Gastroesophageal Reflux Flashcards

Question

What are the clinical presentations of GERD in children?

Answer 1

The presentation of GERD in infants and children is variable and depends on the patient’s age and overall medical condition. The surgeon must consider both this variability and the patient characteristics when evaluating a child with symptoms of possible GERD. Although the symptoms of GERD are variable for each patient, the actual frequency of symptoms seen in infants who have required surgical intervention for GERD has been reported. When considering the symptoms associated with GERD, persistent regurgitation is the most common complaint reported by parents of children with GERD. However, in infants, vomiting is often physiologic and can be “normal.” This type of vomiting is termed chalasia of infancy and is seen early in life, usually during burping, after feeding, or when placed in the recumbent position. Chalasia does not interfere with normal growth or development, and rarely leads to other complications. It is a self-limited process, with most infants transitioning to being asymptomatic by 2 years of age or near the time of initiating solid foods. No treatment is necessary in patients who have chalasia, and no diagnostic evaluation should be pursued. However, when persistent regurgitation is the result of GER, it can lead to complications, including significant malnutrition and growth failure due to insufficient caloric intake. In infants, another presenting symptom is irritability due to pain. Painful esophagitis can be the result of the reflux. Discomfort leads to crying despite consoling measures. Occasionally, small volumes of feeds briefly assist in alleviating pain. However, this is generally not a lasting effect. In contrast to infants, children with GERD more often present with complaints of pain. As with adults, the pain is retrosternal in nature and often described as heartburn. Long-standing GERD with esophagitis can lead to chronic inflammation or even ulcer formation with eventual scarring and stricture. Dysphagia develops as a result of a narrowed esophageal lumen, as well as possible esophageal dysmotility secondary to long-standing mucosal inflammation. Obstructive symptoms and pain are the two most common associated complaints when an esophageal stricture is present. Barrett esophagitis is a premalignant condition that is associated with prolonged GERD. It occurs when metaplasia develops in the esophageal squamous epithelium that is replaced with columnar epithelium. In adults, it is thought to be the result of chronic esophageal injury. Whether it develops from gastric acid injury or exposure to alkaline reflux is currently a controversial topic. Although uncommon in infants and children, when it does develop, serious complications often result. In addition to the increased risk for adenocarcinoma, approximately 50% of these patients will develop stricture and many patients will develop ulcers. Aggressive GERD management, along with vigilant long-term surveillance via yearly esophagogastroscopy, should be pursued to minimize these often difficult and possibly fatal complications. Respiratory symptoms are commonly seen in infants and children. Delineating the role of GER as an etiologic agent for ongoing respiratory complaints can be difficult because of the similarity of the symptoms that are seen with other pulmonary diseases and the fact that primary aspiration from oropharyngeal dysmotility, rather than GER, may be the inciting factor. Chronic cough, wheezing, choking, apnea, or near sudden infant death syndrome (SIDS) can all be symptoms attributable to GER. Recurrent bronchitis or pneumonia can occur from aspiration of the refluxate. Esophageal stimulation via acidification of the esophageal mucosa causes vagally mediated laryngospasm and bronchospasm, which clinically presents as apnea or choking, or mistakenly as asthma. Esophageal inflammation, as seen with esophagitis, likely enhances this mechanism. The effects of GER on premature infants with respiratory problems have been studied. Most of these infants were intubated for varying periods owing to respiratory distress syndrome or bronchopulmonary dysplasia. In the former group, GERD was responsible for deteriorating pulmonary status requiring intubation. In the latter, deterioration of pulmonary status plus failure to thrive and anorexia led to the diagnosis of GERD. All improved with correction of the GERD. Although uncommon, hemorrhage can be a presenting symptom of GERD. Esophagitis, gastritis, and ulcer formation can lead to hematochezia or melena in a small percentage of infants or children. H&A

Answer 2

Several recent articles have discussed management of GER in infants and children. These articles describe nonpharmacologic therapies for GER, which include parental reassurance, dietary modification, and positional adaptation. Reassurance, by showing compassion for the presumed impaired QOL for their infant, is important. Dietary modifications have been suggested to reduce regurgitation to a greater extent and faster than natural evolution. Thickening the formula to help reduce regurgitation has been approved as a management strategy by both the European and North American Societies for Pediatric Gastroenterology. Positional adaptation includes the prone position, the immediate right side position with later left side after feeding, and supine 40° anti-Trendelenburg (supine with head in the air). However, the prone position is no longer recommended owing to the increased risk of sudden infant death. H&A

Answer 3

As previously noted, the clinical history is an invaluable asset when evaluating for the presence of GERD and determining the need for antireflux therapy. The clinical history and symptoms are often sufficient to proceed with management. In many instances, diagnostic evaluation is reserved for patients requiring advanced therapy such as fundoplication or in cases in which the diagnosis is unclear. Medical management for GERD is centered primarily on pharmacologic therapies. Cisapride had been utilized up until 2000, when it was taken off the market by the American and European regulatory authorities owing to its adverse cardiac effects. Interestingly, in a 2010 Cochrane meta-analysis, there was no clear evidence identified that cisapride actually reduced symptoms of GERD. Similarly, metoclopramide and domperidone have also not been found to have efficacy. In one study, when compared with placebo, metoclopramide reduced symptoms in the reflux index, but it has a high incidence of adverse effects including lethargy, irritability, gynecomastia, and galactorrhea, and has caused permanent tardive dyskinesia. Domperidone has been found to have similar cardiac toxicity as cisapride. Currently, there are no effective prokinetic agents on the market. PPIs are considered the preferred option in children, as they are more effective than H2 receptor antagonists, but are not effective if the symptoms are not due to acid reflux. PPIs do not reduce the incidence of reflux episodes, as they only change the pH of the reflux from acidic to nonacidic or weakly acidic. At the same time, although there is lack of evidence of PPI effectiveness in neonates, many physicians continue to prescribe them. PPIs are more effective than H2 antagonists for acid reflux in children and adolescents. However, these drugs are overused in newborns, infants, and young children. If medical management has been ineffective and the complications of GERD are significant, then fundoplication is a very reasonable therapeutic option. In fact, based on the previous discussion, a fundoplication is also a good initial therapeutic option as medical management does not seem to be a good long-term solution and is largely ineffective, especially for young infants and those with nonacid reflux. H&A

