in newborn infants. Infants with recurrent, postprandial, nonbilious emesis may be incorrectly diagnosed with GERD, empirically started on antireflux medications, and discharged from the intensive care nursery. At home, these patients have progressive symptoms despite maximal medical management and multiple changes in feeding regimens. It soon becomes apparent to the astute pediatrician that these patients do not have GERD but rather pyloric stenosis. The symptoms may be quite similar and occur in the same age group of patients. A high degree of suspicion is necessary to differentiate the two disease processes. Diagnostic evaluation of GERD includes an upper gastrointestinal (UGI) series, gastric emptying scintigraphy scan, and a pH prbbe. Although the UGI series may demonstrate reflux, it is obtained primarily to delineate the anatomy and function of the esophagus, stomach, and duodenum. The overall sensitivity of the UGI series for GERD is approximately 85Y0.l~Unsuspected abnormalities affecting gastric emptying, such as pyloric stenosis, malrotation, or duodenal web, may be identified. The gastric emptying scintigraphy scan provides a quantitative measure of gastric emptying and is more accurate than a UGI series. It may also demonstrate gastroesophageal reflux and aspiration of gastric contents into the tracheobronchial tree. Patients with significantly delayed gastric emptying may require concomitant pyloroplasty with fundoplication. The gold standard for the diagnosis of GERD, however, remains the 24-hour pH probe, with a sensitivity of approximately 90% and specificity of approximately 95%.'', l4 After a diagnosis of pathologic GERD is established, a trial of medical management is indicated. Medical management includes positional therapy; thickened, smaller, more frequent feeds; and administration of standard antireflux medications, including ranitidine or omeprazole, metoclopramide, and cisapride. Effective positional therapy includes 24-hour maintenance of an upright ~ ~ .prone ~ ~ posture posture of 60- or the prone position with 30" e l e ~ a t i o n .The with 30" elevation may in fact be Infant seats that flex the thighs onto the abdomen tend to increase intra-abdominal pressure and exacerbate reflux and generally should be avoided.43aH,-antagonists (i.e., ranitidine) or proton pump inhibitors (i.e., omeprazole) decrease gastric acid production, whereas prokinetic agents (i.e., metoclopramide and cisapride) stimulate esophageal peristalsis, increase LES pressure, and increase gastric emptying. Indications for surgical intervention for GERD include failure to respond to maximal medical management, unremitting emesis, failure to thrive, recurrent aspiration pneumonitis, apnea, refractory reactive airway disease, severe esophagitis, esophageal stricture, and hiatal hernia.16The Nissen fundoplication, a 360" complete wrap, is the most commonly performed antireflux procedure. A 270" partial wrap (i.e., anterior Thal or posterior Toupet fundoplication) may be

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indicated in patients with significant underlying esophageal dysmotility, such as those with marked neurologic impairment or those with esophageal atresia or tracheoesophageal fistula. A functional gastroesophageal junction obstruction may be imposed by a 360" complete wrap in patients with abnormal esophageal peristalsis if it is constructed too tightly. Because the Nissen fundoplication is a complete wrap, the incidence of recurrent reflux is low, but the patient may lose the ability to vomit and may develop gas bloat syndrome. A decompressive gastrostomy may be a useful adjunct to Nissen fundoplication to prevent gas bloat syndrome. Although patients with partial wraps retain the ability to vomit and are less prone to gas bloat syndrome, the incidence of recurrent reflux is probably higher. The choice of procedure must be individualized. Bowel obstrixtion in preverbal patients who have undergone antireflux procedures poses a serious problem because the ability to vomit is impaired, thus preventing adequate gastric decompression. Inability to decompress the stomach proximal to an obstruction may result in significant gastric distention, infarction, and perf0rati0n.l~ Chronic retching and gagging may also cause disruption of the wrap. In cases of suspected obstruction, a nasogastric tube should be expeditiously placed to decompress the stomach. If a gastrostomy tube is in place, it should be vented immediately at the first sign of obstruction. Parents should become familiar with appropriate venting of the gastrostomy tube and be encouraged to seek medical attention early to prevent significant complications. ESOPHAGEALATRESIAANDTRACHEOESOPHAGEAL FISTULA

Infants with EA/TEF often have other congenital defects as well, most commonly the VACTERL complex, which includes vertebral anomalies, anorectal malformations, cardiac anomalies, EA/TEF, renal anomalies, and radial limb anomalies. All newborn infants with EA/TEF should undergo a complete evaluation to exclude the VACTERL complex, including spinal radiographs and sonography to rule out vertebral anomalies and tethered cord, thorough examination of the perineum and rectum, cardiac echocardiogram, renal sonography, and limb radiographs. A variety of complications may occur in infants following repair of EA/ TEF, including anastomotic leaks, anastornotic strictures, GERD, recurrent TEF, and associated tracheomalacia. Parents must be made aware of and prepared for these potential complications because they occur quite commonly. Anastomotic leaks occur in the early postoperative period while infants are still in the intensive care nursery. Most anastomotic leaks are small, recognized early, and successfully managed nonoperatively with bowel rest, appropriate drainage, and antibiotic therapy; however, if the patient appears toxic, urgent re-exploration should be performed. Large leaks may require a diverting cervical esophagostomy. A concomitant gastrostomy is useful to allow for enteral feeding during the healing process. The significance of an anastomotic leak in intensive care nursery graduates is the predisposition to development of an anastomotic stricture after discharge to home. Because of natural anastomotic narrowing and intrinsic esophageal dysmotility following EA repair, feeding difficulties may be expected. Parents should be instructed to feed their infants more slowly and burp them frequently. Smaller, more frequent feeds may be better tolerated. Stricturesmay not become apparent until infants undergo the transition from formula to pureed or soft baby foods.