Answer 4

If a preoperative study is performed, the most commonly utilized is the contrast upper gastrointestinal (GI) study. Having said that, while this study may be specific for documenting reflux, it is not helpful in determining the severity of the patient’s symptoms. Furthermore, it is not very sensitive, as the absence of reflux is a very poor indicator of GERD as the cause of the patient’s symptoms. In a study from our institution, we reviewed 843 patients from January 2000 to June 2007 who underwent fundoplication. An upper GI had been obtained in 656 of these patients, and a pH study was performed in 379 of the patients who had an upper GI. The sensitivity of the upper GI for reflux compared with the pH study was 30.8%. An abnormality on the upper GI study besides reflux that impacted the operative plan was found in 30 patients (4.5%) with the most common being malrotation. Malrotation was confirmed in 16 patients, but excluded in six of these patients at the time of fundoplication, and four patients had previously undergone a Ladd procedure. Therefore, the true incidence of the helpful finding of malrotation on the upper GI was 4.2%. In a similar study, there were only four unexpected findings in 572 cases in which an upper GI influenced the subsequent operation in patients undergoing either an antireflux procedure or gastrostomy placement. However, a survey was conducted of pediatric surgeons working in Child Health Corporation of America (CHCA) hospitals. Three hundred and thirty-seven pediatric surgeons were contacted and 121 replied. Eighty percent indicated they required a preoperative upper GI study before an antireflux procedure. Prior to 2010, 24-hour pH probe monitoring had been considered the gold standard for diagnosing GERD over the previous 30 years, when DeMeester and colleagues had established scores that related to the presence or absence of GERD. Boix-Ochoa and colleagues later proposed a revised score that was applicable to pediatric patients aged 2 months to 3 years old and is still used today by some investigators. However, as previously mentioned, data from pH probe monitoring has taught us that not all reflux is acidic. Therefore, combined multichannel intraluminal impedance (MMI) and pH measurements is now the gold standard test for evaluating GERD in children. MMI is sensitive for evaluating GERD and is particularly good at detecting nonacid reflux episodes. MMI detects reflux episodes based on changes in electrical resistance to flow from an electrical current between two electrodes in a probe when a liquid or a gas bolus moves between them. MMI also distinguishes swallows (antegrade flow) from retrograde GER. It can also accurately detect the height of the refluxate while determining whether the refluxate is liquid, gas, or mixed. In one study, the authors found that 78% of GERD episodes that were temporarily associated with breathing irregularities were nonacidic (pH > 4). In another study, the authors found that respiratory symptoms occurred more frequently when GER was nonacidic (pH > 4). There are six channels on the probes, and correct positioning of the probe is very important. The height reached by the refluxate is considered to be localized to the distal esophagus if it is confined to the two most distal impedance channels (channels 5 and 6). The refluxate is considered to be proximal if it reaches either or both of the most proximal channels (1 or 2). Impedance can also be a useful tool to evaluate the effect of reflux therapies including fundoplication. MMI has shown that GERD patients more commonly have liquid type reflux events, whereas non-GERD patients generally have more gas-type reflux events. Also, MMI data confirm that treatment with PPIs does not increase the amount of reflux, but rather converts the reflux to nonacid or weakly acidic in nature. Endoscopic evaluation with biopsy is probably the most sensitive method for diagnosing GERD, but is also more invasive than other tests. Currently, the North American Society of Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHN) has developed guidelines for endoscopy, and these guidelines state that endoscopy is indicated in infants and children with GERD who fail to respond to pharmacologic therapy or is part of the initial management of symptoms for weight loss, unexplained anemia or fetal occult blood, recurrent pneumonia, or hematemesis. Also, in an adolescent in whom Barrett esophagus is suspected, mucosal biopsy should be performed to stage the severity of the esophagitis or to histologically exclude dysplasia or malignancy. Previously, pyloroplasty was often performed at the time of fundoplication to help improve gastric emptying. Gastric emptying is best evaluated using radionuclide scanning via technetium-99m-labeled meal. When documented preoperatively, a number of studies have shown that gastroparesis does not significantly improve when an emptying procedure is performed at the time of the antireflux procedure. One study evaluating patients with delayed emptying undergoing fundoplication showed significantly improved gastric emptying for both solids and liquids after fundoplication alone. Neurologically impaired (NI) children with GERD have been shown to have delayed emptying more often than neurologically normal children. Conflicting data regarding the benefit and complication rates for these patients undergoing emptying procedures at the time of their fundoplications have been reported as well. Currently, evaluation for delayed gastric emptying is not recommended prior to an initial fundoplication unless a second operative intervention would place the patient at significant morbidity or mortality. At the same time, evaluation for delayed gastric emptying may be prudent in patients undergoing a second or especially a third fundoplication, as delayed emptying may be part of the cause for failure of the fundoplication and/or transmigration of the fundoplication wrap. At our institution, over the last 5 years, we have been moving away from preoperative imaging studies unless the information gained is helpful in planning or managing the patient’s operative care. Therefore, we find that the history and symptom complex is most important when evaluating children who are being referred for fundoplication. One scenario in which an impedance study may be helpful is the NI child < 6 months old who is referred for gastrostomy and has been placed on PPI therapy. In this situation, it is often unclear if the patient has significant reflux or not, so an impedance study may be helpful in determining if a fundoplication needs to be performed at the time of the gastrostomy. Similarly, esophagogastrostomy and manometry are rarely employed and used only when circumstances suggest that the information that will be provided will dictate changes in the operative management. One example may be a patient with symptoms of GERD, but a normal MMI-pH study. When esophagitis or other complications of GERD are found in esophagogastrostomy, fundoplication is usually recommended. As a general statement, as opposed to 10 years ago, most patients in our hospital are now managed based on their clinical symptoms rather than radiographic investigations, both for medical management as well as operative therapy. Preoperative testing is usually reserved for patients with a confusing history and symptom complex. H&A

Answer 5

Operative management usually follows failed medical management for growth failure (failure to thrive or gain weight appropriately), most respiratory symptoms, and other symptoms such as pain and esophagitis. However, in some circumstances, it may be best to proceed with fundoplication without a trial of medical therapy. These select situations include the previously mentioned patient in an intensive care unit with underlying respiratory disease who requires gastrostomy and possibly the NI patient with a similar need for gastrostomy and concern for aspiration. This latter scenario is commonly seen in infants and children, and the decision for or against fundoplication at the time of gastrostomy should be individualized. For example, in a 2 or 3-year-old (or older) NI patient who begins to have difficulty with oral intake and requires tube feedings but has no reflux symptoms, gastrostomy alone without fundoplication is very reasonable. On the other hand, a NI infant who cannot swallow and requires tube feedings in the intensive care unit due to respiratory disease probably should have a fundoplication in addition to a gastrostomy. We have found that a trial of nasogastric feeds can be helpful in determining which NI patient, who needs a gastrostomy due to poor oral intake, would also benefit from a fundoplication. Another scenario is the infant who presents with an ALTE spell and GER is documented but no other etiology is identified. This patient may be best served with a fundoplication as the initial therapy. In a review from our institution involving 81 infants presenting with ALTE, symptoms resolved with fundoplication in 78. The median followup in this study was 1738 days. Two patients required a second fundoplication when their symptoms recurred, and one needed a pyloromyotomy. Interestingly, 96.3% of these patients had been treated with antireflux medication and 87.7% were taking antireflux medications at the time of their ALTE. Therefore, medical management may not be effective in this population. Barrett esophagitis and esophageal stricture are the two other conditions in which initial operative therapy is recommended. The changes of Barrett esophagus will usually resolve in adolescents after fundoplication, although lifelong endoscopic surveillance is still needed. Regarding a stricture, esophageal dilation can be performed at the time of fundoplication. Subsequent dilations may be needed in severe cases. Finally, children with a known hiatal hernia and symptomatic GER are not likely to respond to medical management. Initial fundoplication is a reasonable choice in these patients. H&A