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After infants are advanced to solids, foods should be cut into small portions and chewed carefully before swallowing. Troublesome foods, such as hot dogs, large pieces of meat, popcorn, and uncooked vegetables, should be avoided. Anastomotic strictures occur commonly after EA repair. Factors that predispose to the development of anastomotic strictures include ischemia, excess tension, anastomotic leak or dehiscence, two-layer anastomosis, and GERD.4The most common cause for late anastomotic stricture is GERD.4 Parents should suspect stricture formation if the infant develops feeding difficulties, regurgitation, or dysphagia. Older patients may develop recurrent food impactions. Diagnosis is established by barium esophagogram or esophagoscopy. Treatment is with esophageal dilatation. Multiple dilatations may be required. Tight or extensive stricture formation unresponsive to dilatation may require esophageal resection and replacement. Gastroesophageal reflux disease occurs frequently after EA repair. Associated esophageal dysmotility allows for prolonged exposure to refluxed acids, which may increase the severity of mucosal injury. Stricture may occw either at the anastomosis or in the distal esophagus. Approximately 25% of patients require an antireflux procedure because of significant GERD.4 Recurrent TEF may be heralded by coughing, gagging, choking, cyanosis, apnea, and recurrent pneumonias. Wants may cough and sputter with each feed. If suspected, prone cine esophagography or bronchoscopy may establish the diagnosis. Treatment is with thoracotomy and division of the fistula. Tracheomalacia of varying degrees occurs in almost all infants with EA/ TEE At the mild end of the spectrum, infants can be-expected to have a chronic barking or "seal-like" cough, which may persist until 4 or 5 years of age. Parents should be assured that this is a "normal" finding that will resolve with time. Humidified air may be helpful to minimize symptoms in the interim. Parents should also be aware that these infants may have significantly increased difficulties with viral upper respiratory tract infections and croup because of the underlying tracheomalacia. At the severe end of the spectrum, infants may develop cyanosis, bradycardia, inspiratory and expiratory stridor, or apnea either during or shortly after feedings. Diagnosis of tracheomalacia is established either by bronchoscopy or cine computed tomography scan of the trachea. With severe, life-threatening symptoms, such as apnea, aortopexy or tracheal stenting may be performed. Less severe symptoms tend to improve by 1 or 2 years of age. NECROTIZING ENTEROCOLITIS Necrotizing enterocolitis (NEC), with its predilection for premature or lowbirthweight infants, is the most commonly encountered surgical and gastrointestinal emergency in the intensive care nursery. About half to two thirds of cases of NEC are successfully managed nonoperatively with fluid resuscitation, hemodynamic support, bowel rest, and broad-spectrum antibiotics. The remainder require surgical intervention, which generally includes resection of gangrenous bowel with diverting stoma formation. Closure of the stomas and restoration of intestinal continuity is generally delayed for at least 6 to 8 weeks. Earlier closure may be required in infants with excessive stoma output, marked electrolyte abnormalities, cholestatic jaundice caused by hyperalimentation, or failure to thrive. Stoma1 stenosis occurs commonly because of the tenuous, friable nature of the exteriorized bowel in patients with NEC. When this occurs, most surgeons opt for early closure rather than revision of the stoma. The late

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sequelae of NEC include short gut syndrome, malabsorption, intestinal stricture, and internal fistula formation. Recurrent NEC has also been reported.53 Short gut syndrome occurs as a result of extensive bowel resection, which may be required in cases of severe NEC. Infants with short gut syndrome often require prolonged hyperalimentation and are thus susceptible to all of the complications associated with prolonged hyperalimentation. Enteral feeds, often elemental, must be advanced very gradually to allow intestinal adaptation to occur. This may be a long, frustrating process for patients, parents, and caregivers alike. Although malabsorption may occur as a result of short gut syndrome, it may also occur in infants with sufficient intestinal length and instead be caused by the extensive mucosal injury incurred by NEC. Intestinal absorptive capacity generally improves as the mucosa heals. Elemental formulas may be beneficial to allow for maximal absorption during the healing process. Intestinal stricture occurs in approximately 15% to 25% of patients with NEC3 and is most common following nonoperative treatment of patients with NEC. The most common site of stricture is the colon (To%), followed by the terminal ileum (15"/0).~The left side of the colon is most commonly affected (6O%), with the most common colonic site being the splenic flexure (2iY0).~ Strictures may be single or multiple. In patients managed operatively, intestinal strictures may be asymptomatic because the stricture usually develops distal to the stoma. Therefore, a barium enema before stoma takedown is essential to identify any distal strictures. If a stricture is found, it may be resected during the procedure to close the stoma. Patients undergoing nonoperative treatment for NEC should have a barium enema 4 to 6 weeks after the acute episode of NEC or before discharge from the intensive care nursery to exclude stricture formation. Symptomatic patients require resection of the strictured segment. The treatment of asymptomatic strictures is less clear, with treatment options being observation, balloon dilatation: or resection. MALROTATION

The most common long-term complication of infants undergoing surgery for malrotation with midgut volvulus is development of short gut syndrome caused by the extensive bowel resection often required for this condition. In fact, malrotation with midgut volvulus is responsible for approximately 18% of cases of short gut syndrome in children." The major morbidity and mortality associated with malrotation with midgut volvulus arises primarily from complications of long-term total parenteral nutrition required for short gut syndrome. Although postoperative adhesions are probably the most likely cause of bowel obstruction after surgical correction of malrotation with midgut volvulus, the possibility of recurrent volvulus must be considered also. The incidence of recurrent volvulus has been reported to be between OYOand 10'7'0,5~, 52 and it is thought to arise from inadequate adhesion formation within the peritoneal cavity to maintain appropriate fixation or from insufficient widening of the mesenteric base. Lastly, slowed gastrointestinal motility may occur following operation for malrotation with midgut volvulus. Defective intrinsic enteric innervation has been implicated.", l5 Gradual advancement of feeds, use of prokinetic agents, and patience by parents and caregivers may be necessary. Malrotation with midgut volvulus is a true surgical emergency. It should be stressed to parents and primary pediatricians that bilious emesis is a severe

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symptom that should prompt urgent evaluation. Bilious emesis should be attributed to malrotation with midgut volvulus until proven otherwise. A UGI contrast study is useful to make the diagnosis and should be performed expediently after the diagnosis is suspected. Early recognition and management are essential to preventing the significant morbidity and mortality often associated with this condition. HIRSCHSPRUNG’S DISEASE

Hirschsprung’s disease, or congenital intestinal aganglionosis, is usually diagnosed during the neonatal period. Newborns classically present with bilious emesis, abdominal distension, failure to pass meconium, or severe constipation. Abdominal radiographs demonstrate an obstructive pattern. Barium enema may demonstrate a narrow rectum, transition zone from proximal dilated ganglionic bowel to distal decompressed aganglionic bowel, and failure to pass barium from the rectum within 24 hours. In the early newborn period, however, the barium enema may be nondiagnostic. Definitive diagnosis is established by suction or full-thickness rectal biopsy taken 2 cm or 3 cm above the dentate line, which reveals the absence of ganglion cells in the submucosal plexus and hypertrophied nerve fibers. After the diagnosis of Hirschsprung’s disease has been established, treatment is surgical. Traditionally, this has been perfomied in two stages, with the first stage being a leveling colostomy created at the transition zone between ganglionic and aganglionic bowel and the second stage being a definitive endorectal pull-through procedure, usually performed at 6 to 9 months of age. The most commonly performed operations are the Save-Boley and Duhamel endorectal pull-through procedures. Recent technical advances include one-stage open endorectal pull-through procedures and laparoscopic approaches, often performed during the neonatal period. Longer follow-up is necessary to determine whether outcomes associated with these newer techniques are comparable with the traditional two-stage procedures, but preliminarily they seem quite promising. With the traditional two-stage approach, infants are usually discharged home from the intensive care nursery with a colostomy and scheduled for a definitive pull-through procedure at 6 to 9 months of age. Enterocolitis is the most severe complication of Hirschsprung’s disease, with a reported incidence of 20% to 58%.7,’8,49 Parents should be aware that these infants remain at risk for enterocolitis despite a colostomy and even after the definitive pull-through procedure, especially if there is a history of a previous episode of enterocolitis. The clinical presentation of enterocolitis may include fever; lethargy; abdominal distension; and profuse, foul-smelling diarrhea. Sepsis and toxic megacolon may also occur. Parents should be instructed to seek medical attention for their infants immediately if any of these symptoms and signs appear because the morbidity and mortality of enterocolitis may be quite high. Early recognition and treatment with intravenous fluid resuscitation, broad-spectrum antibiotics, and frequent rectal or colostomy irrigations are essential to a good outcome. After the endorectal pull-through procedure has been performed, the perineum may become severely excoriated and ulcerated from contact with stool, which initially may be loose and frequent. In most cases, the perineum has been protected from stool contact for a prolonged period of time because of the presence of a colostomy and may be exquisitely sensitive. Parents must cleanse the perineum gently and thoroughly after each bowel movement, pat the area