Answer 6

LAPAROSCOPIC NISSEN FUNDOPLICATION The patient is placed at the end of the operating table so that the surgeon can stand at the foot of the bed and the assistant to his or her right. The scrub nurse stands to the surgeon’s left. For infants, the legs should be placed in a frog-leg position. For older children, the lithotomy position can be used with stirrups. NI children may have contractures that preclude the lithotomy position, and careful consideration should be given to ensure they have appropriate padding of their pressure points. Although a single monitor placed over the patient’s head is usually sufficient, two monitors, placed to the right and the left of the patient’s head, can be used as well. An orogastric tube is introduced by the anesthesiologist to decompress the stomach. The bladder is usually emptied using a Credé maneuver. After prepping and draping, a 5-mm vertical incision is made in the center of the umbilicus and carried down through the umbilical fascia. A Step sheath (Medtronic, Minneapolis, MN) is gently introduced into the abdominal cavity, followed by introduction of a cannula with a blunttipped trocar through the sheath. By using this open technique, injury to the underlying viscera should be minimized. The sheath can be secured to the umbilical skin for stabilization should the surgeon desire. A pneumoperitoneum is created to a pressure of 12–15 mmHg, and diagnostic laparoscopy is performed with a 5-mm, 45° angled telescope. Four stab incisions are then placed in infants, and three stab incisions and a 5-mm port for the ultrasonic scalpel are utilized in children older than 5 years of age. A liver retractor is introduced through the lateral right incision. The two main working sites are the instruments positioned on either side of the midline. The assistant’s instrument is in the patient’s left lateral abdomen. We have standardized our technique and have utilized it for many years. Initially, the superior short gastric vessels are ligated and divided. Electrocautery connected to a Maryland dissecting instrument is used in the younger patients. As previously mentioned, the 5-mm ultrasonic scalpel or 3-mm sealer (JustRight Surgical, LLC. Boulder, CO) can be used in older children. The retroesophageal window is initially made from the patient’s left side because it is easy to accomplish after ligation/division of the short gastric vessels. We do not mobilize the esophagus very much at all to help reduce postoperative transmigration of the fundoplication wrap. (This will be discussed later.) Once the left side of the patient’s gastroesophageal junction has been identified, the stomach is flipped to the patient’s left and attention is turned toward the right aspect of the esophagus and upper stomach. The gastrohepatic ligament is incised to expose the esophagus and stomach on the right side. Great care must be taken to always know the location of the left gastric artery. It is imperative that the fundoplication wrap is positioned above the left gastric artery rather than inferior to it. The opening in the retroesophageal window is then completed from the right side so that the fundus can be brought posteriorly for the Nissen fundoplication. Again, as little esophageal mobilization as possible is performed at the gastroesophageal junction. At this point, a single suture is sometimes needed posterior to the esophagus to close a small hiatal hernia that may have either been present initially or was created during the minimal dissection. This is usually accomplished with a 2-0 silk suture. Previously, we have placed esophagocrural sutures with 3-0 silk at the 8, 11, 1, and 4 o’clock positions to further obliterate the space between the esophagus and the crura to prevent transmigration of the fundoplication wrap. Now, after a recently completed prospective randomized trial, we have found these sutures are not necessary, so we no longer utilize them routinely. The bougie is then introduced. A table describing the appropriate bougie size for neonates weighing <15 kg has been developed, validated, and used for >15 years. The fundoplication is then performed using a standard Nissen technique. Usually, three 2-0 sutures are utilized to perform the fundoplication. The most superior suture also incorporates a small portion of the anterior esophagus to anchor the wrap around the intra-abdominal esophagus. The length of the fundoplication is measured. Usually a length of approximately 2 cm is desired. For older children, 2.5–3.0 cm may be appropriate. Bupivacaine is instilled in the incisions, and the umbilical fascia and skin are closed. Steri-strips are usually used to close the stab incisions. H&A

Answer 7

GASTROSTOMY If a gastrostomy is also needed, the stab incision or cannula site in the patient’s left mid-epigastric area is the one utilized for exteriorization of the gastrostomy button. If a fundoplication has not been performed, then the same site is used for locating the button. In either event, this site is marked before insufflation so as not to distort its location when the abdomen is distended with CO2 to keep the gastrostomy off of the costal margin. A silicone catheter is introduced by the anesthesiologist into the stomach, which is then insufflated with 30–60 mL of air to prevent incorporating the back wall of the stomach with the suture utilized to secure the stomach to the anterior abdominal wall. The anterior wall of the stomach is grasped with a locking grasper and brought toward the anterior abdominal wall. For laparoscopic gastrostomy; two 2-0 PDS sutures (Ethicon, Inc., Somerville, NJ) are placed through the anterior abdominal wall cephalad to the grasper, through the stomach, and out through the anterior abdominal wall inferior to the instrument that has been used to grasp the stomach. Next, a needle followed by a guide wire are introduced through the abdominal wall and stomach in the center of the square formed by the two PDS sutures. Dilators from a Cook Vascular Dilator Set (Cook, Inc., Bloomington, IN) are used to serially dilate the anterior abdominal wall and gastrotomy. In infants, a 16- French dilator is usually the largest needed. In older children, the 20- French dilator may be required. The gastrostomy button is then placed over the guide wire and into the stomach. Under visualization, the balloon on the Mic-Key (Ballard Medical Products, Draper, UT) gastrostomy button is inflated. Attention must be paid to be sure that the button is, in fact, in the stomach and not external to the stomach. This can also be confirmed with the angled telescope by looking around each side of the stomach with the button in place. The PDS sutures are then secured over the button to prevent its dislodgement. Our protocol is to cut these sutures in 5 days. Others may cut them sooner. This technique was initially described by Georgeson and Owings, and details about complications have been published. H&A

Answer 8

Postoperatively, if a gastrostomy button was placed with the fundoplication (or if a button was placed primarily), feedings are usually started several hours later and advanced over the evening and the next morning. Most (90%) patients are ready for discharge the day after the operation. The parents will have been instructed on the use of the gastrostomy during the patient’s overnight hospitalization and can advance the feedings as needed. The patient can also be seen in the clinic should further questions or issues arise. If the patient did not need a gastrostomy, then liquids are allowed several hours after the procedure. It is very important to mention to the family that there is edema around the fundoplication after the operation. Therefore, for the first 3 weeks, especially in older children, the diet should be a mechanical soft diet that has the consistency of pudding, apple sauce, mashed potatoes, and so on. Essentially, meats and pizza should not be allowed because these food substances can become lodged above the fundoplication wrap. After 3 weeks, the edema usually resolves and small portions of meats and pizza can be added to the diet. Patients are seen 2 weeks, 3 months, 6 months, and 1 year after the operation. An upper gastrointestinal contrast study is performed at 1 year to evaluate for transmigration of the wrap or any other abnormalities. H&A

Answer 9

Our group has been interested in the efficacy of laparoscopic fundoplication for the past 20 years. A number of articles have been published from our institution detailing our thoughts about indications, complications, the operative technique, and ways to improve our results. Also, there have been many articles from other centers published in the last 10 years looking at long-term outcomes. In early 2002, in looking at our outcomes from January 2000 through March 2002, we believed that the need for repeat fundoplication was higher than desired. 121 In 130 patients undergoing laparoscopic Nissen fundoplication during that time, the incidence of redo fundoplication was 12%. All patients who required a redo operation had transmigration of the fundoplication wrap. During that time period, the esophagus was being extensively mobilized to try to create at least a 2-cm length of intra-abdominal esophagus. Moreover, there was no attempt to obliterate the space between the esophagus and the crura. These principles derived from prior training as well as literature reports in adults. Although the operations proceeded nicely and no conversions were needed, this 12% incidence of redo procedures seemed high. However, historical reports for the open operation have also documented a relatively high incidence of repeat fundoplications from 6–12%. In an attempt to reduce the incidence of postoperative transmigration of the wrap, two modifications were made in our operative technique beginning in April 2002. First, there was minimal mobilization of the esophagus. It was believed that the main reason for wrap transmigration was that the esophagus was being mobilized and a space was being created between the esophagus and crura to allow for the transmigration to occur. Therefore, the phrenoesophageal membrane was kept intact to obliterate this space. Second, to further obliterate this potential space, sutures were placed between the esophagus and crura. Initially, only two sutures were used, but eventually four sutures were placed for the purpose of further obliterating this space. No other modifications in the operative technique were made. In looking at the results from April 2002 through December 2004, the incidence of transmigration was reduced to 5%. This was actually reduced even further when looking at the patients in whom four esophageal crural sutures were utilized rather than two or three. In 2005, conversations with Georgeson and colleagues at the University of Alabama–Birmingham prompted a prospective, randomized trial looking at the operative technique. It was believed that the efficacy of esophageal mobilization should be evaluated. The primary endpoint was transmigration of the wrap. A power analysis based on the difference between the 12% and 5% repeat fundoplication rate previously mentioned was made, and the study was powered at 360 patients. The patients were randomized on the day of the surgery. One group was randomized to receive minimal esophageal mobilization with placement of four esophagocrural sutures. The other group was randomized to extensive esophageal mobilization to create a 2-cm length of intra-abdominal esophagus along with the four esophagocrural sutures. Patients were also randomized according to neurologic status. In addition, all patients received an upper GI contrast study at 1-year postoperatively to evaluate for transmigration of the fundoplication wrap. The study was stopped early after 177 patients had been entered because the findings overwhelmingly favored minimal esophageal mobilization, with an 8% transmigration rate in the minimal dissection group compared to a 30% rate in the extensive dissection group (P = 0.002). Neurologic status did not impact these outcomes. Furthermore, reoperation rates were higher in the extensive dissection group compared to the minimal dissection group (18% vs 3%, P = 0.006). From this study, it is clear that minimal dissection in the pediatric patient without a hiatal hernia is important to prevent postoperative transmigration of the fundoplication wrap. In a follow-up to the aforementioned study, our group followed patients from our center who participated in the previously discussed two-center prospective randomized trial. 20 Of the 177 patients mentioned previously in the two institutional trial, we attempted to contact the 122 patients who enrolled from our institution (61 with extensive esophageal dissection/mobilization and 61 with minimal esophageal dissection/mobilization). Fourteen of the patients were deceased at the time of our late follow-up, which was a median of 6.5 years from initial enrollment in the study (range 5.3–7.6 years). There was no significant difference in age, gender, neurologic impairment, mortality, or percentage of patients who were successfully contacted in each group for this late follow-up. In the patients from our center in the original randomized trial, postoperative wrap transmigration identified by an upper GI contrast study at 1 year was found in 22.7% of those with extensive mobilization/ dissection and 2.8% in those with minimal mobilization/ dissection. At the time of our publication of the original study, a total of 12 patients from our institution undergoing extensive dissection/mobilization and two patients who underwent the minimal dissection/mobilization required reoperation for an incidence of 19.7% and 3.3%, respectively (P = 0.01). Since that time, one additional patient in each group has required reoperation. Interestingly, the incidence of dysphagia requiring esophageal dilations in our group of patients was 12.5%. However, most of these were after the redo operation, not as a result of the primary fundoplication. Only two patients required dilation after the primary fundoplication, and they were in the extensive dissection/mobilization group. In 2017, we reported our final prospective randomized trial on modification of our operative technique. This was a single-institution study comparing minimal to extensive esophageal dissection/mobilization to identify whether or not the four esophagocrural sutures utilized in the previous study were needed. There was minimal esophageal dissection in all patients. One group underwent the minimal esophageal dissection and fundoplication while the other group underwent minimal esophageal dissection with the four esophagocrural sutures and fundoplication. Exclusion criteria included a preoperative hiatal hernia that was identified in one patient at the time of operation. The primary outcome variable was postoperative wrap migration. A sample size of 120 was calculated using our previous data with a power of 0.82, as we were accounting for some attrition. Again, a contrast study was planned at 1 year postoperatively. The mean follow-up in this study was conducted at a minimum of 1.5 years postoperatively. One hundred and twenty patients were enrolled between February of 2010 and February of 2014. One patient was excluded with the finding of a hiatal hernia, and 13 did not survive, which left 53 patients in whom the esophageal sutures were utilized and 54 in whom they were not placed. There was no difference in preoperative demographics. The operating time was significantly longer in the group in whom the sutures were utilized by a mean of 20 minutes (P < 0.01). A contrast study was obtained at 1 year postoperatively in 62% of those in whom the sutures were used and 68% of those in whom the sutures were not used, and there were no wrap herniations in either cohort (P = 1.0) (Table 28.6). There has been one reoperation for wrap loosening in the nonsuture group, whereas there have been no reoperations in the group in whom sutures were used (P = 1.0). Reflux symptoms and medications were no different at 1 month, 1 year, and final follow-up. Based on this study, it is our feeling that placement of the esophagocrural sutures does not offer any advantages and increases operative time in patients requiring a laparoscopic fundoplication. Therefore, our group no longer utilizes these esophagocrural sutures and employs minimal esophageal dissection and mobilization in all patients to reduce the incidence of postoperative wrap transmigration. There have been a number of other recent reports looking at outcomes following fundoplication in children. There is one other prospective randomized trial in the last 5 years. In this study, the authors compared recurrence of GERD in children randomized to laparoscopic (LF) versus open Nissen fundoplication (OF). This was a two-center study based in Norway with the main outcome measure being recurrence of GERD, which was defined as GERD combined with a reflux index > 4 on pH monitoring and/or GER and/or herniated wrap on upper GI contrast study. Postoperative follow-up included 24-hour pH monitoring, upper GI contrast study, clinical examination at 6 months, and phone interviews after 1, 2, and 4 years. Eighty-seven children were randomized, with 44 undergoing LF and 43 undergoing OF. Median age was 4.7 years. Twenty-three patients in both groups were neurologically impaired. The median follow-up was 4.0 years (0.3–8.9). Significantly more patients undergoing LF (37%) experienced recurrence of GERD compared to those undergoing OF (7%). The authors concluded that children operated with LF have a higher recurrence rate of GERD than those operated with OF. In an earlier study looking at 30-day outcome in this same group of children, the authors noted that 48 of the patients (55%) sustained complications within 30 days of the operation. Twenty-four of the patients were readmitted to the hospital because of complications after discharge. Also, the duration of the operation was documented as 150 + 34 minutes for LF and 89 + 25 minutes for OF (P < 0.001). Furthermore, the median length of stay was 7.0 days. It was also noted that of the 88 patients, 75 of the patients came from one of two hospitals. In another study from the same group of authors, they looked at a prospective cohort of patients undergoing Nissen fundoplication between 2003 and 2009. 134 Forty-six of these patients were NI and 41 were not. In this cohort review, the hospital stay was longer for the NI children (9 days [4–57] vs non-NI: 4 days [2–16] P < 0.001). More than 90% of the parents in both groups reported that the fundoplication had improved the child’s overall condition. Recurrence of GERD was diagnosed in 12 NI and 7 nonNI patients (P = 0.31). With this review, the authors concluded that early complications, recurrence, and parental satisfaction after fundoplication did not differ between NI and non-NI patients. In another recent report, the authors evaluated the 4year results following an RCT comparing OF and LF in children. 135 In this review, recurrent GER was documented by upper GI contrast study and/or 24-hour pH study. Twenty patients had been randomized to OF and 19 to LF. The incidence of recurrent GERD was 12.5% in the OF group and 20% in the LF group (P = n.s.). However, only one patient in each group required a redo fundoplication. Interestingly, the nutritional status and QOL improved in both groups (P = n.s.). Also, LF was associated with a reduced incidence of retching (P = 0.01). In a monocentric retrospective study, chart review was performed on children who had undergone fundoplication between 2006 and 2013, One hundred and nineteen patients with a mean age of 4.8 years underwent fundoplication. At 6 months, 21 of these patients (17.6%) had required a second fundoplication, and 64 (53.8%) had been placed back on antireflux medications. The authors concluded that, although fundoplication has a role in the treatment of severe reflux disease in children, the majority of children needed to restart their antireflux medications within 6 months of surgery. In a recent report looking at parental satisfaction following fundoplication with a median follow-up of 7.3. years, it was found that a high percentage of parents reported improved gastrointestinal reflux-related symptoms and a high level of satisfaction following fundoplication. In a database review from 41 U.S. hospitals in the Pediatric Health Information System database, it was found that 8.2% of patients admitted to these hospitals underwent a fundoplication in the 9 years between January of 2002 and December of 2010. Interestingly, more than half of these patients undergoing fundoplication (52.7%) were 6 months of age or younger. Several recent studies have looked at the need for fundoplication after an initial gastrostomy alone. In one study of 684 patients undergoing gastrostomy alone, of which 124 were open, 282 laparoscopic, and 278 endoscopic (PEG technique), subsequent fundoplication was needed in 62 patients (9.1%) with the mean interval of fundoplication after gastrostomy of 20.7 months. Cerebral palsy and anoxic brain injury had the most significant correlation with the need for subsequent fundoplication and were independent predictors. Interestingly, the laparoscopic approach for gastrostomy had a negative correlation with the subsequent need for fundoplication. In a smaller study, in NI patients who underwent gastrostomy alone, a subsequent fundoplication was needed in 17% of the patients. In another study, the authors looked at the need for fundoplication in patients undergoing open gastrostomy (OG) versus percutaneous endoscopic gastrostomy (PEG). Sixty-nine children were evaluated (PEG = 56, OG = 13). A higher percentage of patients who underwent OG (54%) needed fundoplication compared to 27% following the PEG gastrostomy. In an interesting study, 26 NI patients who underwent laparoscopic gastrostomy were evaluated to see if their GER improved or worsened postoperatively. After evaluating these patients with pH-MMI monitoring, as well as gastric emptying studies, the authors found that the laparoscopic approach reduced GER in NI patients by improving gastric emptying. Finally, the use of the anterior hemifundoplication, as opposed to the Nissen fundoplication, has also been recently evaluated. The authors evaluated QOL from the parents of both neurologically healthy and neurologically delayed children after the anterior hemifundoplication. In both groups, the authors found a significant improvement in QOL for the children and their parents along with improvement in their key symptoms. In another paper from our institution looking at 39 children undergoing open fundoplication for hypoplastic left heart syndrome, it was noted that there was a high morbidity and mortality in this group of patients with a recommendation that routine fundoplication in this population should be performed only under prospective protocols, as there were 11 deaths (28%) in this group of patients, with most of the deaths occurring during the first and second stage of cardiac repair. H&A