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dry, and apply a protective balm. A specially formulated protective balm, consisting of sucralfate, cholestyramine, and petrolatum ointment (”Dr. Glick‘s Butt Balm”), is used at the authors’ institution and has been particularly effective for prevention and treatment of perineal excoriation in these patients. Meticulous care is necessary to prevent troublesome skin breakdown. Common problems after endorectal pull-through procedures for Hirschsprung’s disease may include varying degrees of diarrhea, constipation, fecal soiling, and incontinence. Because of these problems, bowel training may be quite difficult and may take longer than for normal infants. Much patience, perseverance, and encouragement are required. Rectal adaptation occurs with time in most cases. Overall, by the age of 4 years, approximately 75% of patients can be expected to achieve good to excellent anorectal control, regardless of the type of pull-through procedure perf0rmed.3~Approximately 20% of patients have relatively minor problems with constipation, fecal soiling, and incontinence, whereas 5% experience severe problems with fecal soiling and incontinence, resulting in significant psychosocial maladj~stment.~~ Infants with associated Down’s syndrome or neurologic impairment tend to fare worse. Severe problems with constipation and incontinence after endorectal pull-through for Hirschsprung’s disease may suggest coexistent neuronal intestinal dysplasia of the remaining ganglionic segment. ANORECTAL MALFORMATIONS

Anorectal malformations occur in approxhnately 1 in 5000 newborn infants and are commonly associated with other congenital defects, most commonly the VACTERL complex. The major morbidity and mortality in these infants may in fact be related to the severity of the associated anomalies, especially cardiac and renal defects, rather than the anorectal malformation. All newborn infants with anorectal malformation should undergo a complete evaluation, as previously discussed, to exclude the VACTERL complex. The first step in the diagnosis of anorectal malformation is a complete examination of the perineum to determine the presence or absence of an anal orifice, the location of the anal orifice, and whether a fistula is present. A speck of meconium on the perineum suggests an anocutaneous fistula. The presence of a perineal fistula implies a low defect. Passage of meconium or flatus from the urethra indicates a rectourethral or rectovesicular fistula, whereas passage of meconium from the vagina indicates a rectovaginal fistula. The presence of a rectourethral, rectovesicular, or rectovaginal fistula or imperforate anus without an obvious fistula suggests a high defect. The importance of distinguishing low versus high anomalies is that the treatment is quite different. Treatment varies slightly between male and female infants as well. Algorithms for treatment of newborn male and female infants with anorectal malformations are presented in Figures 2 and 3. In general, low lesions do not require colostomy and are treated in the neonatal period by perineal anoplasty. High lesions require diverting descending colostomy followed by definitive posterior sagittal anorectoplasty (PSARP) or posterior sagittal anorectovaginourethroplasty (PSARWP) at 3 to 12 months of age. A distal colostogram should be obtained routinely before definitive repair to delineate the exact anatomy of the defect. Specific issues in girls with cloaca1 anomalies are beyond the scope of this article, and readers are referred to pediatric surgical textbooks for the details involved in these complicated defects. Two weeks following definitive repair, serial anal dilatations with lubricated

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BROWN et a1 Newborn male with anorectal malformation

r

Observation 18-24 h. Abdominal ultrasound

i

Perineal inscadion

Clinical evidence

Perineal fistula "Bucket Handle" Midline Raphe fistula

No clinical evidence (Questionable)(10-20%)

"Flat Bottom" Meconium in urine

Cross-table lateral film with patient in prone position

/ \

I Colostomy

I

>1 cm Bowel-skin distance

4 crn Bowel-skin distance

4-0 weeks Rule out associated maHormations Verify normal growth

4 Minimal PSAP no colostomy

+ PSARP

1 Minimal PSAP no colostomy

Figure 2. Treatment of the newborn male with anorectal malformation (ARM).

Hegar dilators are begun. The first dilatation is performed by the pediatric surgeon, with subsequent dilatations performed by the family twice a day. Each week, the size of dilator is increased, usually by 1-mm caliber increments until the desired size is reached. After the desired size is achieved (usually 2-3 mo following definitive repair), the colostomy may be closed; however, the dilatation process must be continued with gradual tapering over time. The importance of postoperative dilatation to avoid development of anal stricture cannot be overemphasized. Once the colostomy is closed, the infant may develop a severe diaper rash caused by multiple bowel movements and exquisitely sensitive perineal skin. A variety of commercial protective balms are available that typically consist of various combinations of vitamin A and D ointment, aloe, neomycin, zinc oxide, aluminum and magnesium hydroxide, sucralfate, and cholestyramhe. "Dr. Glick's Butt Balm," consisting of sucralfate, cholestyramine, and petrolatum ointment has been used extensively at the authors' institution for this purpose. Parents may create their own concoctions as well. A constipating diet may be useful to add bulk to the stool. The major problems following PSARP or PSARVUP include constipation, fecal soiling, and incontinence.MIn the early postoperative period, some signs predict future problems. Good prognostic signs include one to three bowel movements per day without soiling in between, sensation when passing stool (i.e., pushing or making faces), normal sacral anatomy, well-formed and contoured buttocks, and urinary continence.44Poor prognostic indicators include constant fecal soiling, absence of sensation during defecation, abnormal sacral anatomy, flat bottom, and urinary incontinence.44