Answer 10

Our group has spent considerable time and effort trying to prevent the need for a second operation. In addition, we have also described the use of biosynthetic mesh to enhance hiatal repair at the time of a redo Nissen fundoplication. A prospective randomized trial has described the efficacy of a biologic prosthesis to reduce recurrence following laparoscopic paraesophageal hernia repair in adults. In a 2016 report, we retrospectively reviewed all our patients undergoing a redo operation following an initial laparoscopic Nissen fundoplication. Some of these patients were included in the previously mentioned prospective randomized trials. Between 2000 and 2013, 796 patients underwent a laparoscopic antireflux operation. Eighty-two patients (10%) required a reoperation. The mean age at the initial fundoplication was 1.8 + 3.6 years. Interestingly, 15 of the 82 patients (18.3%) required more than one reoperation. Of the 102 reoperations performed, 68 were successfully managed laparoscopically, 3 required conversion to an open operation, and 31 were performed open from the outset. In comparing patients requiring more than one reoperation, there was no difference in age, weight, use of mesh, or time to subsequent reoperation compared with patients that required only one redo procedure. However, in looking at patients operated on before 2009 and after 2009, which correlates to the finding that minimal esophageal mobilization/dissection leads to a reduced need for another fundoplication operation, there was a marked difference in the number of patients requiring reoperation. Prior to 2009, 70 patients (13.7%) required at least one more operation, whereas only 12 patients (4.2%) required another operation after 2009. Also, the indications for reoperation changed before and after 2009, as 78.5% of the patients undergoing a redo operation prior to 2009 were due to wrap transmigration, whereas only 33.3% underwent a redo operation after 2009 due to wrap transmigration (P <0.01). Wrap disruption (50%) as the cause for the need for a redo fundoplication was more common after 2009 versus before 2009 (5.7%). Also, 13 patients required more than one reoperation prior to 2009, whereas only two patients required more than one reoperation after 2009. Finally, we found that if a patient requires a second operation, the risk of requiring another operation after that one is 18%. Bansal and Rothenberg has also recently reviewed his experience with 252 patients undergoing a laparoscopic redo fundoplication. Many of these patients had their initial operation at another hospital. Eighty-four of these patients had undergone previous open fundoplication, and 32 had undergone more than one previous fundoplication. Their reported results are excellent, with an average time to full feeds of 1.4 days and an average hospital stay of 1.6 days. In this report, the postoperative complication rate was 3.6% and the wrap failure rate was 6.2%, with the most common cause due to transmigration of the wrap. It remains unclear exactly as to the mechanism whereby a fundoplication, especially using the Nissen technique, relieves/improves gastroesophageal reflux. There is no doubt that there is a mechanical effect through a ball-valve phenomenon whereby liquids and food enters the stomach, but cannot reflux back into the esophagus due to the fundoplication. In addition, by performing a fundoplication, irregardless of the technique used, the angle of His is accentuated, and the accentuation angle of His is one of the main natural barriers to reflux. A recent interesting paper from colleagues in Japan looked at 13 NI patients between 8 months and 20 years of age. The authors performed impedance/pH studies before their laparoscopic Nissen fundoplication and 1 year after it. The authors found that the laparoscopic Nissen fundoplication prevented high volume reflux, mainly acid reflux episodes, from rising to the proximal esophagus and this protection is expected to improve the mucosal integrity up to the middle of the esophagus. There was a marked reduction in the number of reflux episodes and the number of proximal reflux episodes following the laparoscopic Nissen fundoplication. Finally, previous studies have shown that the Nissen fundoplication decreased the number and magnitude of the transient LES relaxations. Also, a previous study showed that the Nissen fundoplication disrupted the vagal input to the GE junction, which was associated with a reduction in transient LES relaxations, so that disruption of the efferent vagal input may be a mechanism by which the Nissen fundoplication also reduces/prevents gastroesophageal reflux. Laparoscopic fundoplication has evolved into the preferred technique for surgical management of GERD. However, it is only through critical evaluation of one’s experiences that advances are made in improving our results for our patients. There really is no doubt that patients have less discomfort and earlier discharge from the hospital after the laparoscopic operation. Moreover, there is a faster return to regular activities as well. However, the operative technique continues to need ongoing evaluation with proper data collection and critical analysis to improve the outcomes. H&A

Answer 11

E Gastro- oesophageal reflux is the passage of gastric contents into the oesophagus, not caused by noxious stimuli, and is an extremely common, usually self-limiting condition, which is not pathological. The peak incidence is around 4 months of age and resolves by 1–2 years of age in most patients. Gastrooesophageal reflux should be of concern only when associated with complications when it is termed gastro-oesophageal reflux disease. Early detection and treatment of gastro-oesophageal reflux in children may prevent associated problems. In infants gastro-oesophageal reflux most commonly manifests as regurgitation, vomiting and ‘spitting up’. These symptoms occur in up to 67% of otherwise healthy infants aged 4–5 months, declining to 21% by 6–7 months of age and to less than 5% by 12 months of age. In older children the incidence has been reported to be 1.8%–22% in those aged 3–18 years. Neurologically impaired children are affected more commonly than normal children; studies show gastro-oesophageal reflux in up to 75% of neurologically impaired children. The severity of the reflux has also been shown to be related to the severity of the neurological dysfunction. SPSE 1

Answer 12

C Pathophysiology Four components of gastric juice can damage the oesophageal epithelium. These are: ● hydrochloric acid ● pepsin ● bile salts ● pancreatic enzymes. In typical circumstances, however, hydrochloric acid and pepsin are the main factors, as the bile salts and pancreatic enzymes are inactivated at acidic pH. A small amount of acid/pepsin reflux is normal; however, in gastro-oesophageal reflux disease a combination of defects in the antireflux barrier and luminal clearance mechanisms allows the refluxate to be in direct contact with the oesophageal epithelium for a prolonged period and to damage the epithelium. There are three lines of defence against damage from acid reflux, the first being the antireflux barrier consisting of a lower oesophageal sphincter, the diaphragmatic pinchcock and the angle of His. This barrier serves to limit the frequency and volume of refluxed gastric contents. When this line of defence fails the second becomes more important, namely, gastric clearance together with salivary and oesophageal secretions to neutralise the acid. The third mechanism is tissue or oesophageal mucosal resistance. The antireflux barrier The lower oesophageal sphincter The lower oesophageal sphincter is a high- pressure zone near the gastrooesophageal junction, although there is no anatomical structure. oesophageal peristalsis normally begins at the pharynx and progresses down the oesophagus and produces, at an appropriate time, relaxation of the lower oesophageal sphincter to allow the food bolus to pass into the stomach. It is thought that this is controlled by afferent and efferent vagal pathways. It is vital that an adequate length of intra-abdominal oesophagus is present, as this is compressed when the intra-abdominal pressure increases and therefore acts as a valve preventing reflux from the stomach into the lower oesophagus. The greater the length of the intra-abdominal oesophagus the more effective the lower oesophageal sphincter becomes. The key to antireflux procedures is ensuring an adequate length of intraabdominal oesophagus (>2 cm). The pinchcock action The oesophagus passes through the diaphragm at the hiatus, a sling-shaped orifice in the right crus of the diaphragm. The crural diaphragm constitutes the external mechanism of the lower oesophageal sphincter. As the oesophagus passes through the hiatus it is surrounded by the phreno-oesophageal membrane. The insertion of this membrane marks the junction of the intrathoracic and intra-abdominal oesophagus. Angle of His The angle of His is the angle formed by the juncture of the oesophagus and the stomach. In children with a normal length of abdominal oesophagus, the angle is acute. This acute angle creates a double antireflux mechanism, so that when a patient attempts to vomit, the vomitus is forced into the fundus, increasing the already acute angle further, thereby reducing the amount able to escape in the oesophagus. If the angle is obtuse however, the oesophagus is turned into a funnel and the vomit is channelled into the oesophagus. Mucosal rosette In the presence of a normal angle of His, a convoluted fold of mucosa with a rosette-like configuration is seen at the gastro-oesophageal junction. When there is an increase in the intra-abdominal pressure the folds are squeezed together and it acts as a weak antireflux valve. SPSE 1

Answer 13

E Patients with abdominal wall defects (gastroschisis, omphalocele, prune belly syndrome) are at increased risk of developing gastro-oesophageal reflux, as are those with congenital diaphragmatic hernia. Sandifer’s syndrome is associated with gastro- oesophageal reflux and oesophagitis. This syndrome includes voluntary dystonic contortions of the head, neck and trunk. These movements have been shown to increase the peristalsis in the lower oesophagus. As mentioned, neurologically impaired children have an increased risk of developing gastro-oesophageal reflux and are more likely to suffer complications. Asthma and gastro-oesophageal reflux Asthma is a well-recognised disease and one of the most common illnesses in childhood, with an estimated 5 million children or more having asthma in the united States. Gastro-oesophageal reflux disease is also a common disease and the two diagnoses often present together, but with a frequency greater than one would expect by chance alone. Asthma is a disorder caused by chronic inflammation of the airways in which, in addition to increased airway mucus secretion and smooth muscle hyperactivity, a number of inflammatory cells including eosinophils, mast cells, macrophages and T-lymphocytes play a critical role. These different cell types secrete a wide variety of mediators. The natural history of asthma, irrespective of the age of onset is one in which chronic inflammation can result in airway remodelling, irreversible airflow obstruction and an accelerated decline in lung function. In short, the longer the history of asthma the greater the risk of irreversible lung damage. Asthma is known to promote gastro-oesophageal reflux by a number of mechanisms including cough-induced increase in intra-abdominal pressure, hyperinflation of the lungs leading to alteration in the angle of His, airway obstruction leading to negative intrathoracic pressure, and specific asthma medications that reduce the pressure of the lower oesophageal sphincter. Several theories have been proposed to explain the association between asthma and gastro-oesophageal reflux. These include the theory that acid reflux causes direct stimulation of the airways and promotes inflammation on the basis that the common embryological origin of the respiratory and gastrointestinal systems results in a shared innervation via the vagus nerve and thereby shared autonomic reflexes. Therefore, stimulation of receptors in the distal oesophagus can lead to a vagal reflex and bronchial constriction. There are numerous clinical clues suggestive of pulmonary symptoms being due to reflux, including asthma occurring after 3 years of age, symptoms of reflux preceding asthma, failure to exhibit an improvement in pulmonary function despite good compliance with standard asthma medications, and a family history negative for pulmonary disease and positive for gastro-oesophageal reflux–related disorders. Respiratory symptoms suggestive of asthma can arise from a number of different disorders including gastro-oesophageal reflux, which often results in symptoms that are consistent with asthma or pneumonia. Asthma management focuses on the regulation and control of chronic airway hyperactivity and inflammation. Those children who do not respond to standard asthma medication should be investigated for other causes of their pulmonary symptoms including gastro-oesophageal reflux. up to 75% of adults with asthma report symptoms of gastro-oesophageal reflux. In addition among those individuals with asthma, abnormal oesophageal pH studies were observed in 55%–83% of adults, and 50%–63% of children. mathew et al. (2004) noted that the severity of the asthma was directly related to the degree of reflux. Twenty-four-hour pH studies in 68 children demonstrated a reflux prevalence of: ● 0% in those with mild intermittent asthma ● 11% in those with mild persistent disease ● 23% in children with moderate persistent asthma ● 57% in those with severe persistent asthma. The reverse also seems to be true in that the relative risk of pulmonary disease is increased in individuals with gastro-oesophageal reflux. other studies have confirmed that children with gastro-oesophageal reflux are at increased risk of developing sinusitis, laryngitis, pneumonia and bronchiectasis. Oesophageal atresia and gastro-oesophageal reflux Gastro-oesophageal reflux after repair of oesophageal atresia is common, occurring in at least 50%. A previous report of nine children reported 100% gastro-oesophageal reflux in those with oesophageal atresia not associated with a tracheo-oesophageal fistula. The mechanism for this may be due to the mobilisation of the lower oesophagus leading to shortening of the intra-abdominal portion of the oesophagus and widening of the angle of His. It may also be related to a congenital defect in the motor function of the lower oesophagus. A study looking at the prevalence of gastro-oesophageal reflux in 40 adults who had undergone correction for oesophageal atresia more than 28 years previously, identified 34% having difficulty swallowing solid food, 18% reported heartburn and 21% retrosternal pain. Sixty-one per cent of the patients agreed to undergo an oesophagogastroduodenoscopy and biopsy, which identified Barrett’s oesophagus in one and oesophageal squamous cell carcinoma in another patient. They were unable to confirm that patients with oesophageal atresia should undergo routine screening. In 1993, a study looked at 80 patients with oesophageal atresia and tracheooesophageal fistula treated from January 1974 to December 1988. Thirty-four (55%) were identified as having gastro-oesophageal reflux. After an unsuccessful trial of medical therapy, 21 underwent a Nissen fundoplication. only eight patients had an uncomplicated postoperative course. Wrap disruption occurred in 33%, and 8 of 21 developed severe dysphagia, requiring prolonged feeding with gastrostomy, probably due to the inability of the dyskinetic oesophagus to overcome the increased resistance caused by the wrap. Three out of 21 (14%) died from complications related to antireflux surgery. Seventy-one per cent of the children who underwent a Nissen fundoplication eventually had excellent long-term results, but the authors did question whether a Nissen was appropriate in those with a previous history of oesophageal atresia considering the significant morbidity and mortality. SPSE 1