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Constipation may occur early in the postoperative period following colostomy closure and is most often the result of a hypomotility disorder of the ectatic rectum. Left untreated, hypomotility results in incomplete evacuation of the rectosigmoid colon, constipation, and impaction. Postoperative constipation is treated with individualized laxative regimens, with enemas used as a last resort. In infants with a good prognostic defect, fecal soiling is a result of constipation and chronic impaction, rather than incontinence, and is treated with an individualized laxative regimen." In infants with poor prognostic defects, fecal soiling denotes incontinence, and a trial of a bowel management program that involves the use of enemas and colonic irrigations, dietary manipulations, and constipating medications (i.e., Lomotil or Imodium) is indicated.MFailure to respond to an aggressive bowel management program may require permanent diversion. Problems with fecal incontinence after PSARP or PSARVUP require a complete evaluation, including detailed history, review of operative records, physical examination, radiographs of lumbosacral spine and determination of the sacral ratio, contrast enema to determine length of remaining colon and to assess motility, magnetic resonance imaging to assess position of anus in relation to sphincter mechanism and rule out tethered cord, and voiding cystourethrogram and renal sonography." An algorithm for treatment of fecal incontinence following repair of anorectal malformations is presented in Figure 4. Pediatricians and pediatric surgeons are often called upon to evaluate infants for possible ectopic anuses. It is important to carefully examine the perineum of many normal infants and take note of the proper anatomic relationships

Newborn female with anorectal malformation Observation 16-24 h. Abdominal ultrasound /Penneal

Fistula (95% of cases)

inspection

\

N; fistula (5%)

1

Cloaca

Cross-table lateral film with patient in Drone wsition

/'\

Emergency GU evaluation 1

Colostomy and it necessary Vaginostorny; urinary diversion

6 Months

PSARVUP

I

Colostomy

1

4 cm Bowel-skin distance (extremely unusual)

-

> I cm Bowel-skin distance or questionable

4-8 weeks Colostomy Rule out associated malformations 4-8 weeks verify normal Rule out associated malformations

Limited PSARP

Verify normal growth

I

PSARP

Figure 3. Treatment of the newborn female with anorectal malformation (ARM).

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BROWN et a1 Evaluation(contrast enema, sacrum, VCUG. MRI. history, exam)

(b.dP W n O W ~ o o sacrum d Originally good defect' Poor sacrum Good-looking perineum Originally very high defect Mislocated rectum Flat bottom, paucity VSMt No sensation Poor bowel movement pattern

/ 1

4

+

Good sacrum Originally good defect Good sphincter Well-located rectum Severe constipation

I

t

Bowel management program

Sigmoid resection

Tmlnable Good sacrum Originally good defect Good sensation Well located reclum Good bowel movement pattern

I

+

Bowel training program

/ \

Unsuccessful

Successful

I

Consider continent appendiscotomy

I

Permanent ColOstMny

Figure 4. Treatment of fecal incontinence after repair of anorectaf malformation (ARM).

so that one may recognize an abnormal perineum and ectopic anus. Although it is useful to note the distance between the anus and the vaginal fourchette or base of the scrotum (i.e., the perineal body), this may be quite variable and is not completely reliable. The most important aspect of the examination is to determine whether the anus is correctly situated between the sphincter muscles. One clue to proper positioning is that the anus is surrounded by pigmented anoderm. Stimulation of the anal wink reflex is also helpful because the sphincter muscles should contract around the normally positioned anus. Infants with an ectopic anus may also have a palpable rectal shelf on digital examination. Rectal examination should also include careful palpation of the contour of the sacrum to exclude any associated sacral anomalies or masses. If one is concerned about the possibility of an ectopic anus and cannot demonstrate proper position of the anus between the sphincter muscles on examination in the office, referral to the pediatric surgeon should be made. Examination under anesthesia using the nerve stimulator may be helpful.

BlLlARY ATRESIA

Biliary atresia occurs in approximately 1 in 10,000 to 20,000 newborns and is the most common cause of surgical jaundice in infants. The clinical presentation of biliary atresia includes jaundice with direct hyperbilirubinema, dark urine, acholic stools, and hepatomegaly. Jaundice in newborns persisting beyond 2 weeks cannot be simply attributed to physiologic jaundice and demands prompt and thorough evaluation. The major objective is to distinguish between obstructive jaundice versus jaundice caused by hepatocellular dysfunction. Hepatocellular dysfunction may result from a variety of infectious, hematologic,

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metabolic, and genetic disorders. Complete evaluation of jaundiced infants includes complete blood count, liver function tests, coagulation tests, hepatitis serologies, toxoplasmosis, rubella, cytomegalovirus, herpes (TORCH) titers, a-1antitrypsin level, sonography, HIDA scan, and exploratory laparotomy with operative cholangiography and liver biopsy.Z7Most infants diagnosed with biliary atresia should undergo hepatic portoenterostomy. For optimal results, the procedure should be performed before 8 weeks of age, thus emphasizing the importance of early diagnosis. Postoperatively,infants are begun on prophylactic oral antibiotics (i.e., trimethoprim-sulfamethoxazole) to reduce the incidence of cholangitis. Prophylactic oral antibiotics are generally continued for 3 to 5 years if the child remains asymptomatic. A variety of choleretic agents, including corticosteroids, phenobarbital, glucagon, prostaglandins, cholestyramine, dehydrocholic acid, and ursodeoxycholic acid have been used to maximally stimulate bile flow during the early postoperative period. Enteral feeds are begun as early as possible, and formulas high in medium-chain triglycerides (i.e., Pregestamil, Mead-Johnson) are preferred because of impaired fat absorption. Fat-soluble vitamins A, D, E, and K must be administered daily. The major complications after hepatic portoenterostomy for biliary atresia include cholangitis, malnutrition, fat-soluble vitamin deficiencies, and progressive hepatic failure with portal hypertension resulting in ascites, splenomegaly or hypersplenism, and bleeding esophageal varices. Cholangitis is the most common and most severe complication, occurring in approximately 40% to 100% of infants.Z8,35,42 The clinical presentation of cholangitis includes fever, worsening jaundice, decreased bile flow, acholic stools, and leukocytosis. Treatment includes administration of intravenous broad-spectrum antibiotics and choleretic agents, often including pulse doses of corticosteroids with rapid tapering. Early recognition and treatment of cholangitis is critical because severe and recurrent episodes may result in progressive deterioration of hepatic function and cessation of bile flow. With appropriate therapy, symptom$ usually resolve within 24 to 48 hours, but jaundice may persist for up to 1 week. The major determinants of long-term survival following hepatic portoenterostomy include the patient’s age at operation, establishment of bile flow, presence of microscopically detectable hilar ductal structures, degree of hepatic parenchymal disease, and the technical ease of the 0peration.4~Good prognostic indicators include early diagnosis, establishment of adequate bile flow, and complete resolution of jaundice. Poor prognostic indicators include late diagnosis, failure to establish bile flow, and persistent, progressively worsening jaundice and hepatic dysfunction. The 10-year survival of all infants with biliary atresia 34 Liver transtreated with hepatic portoenterostomy ranges from 30% to 5!%0.~~, plantation is considered a complementary or rescue operation and is generally reserved for infants who are diagnosed late, fail to establish adequate bile flow after hepatic portoenterostomy, or demonstrate progressive hepatic failure despite partial relief of biliary obstruction by hepatic portoenterostomy. It is important for primary care providers and pediatric surgeons to be able to recognize impending failure of hepatic portoenterostomy and refer patients earlier rather than later for liver transplantation. ABDOMINAL WALL DEFECTS