Answer 14

E Symptoms of gastro-oesophageal reflux Children with gastro-oesophageal reflux classically complain of abdominal pain. A key component in differentiating acid-related disorders in children with chronic or recurrent abdominal pain is the location, quality and timing of the pain. When taking a history from the patient, it is useful to have the child standing as they will often point to the site of pain. Pointing to the epigastrium or mesogastrium is suggestive of gastro-oesophageal reflux. Children may find it difficult to articulate the feeling of heartburn, which may be a reason for epigastric pain being a more common presenting feature. one study of 76 children found the presenting symptoms to be recurrent abdominal pain in 64%, heartburn in 34%, respiratory symptoms in 29%, regurgitation in 22%, retrosternal pain in 18% and vomiting in 16%. Review of a child’s medical history may be helpful in reinforcing the diagnosis of gastro-oesophageal reflux because recurrent ear infections, sore throats and dental problems are consistent with known extraoesophageal manifestations of gastro-oesophageal reflux . Symptoms of gastro-oesophageal reflux: ● vomiting ● rumination ● failure to thrive ● oesophageal manifestations ― anaemia, haematemesis ― chest pain, heartburn ― dysphagia ― stricture ● respiratory manifestations ― aspiration pneumonia ― laryngospasm, hoarseness ― reactive airway disease ― chronic cough ― choking ― apnoea ● neurological problems ― infantile irritability ― seizure-like events ― Sandifer’s syndrome ● other symptoms ― glue ear ― increased dental carries ― halitosis.

Answer 15

A Investigations Diagnostic procedures other than clinical evaluation should be used when the results will strongly influence treatment or will identify complications. For the infant with frequent regurgitation who is otherwise thriving, no investigations are necessary. Despite a number of diagnostic methods ranging from upper gastrointestinal series with barium or technetium scans to 24-hour oesophageal pH monitoring or endoscopy, there is no gold standard for the investigation of acid-related disease in children. Upper gastrointestinal barium study upper GI studies may detect anatomical abnormalities but are insensitive and non-specific for the diagnosis of gastro-oesophageal reflux and Helicobacter pylori-associated mucosal injury. Associated conditions such as hiatus hernia, pyloric obstruction and malrotation may be identified. An experienced radiologist may be able to evaluate the oesophagus for structural and mucosal irregularities, and may also be able to comment on oesophageal peristalsis. Scintigraphy Episodes of gastro-oesophageal reflux are demonstrated on scintigraphy through the presence of the isotope in the oesophagus, using serial images 30–60 minutes after the instillation of the isotope into the stomach. The sensitivity is higher than conventional barium studies and exposes the patient to lower doses of radiation. It has three clinical applications. 1 Diagnosis of gastro-oesophageal reflux. 2 Detection of pulmonary aspiration. 3 Assessment of the rate of gastric emptying. 24-hour oesophageal monitoring This technique was developed in the 1970s for use in adults but it was quickly adapted for use in children. A pH probe of appropriate size is positioned transnasally at the junction of the middle and lower thirds of the oesophagus. The pH is continuously measured and recorded. A pH of less than 4 denotes reflux of gastric contents. The frequency and duration of reflux episodes are recorded. A diary is also kept to document what the patient is doing at certain times (e.g. sleeping, feeding, coughing, and so forth). The test has been found to have a sensitivity and specificity of at least 94%. However, pH studies are unable to identify non-acidrelated or alkaline-reflux-related disease. The test is indicated in the following circumstances. 1 Infants who have respiratory symptoms. 2 Infants who are irritable, crying and poor feeders. 3 Children with reactive airway disease. 4 Children who are unresponsive to medical therapy and in whom the role of gastro-oesophageal reflux is uncertain. 5 Children who become symptomatic after fundoplication. Upper gastrointestinal endoscopy and biopsy Clinical suspicion of oesophagitis and dysphagia are indications for this procedure. An upper gastrointestinal endoscopy alone has a low sensitivity for oesophagitis, therefore biopsies are taken. Biopsy and microscopic diagnosis are both highly specific and sensitive in the diagnosis of oesophagitis (95%). oesophagoscopy can also identify Barrett’s, strictures and ulcers. Routine gastric biopsies are taken in children undergoing endoscopy to identify those with H. pylori. When testing for H. pylori, serological testing should be avoided as antibody responses in children differ from those of an adult. Antibody levels in children may remain elevated for years after the infection has been eradicated. Manometry oesophageal manometry studies the contractile activity of the oesophagus and the upper and lower sphincters. This investigation is important in research of gastro-oesophageal disease, but is of little value as a diagnostic tool. SPSE 1

Answer 16

C Difficulty in feeding and feed refusal is a common problem in neurologically impaired children and in those with gastro-oesophageal reflux. The most difficult clinical problem is managing the severely neurologically impaired child with persistent vomiting. Vomiting is common in this group with 15% of severely impaired institutionalised children having recurrent vomiting. Seventy-five per cent of these children have been shown to have gastro-oesophageal reflux, and the severity of the neurological dysfunction is directly related to the degree of gastro-oesophageal reflux. many of these children are unable to communicate verbally, making diagnosis difficult and these children are often non-ambulatory, confounding the problem of gastro-oesophageal reflux because of the lack of gravitational forces to aid stomach emptying. Increased abdominal pressure also probably plays a role, with an increased incidence of scoliosis, spastic quadriplegia and seizures all contributing to an increase in the intra-abdominal pressure leading to the normal antireflux barrier being overcome. Neurologically impaired children also have more associated complications of gastro-oesophageal reflux with oesophageal strictures and oesophagitis being more common as well as pneumonia. These children are also more likely to encounter postoperative complications following antireflux surgery. SPSE 1

Answer 17

B The placement of a gastrostomy increases the risk of gastro-oesophageal reflux due to opening of the angle of His, lowering of the lower oesophageal sphincter and a reduction in the lower oesophageal length. The development of post-gastrostomy gastro-oesophageal reflux has been shown to be as high as 66% in neurologically impaired children with normal preoperative studies. Lesser curvature gastrostomy tubes are associated with less gastrooesophageal reflux than greater curve tubes, because there is no change in the angle of His. It is not possible to place a percutaneous gastrostomy tube in the lesser curve of the stomach. SPSE 1