Omphalocele

The major morbidity of omphalocele in newborns is that of associated anomalies, the rate of which approaches 50%. Associated anomalies may include

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severe chromosomal aberrations (trisomy 13,18, and 21), prune belly syndrome, and Beckwith-Wiedemann syndrome. Epigastric omphalocele may occur as a component of the pentalogy of Cantrell, which includes omphalocele, central diaphragmatic defect, pericardial defect, sternal defect, and various congenital cardiac defects. Hypogastric omphalocele may coexist with bladder or cloaca1 exstrophy, vesicointestinal fissure, colon atresia, imperforate anus, sacral vertebral defects, and meningomyelocele. Infants with omphalocele may also have an increased incidence of Wilms’ tumor, neuroblastoma, and adrenocortical tumors. Many infants with omphalocele are premature. The prognosis for infants with associated anomalies is much worse than for those with an isolated defect. Depending on the size of the omphalocele and the stability of the patient, operative approaches may include primary fascialclosure, gradual silo reduction with delayed fascial closure, or use of escharotic agents with delayed fascial closure. If the defect is large, prosthetic materials may be required to facilitate fascial closure. Infants may be left with sizeable ventral hernias that will require repair at a later date. In contrast to infants with gastroschisis, most infants with omphalocele tend to tolerate enteral feeds much sooner. Complications to be anticipated following omphalocele repair include prolonged pulmonary compromise, secondary infection of prosthetic materials, ventral hernia, recurrent small bowel obstruction caused by adhesions, and gastric outlet obstruction caused by excessive splenic or hepatic compression.” These infants also have an increased incidence of GERD and inguinal hernia.” Gastroschisis

Considerably fewer associated anomalies are identified in infants with gastroschisis (21% versus 54%) but may include malrotation with potential for midgut volvulus, intestinal atresia (most commonly jejunoileal atresia), Meckel’s diverticulum, and intrauterine growth retardation.” The operative approach is similar to that for omphalocele and includes either primary fascial closure or gradual silo reduction with delayed fascial closure. Infants with gastroschisis tend to have markedly delayed intestinal motility following repair and may require prolonged hyperalimentation. Enteral feeds must be introduced and advanced gradually. Failure to tolerate slow advancement of enteral feeds in infants with gastroschisis should prompt a UGI contrast study to exclude coexistent intestinal atresia. Potential complications related to gastroschisis include growth retardation in as many as 30% of infants, short gut syndrome, increased incidence of NEC, cholestatic jaundice caused by prolonged hyperalimentation, prolonged pulmonary compromise, ventral hernia, small bowel obstruction caused by adhesions, and cryptorchidism.” As in omphalocele, these infants are also more prone to GERD and inguinal hernia.” DISORDERS OF THE UMBILICUS

Infants discharged from the intensive care nursery may have umbilical stumps in various stages of healing. Meticulous care of the umbilical stump is important to prevent severe infection. Omphalitis may present initially with mild erythema and drainage but if left untreated may rapidly progress within hours to widespread cellulitis, fasciitis, and sepsis. Early recognition and treatment with appropriate intravenous antibiotics is critical. Fulminant omphalitis

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may progress to highly lethal necrotizing fasciitis manifest by rapidly progressive erythema, abdominal wall induration, and discoloration of the umbilicus.z4 In addition to broad-spectrum antibiotics, treatment includes wide debridement of necrotic skin, subcutaneous tissue, fascia, umbilical vessels, and urachal remnant.31 An umbilical granuloma may form following separation of the cord and appears as a mass of beefy red granulation tissue at the base of the umbilicus. It may be associated with persistent oozing of serous fluid and mild surrounding erythema. It may be eradicated by several applications of silver nitrate. Failure to respond to silver nitrate may suggest an umbilical polyp rather than an umbilical granuloma. An umbilical polyp similarly is a glistening red nodule at the base of the umbilicus, which is a remnant of the vitelline duct. It may contain small bowel or gastric mucosa. Treatment is with excision, including any contiguous intra-abdominal vitelline duct vestiges. If the specimen is removed in the pediatrician’s office, it should be sent to the pathologist for histologic examination. Other disorders of the umbilicus may include urachal cysts and sinuses and patent omphalomesenteric cysts and sinuses. Urachal sinuses may drain urine, whereas patent omphalomesenteric sinuses may drain intestinal contents. Sonography or contrast injection of the sinus tract may occasionally be helpful in the diagnosis. Treatment involves excision of entire cyst and sinus tract down to its intra-abdominal attachment to either the bladder or bowel. Other unusual umbilical masses include dermoid cysts, vascular malformations, and ectopic liver. An umbilical hernia results from failure of closure of the fascial ring through which the umbilical cord protrudes at birth. Umbilical hernias occur most commonly in black infants and in premature, low birth weight infants. Infants with Down’s syndrome, congenital hypothyroidism, mucopolysaccharidoses, or Beckwith-Wiedemann syndrome also have an increased incidence of umbilical hernia. Umbilical hernias are usually recognized early in infancy as the cord sloughs and the umbilicus heals. They are usually asymptomatic,rarely incarcerate, and spontaneously close by age 3 or 4 years in most patients. The two most important factors predictive of spontaneous closure include age of the patient and size of the fascial defect. In a classic study by Walker57involving the 6-year follow-up of 314 black infants younger than 3 months of age with umbilical hernia, 96% of fascial defects less than 0.5 cm closed spontaneously, whereas none of the defects more than 1.5 cm closed Spontaneously. Operative repair is indicated for failure of the fascial defect to close by age 3 or 4 years, the presence of a large fascial defect with a proboscis-like hernia, and incarceration. INGUINAL HERNIAS AND HYDROCELES

Inguinal hernias are among the most common congenital disorders that pediatricians and pediatric surgeons encounter. Frequently, the diagnosis is made while these infants are still in the intensive care nursery, especially in premature infants in whom the incidence has been reported to be as high as 30%.8,25, 46 Infants with cystic fibrosis, connective tissue disorders, abdominal wall defects, bladder exstrophy, or congenital dislocation of the hip; preterm infants with intraventricular hemorrhage; and infants with meningomyelocele and hydrocephalus requiring ventriculoperitoneal shunts all have an increased incidence of inguinal hernia. Because of the high risk for inguinal hernia and incarceration, which exceeds 60% during the first 6 months of life, most pediatric surgeons prefer to repair inguinal hernias in infants before their discharge from

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the intensive care nursery8 If an infant is discharged from the intensive care nursery before inguinal hernia repair, parents must be taught to recognize the clinical presentation of incarceration, including irritability, crampy abdominal pain, and vomiting in association with a firm, tender mass in the groin, often extending into the scrotum. Parents should be instructed to seek medical attention for the infants immediately if an incarcerated inguinal hernia is suspected to avoid strangulation with subsequent infarction of bowel or the testicle in boys. If recognized early, approximately 80% to 90% of incarcerated inguinal hernias can be reduced nonoperatively with gentle, constant pressure. Sedation may occasionally be required. Strangulated, nonviable bowels are extremely unlikely to be reduced without operation. After the incarcerated inguinal hernia is reduced, infants are admitted to the hospital and observed, and hernias are repaired electively the following day. If the hernia cannot be reduced nonoperatively, the infant is taken emergently to the operating room for reduction and repair. The complication rate for emergent repair of incarcerated hernias exceeds 20%, whereas the complication rate for elective repair is only approximately 1.5%. In premature infants in whom inguinal hernias develop following discharge from the intensive care nursery, surgery should not be delayed. If surgery is performed before 60 weeks' postconceptual age, admission is required postoperatively for monitoring because of the increased risk for apnea and bradycardia. Spinal anesthesia may be preferred in these high-risk infants. Term infants should undergo outpatient repair electively after the inguinal hernia is recognized. The major complications of inguinal hernia repair include recurrence, iatrogenic cryptorchidism, and injury to the spermatic cord structures. Wound infection is distinctly unusual and occurs in less than 1% of cases. The recurrence rate after uncomplicated hernia repair is approximately 0.5% to 1.0% in term infantss," and is as high as 2%in premature infant^.^^,^ The incidence increases after repair of incarcerated hernia to approximately 3% to 6%.8,lo Recurrences are more common in premature infants and in those with persistently increased intra-abdominal pressure following repair, including those with cystic fibrosis, connective tissue disorders, ascites, or ventriculoperitoneal shunts. Recurrence may also occur as a result of a missed direct inguinal or femoral hernia. Iatrogenic cryptorchidism is a preventable problem and most often results from failure to carefully place the testis back into the intrascrotal position following inguinal hernia repair.26If the testis is highly retractile immediately following inguinal hernia repair and will not stay in the intrascrotal position, orchidopexy may be considered. If an undescended testis is recognized before or during inguinal hernia repair, concomitant orchidopexy should be performed. After inguinal hernia repair, parents and caregivers should examine the infant and confirm that the testes are properly placed in the intrascrotal position. If there is any question, the pediatric surgeon should be notified. Injury to the spermatic cord structures may occur during inguinal hernia repair and is probably most common in premature infants because of small, fragile structures and in infants with incarcerated inguinal hernias because of edematous, friable structures. The key to prevention of injury is recognition and gentle, meticulous dissection of spermatic cord structures. Hydroceles occur commonly in male infants and may be classified as either communicating or noncommunicating. A communicating hydrocele indicates free communication with the peritoneal cavity and should be treated as a hernia. Classically, parents describe a scrota1 mass that fluctuates in size. A noncommunicating hydrocele has no communication with the peritoneal cavity,

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does not fluctuate in size, and generally resolves spontaneously within the first 6 to 12 months of life. Operative repair is indicated for hydroceles that fail to resolve by 1 or 2 years of age. If the hydrocele ascends from the scrotum into the groin, differentiation from an incarcerated inguinal hernia may be difficult. Transillumination is not reliable for differentiating a hydrocele from a hernia because fluid-filled bowel has the same appearance as a hydrocele when transilluminated. If concern for an incarcerated hernia exists, immediate referral to a pediatric surgeon is a high priority. CRYPTORCHlDlSM Cryptorchidism, or undescended testis, occurs approximately in 0.5% to 1.0% of term infants and in approximately 5% of premature infants? The right side is affected in 50% of cases, whereas the left side is affected in 25% of cases6,22 Bilateral undescended testes occur in 25% of cases.6, This incidence parallels the incidence of inguinal hernias and the pattern of testicular descent. A cryptorchid testis may be intra-abdominal, at the level of the internal or external ring, high scrotal, or ectopic. A cryptorchid testis must be differentiated from the retractile testis because no operative intervention is required for the retractile testis. This requires a careful examination by a gentle examiner with warm hands. It may be helpful to examine the patient while he is either in the standing or the cross-legged sitting position. If the testis can be brought into the scrotal position, cryptorchidism is excluded, even if it retracts immediately on release. The parents may then be reassured that this is a normal variant, and that no operation is required. After discharge from the intensive care nursery, infants with cryptorchidism must be followed carefully with frequent, serial examinations both by their parents and their pediatricians. Orchidopexy is required for testes that fail to descend by 1 year of age because infants with cryptorchid testes are at increased risk for impaired fertility and malignant change affecting not only the undescended but also the contralateral descended testis. Orchidopexy does not reduce the incidence of malignant change but rather provides for easier detection. The major indications for orchidopexy are preservation of fertility, earlier detection of malignant change, prevention of trauma or torsion, and psychologic and cosmetic considerations. CONGENITAL DIAPHRAGMATIC HERNIA Congenital diaphragmatic hernia (CDH) occurs in approximately 1 in 5000 newborn infants or 1 in 2200 if stillborn infants are included." CDH is left-sided in approximately 80% of patients and right-sided in approximately 20% of patients. Associated anomalies are present in approximately 30% of infants with CDH and portend a worse pr0gnosis.5~Autopsy studies on stillborn infants with CDH report a 95% incidence of major associated anomalies, including anencephaly, meningomyelocele, hydrocephalus, encephalocele, ventricular-septal defects, vascular rings, coarctation of the aorta, trisomy 13 and 18, esophageal atresia, omphalocele, and cleft palate.' With major advances in neonatal intensive care strategies, including the use of exogenous surfactant, high-frequency ventilation, permissive hypercapnea, nitric oxide, and extracorporeal membrane oxygenation (ECMO), the reported survival of infants with CDH has improved dramatically over the years and approaches 80% in some centers. More recent

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advances in the treatment of CDH include the use of partial liquid ventilation with perflubron, the impact of which is yet to be fully e1~cidated.l~” Infants with CDH are among the most critically ill infants managed by neonatologists and pediatric surgeons. CDH survivors require extremely close follow-up after discharge from the intensive care nursery.41A multidisciplinary team approach is essential to facilitate early detection and prompt treatment of anticipated complications. Before discharge from the intensive care nursery, infants with CDH should have a baseline room air arterial blood gas, chest radiograph, and electrocardiogram performed. A complete baseline neurodevelopmental evaluation is mandatory, including a computed tomography scan of the head, electroencephalogram, and brainstem auditory evoked responses. At 6, 12, 24, and 36 months of age, CDH survivors should undergo a complete history and physical examination, room air arterial blood gas determination, chest radiograph, electrocardiogram, and pulmonary function tests, if possible. The need for hrther head CT scans, electroencephalograms,auditory evaluation, and ophthalmology evaluation is determined on an individual basis by the neurodevelopmental specialist. Frequent home nurse visits, nutritional counseling, physical therapy, and parent education are other important components of early intervention programs. The major long-term complications identified in CDH survivors include failure to thrive, GERD, developmental delay, sensorineural hearing loss, anterior chest wall deformities, scoliosis, bronchopulmonary dysplasia, and recurrent diaphragmatic hernia? Early on, because of pulmonary hypoplasia and abnormal lung development, infants with CDH may be more susceptible to the development of upper respiratory tract infections and pneumonias and experience a more severe, protracted clinical course when the): do occur. It is imperative that parents be instructed to seek prompt medical attention for their infants with the onset of any respiratory symptoms or fevers. Early recognition and treatment are essential to a successful outcome. interestingly, most patients with CDH eventually recover almost normal lung function. Pulmonary function tests generally demonstrate normal to slightly reduced vital capacity, forced expiratory volume, and maximum voluntary ventilation. Residual volume and functional residual capacity may be slightly increased. Exercise tolerance tests are generally normal. Lung ventilation-perfusion scans demonstrate nearly equal lung volumes bilaterally, but blood flow to the ipsilateral affected side is almost universally diminished. Failure to thrive occurs commonly in CDH survivors, with about 30% of patients remaining below the fifth percentile for weight, despite optimization of caloric intake.I3*36, 56 Possible explanations for poor growth and development in these patients include increased caloric requirements caused by increased work of breathing, poor oral intake because of foregut dysmotility, and the high incidence of clinically significant GERD. Infants with CDH must be weighed weekly following discharge from the intensive care nursery. Nutritional counseling with optimization of caloric intake to ensure appropriate weight gain is critical. GERD occurs commonly in infants with CDH, with a reported incidence ranging from 12Y040to 62Y0.*~ Foregut dysmotility is thought to play an important etiologic role. Extrinsic pressure on the mediastinum and the developing esophagus by herniated viscera during fetal growth is thought to result in esophageal obstruction, dilation, and impaired esophageal motility, Other contributing factors include the perturbed anatomy of the gastroesophageal junction with a shortened intra-abdominal esophagus, disrupted angle of His, and complete or partial absence of the diaphragm. UGI typically demonstrates a dilated and

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ectatic esophagus. Many patients may be successfully managed nonoperatively with the use of positional therapy, H,-blockers, and prokinetic agents. Fundoplication is eventually required in approximately 10% to 15% of patients.29,30, *O Significant developmental delay may occur in CDH survivors, including varying degrees of cognitive disability, decreased extremity tone affecting motor skill development, and sensorineural hearing loss with associated slowed speech and verbal skill acquisitionjl The cause of developmental delay in infants with CDH is multifactorial and may include birth asphyxia and hypoxemia, hyperventilation and alkalinization therapy, the use of ototoxic medications, and complications of ECM0.4I The incidence of developmental delay is significantly increased in infants with CDH who require ECMO. Close outpatient follow-up with a neurodevelopmental specialist, aggressive physical therapy, and parental education are necessary to address and treat these problems. The musculoskeletal growth of the chest wall in CDH survivors is affected not only by the disease process but also by its treatment. Although pectus it poses primarily a cosexcavatum occurs in approximately 20% of metic problem. Surgical intervention is only rarely required. Mild to moderate thoracic scoliosis occurs in approximately 11%of patient^.^, 36 A few patients require braces, and even fewer eventually require surgery. Recurrent diaphragmatic hernia occurs most commonly with large diaphragmatic defects requiring synthetic patch repair, with an incidence ranging from 5%% to 22%.% Infants typically present with feeding difficulties, GERD, coughing, or wheezing. Diagnosis is made by chest radiograph. THORACOTOMY AND LUNG RESECTIONS

The most common indications for thoracotomy in the newborn infant include repair of EA/TEF and pulmonary resections for congenital cystic adenomatoid malformation, congenital lobar emphysema, and pulmonary sequestration. Most infants tolerate thoracotomy and lobectomy quite well, with subsequent growth and expansion of the remaining lung tissue. Concerns in the intensive care nursery graduate after thoracotomy include development of anterior chest wall deformities (i.e., pectus excavatum), scapular deformities, and scoliosis. Most of these abnormalities are of cosmetic importance only. Only rarely is surgical intervention required. Patients should be followed closely during their developmental years to detect associated skeletal deformities and intervene when necessary, however. SURVIVORS OF EXTRACORPOREALMEMBRANE OXYGENATION

The use of ECMO in the critically ill neonate has been associated with significant neurodevelopment sequelae. Indeed, the major causes of death in ECMO patients are intracranial hemorrhage and infarction. The major factors contributing to central nervous system complications associated with ECMO include the need for prolonged anticoagulation, microthrombi from the ECMO circuit, ligation of the common carotid artery, alteration in pulsatile flow, and exposure to plasticizers of the ECMO circuit.20, 33, The incidence of significant neurologic abnormalities in ECMO survivors ranges from 10% to 45% and includes cerebral palsy, sensorineural hearing loss, cognitive disability, seizure disorder, vision loss, and speech delay.4I The long-term sequelae of common

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carotid artery ligation remain to be elucidated. Because of these potential complications, ECMO survivors require extremely close long-term follow-up by neurodevelopmental specialists to promptly identify neurodevelopmental problems and intervene appropriately. Serial visual, auditory, and developmental evaluations are critical. SUMMARY

Care of the intensive care nursery graduate may be quite challenging. It is important that primary care pediatricians become familiar with the complications unique to surgical patients so that they may properly prepare and educate parents and provide appropriate long-term follow-up for these often complex patients. Maintenance of a close relationship with the pediatric surgeon with an open line of communicationregarding the approach to various surgical problems facilitates the effective integration of the intensive care nursery graduate into the primary care pediatrician’s practice and provides the foundation for a successful clinical outcome. References 1. Adzick NS, Harrison MS, Click PL, et al: Diaphragmatic hernia in the fetus: Prenatal diagnosis and outcome in 94 cases. J Pediatr Surg 20357, 1985 2. Ball WS Jr, Kosloske AM, Jewel1PF, et a1 Balloon catheter dilatation of focal intestinal strictures following necrotizing enterocolitis. J Pediatr Surg 20:637, 1985 3. Ballance WA, Dahms BB, Shenka N, et a1 Pathology of neonatal necrotizing enterocolitis. Pediatrics 11786, 1990 4. Beasley SW Esophageal atresia and tracheoesophageal fistula. In Oldham KJ, Co5. 6. 7.

8.

lombani PM, Foglia RP (eds): Surgery of Infants and Children: Scientific Principles and Practice. Philadelphia, Lippincott-Raven Publishers, 1997, pp 1032-1033 Benjamin DR, Juul S, Siebert J R Congenital posterolateral diaphragmatic hernia: Associated malformations. J Pediatr Surg 23899-903, 1988 Benson CD, Lotti M W : The pouch technique in the surgical correction of cryptorchidism in infants and children. Surgery 62:967-973, 1967 Bill AH, Chapman ND. The enterocolitis of Hirschsprung’s disease: History and treatment. Am J Surg 10370, 1962 Boocock GR, Todd PJ: Inguinal hernias are common in preterm infants. Arch Dis Child 60669470,1985

8a. Bower TR, Pringle KC, Soper RT: Sodium depletion causing decreased weight gain and metabolic acidosis in infants with ileostomy. J Pediatr Surg 23:567-572, 1988 9. Carro D Postural treatment of children with a partial thoracic stomach. Arch Dis Child 35:569-580, 1960 10. Clatworthy HW, Thompson AG: Incarcerated and strangulated inguinal hernia in infants: A preventable risk. JAMA 154:123, 1954 11. Coombs RC, Buick RG, Gornall PG, et al: Intestinal malrotation: The role of small intestinal dysmotility in the cause of persistent symptoms. J Pediatr Surg 26553,1991 12. Cucchiana S, Staiano A, et al: Value of 24-hour intraesophageal pH monitoring in children. Gut 31:129-133, 1990 13. DAgostino JA, Bembaum JC, Gerdes M, et a1 Outcome for infants with congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation: The first year. J Pediatr Surg 3010-15, 1995 14. Dalt DA, Mazzoleni S, Montini G, et al: Diagnostic accuracy of monitoring in gastroesophageal reflux. Arch Dis Child W1421-1426, 1989 15. Devane SP, Smith W, Bisset WM, et a1 Persistent gastrointestinal symptoms after correction of malrotation. Arch Dis Child 67218, 1992

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16. Foglia Rp: Gastroesophageal reflux. In Oldham KT, Colombani PM, Foglia RP (eds): Surgery of Infants and Children: Scientific Principles and Practice. Philadelphia, Lippincott-Raven Publishers, 1997, pp 1036-1037, 1042 17. Fonkalsrud EW, Ellis DG, Shaw A, et a1 A combined hospital experience with fundoplication and gastric emptylng procedure for gastroesophageal reflux in children. J Am Coll Surg 180449,1995 18. Fujimoto T, Puri P: Persistence of enterocolitis following diversion of faecal stream in Hirschsprung's disease: A study of mucosal defense mechanism. Pediatr Surg Int 3141, 1988 19. Glick PL, Harrison MR, Aazick NS, et al: Gastric infarction secondary to small bowel obstruction: A preventable complication after Nissen fundoplication. J Pediatr Surg 22:941-943, 1987 19a. Glick PL, Irish MI, Holm BA: New insights into the pathophysiology of congenital diaphragmatic hernia. Clin Perinatol23:625-907, 1996 20. Graziani LJ, Streletz LJ, Mitchell DG: Electroencephalographic, neuroradiologic, and neurodevelopmental studies in infants with subclavian steal during extracorporeal membrane oxygenation. Am Pediatric Neurol 1097-103, 1994 21. Gross RE: Congenital hernia of the diaphragm. Am J Dis Child 71:579, 1946 22. Gross RE, Jewett TC Jr: Surgical experiences from 1222 operations for undescended testes. JAMA 160634-641, 1956 23. Grunow JE, Al-Hafidh AS, Tunell Wl? Gastroesophageal reflux following percutaneous endoscopic gastrostomy in children. Pediatr Surg 24:44, 1989 24. Hally KP, Atkinson JB, Wooley MM, et al: Necrotizing fasciitis: A serious sequela of omphalitis in the newborn. Ann Surg 199:101,1984 25. Harper RG, Garcia A, Sia C: Inguinal hernia: A common problem of premature infants weighing 1,000 grams or less at birth. Pediatrics 56112-115, 1975 26. Kaplan G W Iatrogenic cryptorchidism resulting from hernia repair. Surg Gynecol Obstet 142671,1976 27. Karrer FM, Lilly J R Biliary atresia. In Oldham KT, Colombani PM, Foglia RP (eds): Surgery of Infants and Children: Scientific Principles and Practice. Philadelphia, Lippincott-Raven Publishers, 1997, p 1398 28. Karrer FM, Lilly JR, Stewart BA, et a1 Biliarl atresia registry, 1976-1989. J Pediatr Surg 190:25, 1990 29. Kieffer J, Sapin E, Berg A, et a1 Gastroesophageal reflux after repair of congenital diaphragmatic hernia. J Pediatr Surg 30133S1333, 1995 30. Koot VCM, Bergmeijer JH, Bos AP, et al: Incidence and management of gastroesophageal reflux after repair of congenital diaphragmatic hernia. J Pediatr Surg 2848-52,1993 31. Kosloske AM, Bartow SA: Debridement of periumbilical necrotizing fasciitis: The importance of excision of the umbilical vessels and urachal remnant. J Pediatr Surg 26:808, 1991 32. Krieger NR, Shochat SJ, McGowan V, et a 1 Early hernia repair in the premature infant: Long-term follow-up. J Pediatr Surg 29:978, 1994 33. Kupsky WJ, Kinney HC, Lidov HGW, et al: Neuropathology of infants dying after extracorporeal membrane oxygenation. J Neuropath Exp Neurol48:307,1989 34. Laurent J, Gauthier F, Bernard 0, et a1 Long-term outcome after surgery for biliary atresia: A study of 40 patients surviving more than 10 years. Gastroenterology 99:1793, 1990 35. Lilly JR, Karrer FM, Hall RT, et a1 The surgery of biliary atresia. Ann Surg 210290,1989 36. Lund DP, Mitchell J, Kharasch V, et a1 Congenital diaphragmatic hernia: The hidden morbidity. J Pediatr Surg 29258-264, 1994 37. Meyers WF, Herbst JJ: Effectiveness of positional therapy for gastroesophageal reflux. Pediatrics 69:768-772, 1982 38. Misra D, Hewitt G, Potts SR, et al: Inguinal herniotomy in young infants, with emphasis on premature neonates. J Pediatr Surg 29:1496, 1994 39. Moore SW, Albertyn R, Cywes S: Clinical outcomes and long-term quality of life after surgical correction of Hirschsprung's disease. J Pediatr Surg 31:1496-1502, 1996 40. Nagaya M, Akatsuka H, Kato J: Gastroesophageal reflux occurring after repair of congenital diaphragmatic hernia. J Pediatr Surg 29:1447-1451, 1994

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Address reprint requests to Philip L. Glick, MD Children’s Hospital of Buffalo and State University of New York at Buffalo 219 Bryant Street Buffalo, NY 14222 e-mail: [email protected]