Answer 18

B Medical therapy The major treatment options for those with gastro-oesophageal reflux disease are medical, and antireflux surgery. Drugs that inhibit or neutralise gastric acid The principal clinical indications for reducing acid secretion are peptic ulceration and reflux oesophagitis. Therapy for peptic ulcers and reflux oesophagitis aims to decrease the release of gastric acid with H2 -receptor antagonists or proton pump inhibitors, and/or neutralise the acid with antacids. Proton pump inhibitors The first proton pump inhibitor was the substituted benzimidazole, omeprazole, which irreversibly inhibits H+ /K+ -ATPase (the proton pump), the terminal step in the acid secretory pathway. Both basal and stimulated gastric acid secretion is reduced. omeprazole is currently the only proton pump inhibitor licensed in the uK for the treatment of gastro-oesophageal reflux disease in children over 2 years of age. Histamine H2 -receptor antagonists The histamine H2 -receptor antagonists competitively inhibit histamine actions at all H2 -receptors, but their main clinical use is the inhibitors of gastric acid secretion. They can inhibit histamine, gastrin and acetylcholine-stimulated acid secretion; pepsin secretion also falls with the reduction in volume of gastric juice. Drugs include ranitidine, nizatidine, famotidine and cimetidine. Drugs that increase gastrointestinal motility Domperidone Primarily used as an antiemetic but it also increases gastrointestinal motility (mechanism unknown). Clinically it also increases the pressure at the lower oesophageal sphincter, thus inhibiting gastro- oesophageal reflux. It also increases gastric emptying and enhances duodenal peristalsis. It is therefore useful in disorders of gastric emptying and gastro-oesophageal reflux disease. A recent review, however, concluded that there was no robust evidence of efficacy of domperidone for the treatment of gastro-oesophageal reflux in young children. Metoclopramide metoclopramide is a D2 -receptor antagonist closely related to the phenothiazine group. It acts centrally on the chemoreceptor trigger zone and also has a peripheral action on the gastrointestinal tract itself, stimulating gastric motility and causing a marked acceleration in gastric emptying. It is useful in gastrooesophageal reflux disease and in disorders of gastric emptying. It is a similar drug to domperidone but has more tendency to cross the blood brain barrier and cause the unwanted central side effects due to its blockade of dopamine receptors, including disorders of movement, fatigue, motor restlessness, spasmodic torticollis and oculogyric crises. Cisapride Cisapride was used until 2000. Guidelines (1993) in ESPGAN (the European Society of Paediatric Gastroenterology Hepatology and Nutrition) cited cisapride as a first-line treatment for gastro-oesophageal reflux/gastro-oesophageal reflux disease. However, a more recent systematic Cochrane Review in 2000, and a large multicentre Canadian trial in 1999, have shown that there is little evidence of efficacy in its use. It has now been withdrawn after being found to have had no advantage over placebo for relief of symptoms of gastro-oesophageal reflux and also because of side effects including fatal cardiac arrhythmias. Cisapride stimulates acetylcholine release in the myenteric plexus in the upper gastrointestinal tract through 5-HT4 -receptor-mediated effect. This raises oesophageal sphincter pressure and increases gut motility. Histamine H1 -receptor antagonists Alimemazine/trimeprazine is a phenothiazine H1 -receptor antagonist commonly used for its antipruritic effect and in high dose for its sedative effect. It is also known to have antiemetic properties and antispasmodic effect on smooth muscle. It has been shown to decrease the frequency of retching after a Nissen fundoplication in neurologically impaired children. SPSE 1

Answer 19

B In the nissen fundoplication the following steps are performed. 1 The gastrohepatic ligament is divided with the peritoneal covering of the oesophagus. 2 The oesophagus is mobilised ensuring an adequate length. 3 The gastrophrenic ligaments are divided to mobilise the posterior aspect of the fundus of the stomach. 4 Division of the short gastric vessels is often required. 5 A window is then created posterior to the oesophagus. 6 The right and left leaves of the right crus of the diaphragm are approximated. 7 The fundus is passed from left to right posterior to the oesophagus. 8 A 360-degree wrap is created. 9 The left and right margins of the wrap are sutured anteriorly including the anterior wall of the oesophagus in the sutures. 10 The superior margin of the wrap is sutured to the hiatus. SPSE 1

Answer 20

E more than 90% of children have long-term resolution of their symptoms with Nissen’s fundoplication, with the cure rate for emesis approaching 100%. Pulmonary symptoms are improved in 96% of children and weight gain is improved in the majority, especially if a gastrostomy is positioned at the same time. Asthma is improved in 92% of patients. Neurologically impaired children have an increased risk of developing complications following antireflux surgery and neurological status is a major predictive factor in the success of antireflux surgery. Dedinsky et al. (1987)reported the results of 429 Nissen operations in children, 297 of whom were neurologically impaired. This group accounted for all four deaths, 24⁄28 wrap herniations. Spitz and Kirkane had a 20% morbidity and 12% mortality rate among children with neurological impairment undergoing Nissen fundoplication. SPSE 1

Answer 21

A Other antireflux procedures Thal and Boix–Ochoa In the Thal and Boix–ochoa techniques, the lower oesophagus is mobilised in a similar fashion to the Nissen procedure and the crus is approximated posterior to the oesophagus to re-establish a hiatus of a normal size. The stitch is also used to attach the hiatus to the posterior wall of the oesophagus. A partial 180-degree anterior fundal wrap is then constructed, plicating the upper fundus to the anterior oesophageal wall. This procedure does not require division of the short gastric vessels, is technically easier, and has a shorter operating time. The Boix–ochoa procedure differs from the Thal in that the remaining fundus is fixed by interrupted sutures to the underside of the left hemidiaphragm to maintain the angle of His. In both the Thal and the Boix–ochoa techniques there is a reduced incidence of gas bloat. Toupet’s technique Another partial-wrap technique is the Toupet procedure also developed to minimise gas bloat. In this operation a partial 270-degree wrap is positioned posterior to the oesophagus. After the crura have been approximated to restore the size of the hiatus, the gastric fundus is passed posterior to the oesophagus. Division of the short gastric vessels is often necessary. The posterior aspect of the wrap is sutured to the right crus. The margins of the wrap are then sutured to the left and right margins of the oesophagus, leaving the anterior wall of the oesophagus free. The Boerema is an anterior gastropexy, and the Hill is a posterior gastropexy. SPSE 1

Answer 22

E Reported complications following antireflux surgery include wrap breakdown, dysphagia secondary to a slipped wrap, a wrap that is too tight, torsion of the wrap and gas bloat. Early intestinal obstruction occurs in 2%–6%. Splenic injury and perforation of the oesophagus are rare. As mentioned, complications following Nissen fundoplication in neurologically impaired children are higher than in neurologically normal children. SPSE 1

Answer 23

E All of the above procedures are minimally invasive and are being used/evaluated at centres around the world. ● Laparoscopic nissen fundoplication is perhaps the most commonly performed minimally invasive therapy for gastro-oesophageal reflux. It has become the ‘gold standard’ against which other procedures are compared. ● The Stretta procedure (Curran medical Inc) uses endoluminal radiofrequency energy to cause collagen contraction at the level of the lower oesophageal sphincter. This modulates the physiology of the sphincter and so reduces gastro-oesophageal reflux. ● TIF with esophyX (EndoGastric Solutions) is transoral incisionless fundoplication. under endoscopic guidance a former is used to reduce any hiatus hernia and fold the fundus of the stomach. The fundus is then secured with endoscopically applied fasteners to form a partial wrap. ● endoCinch (Bard) is another endoscopically guided device that allows pleats to be stitched into the lower oesophagus below the sphincter. These pleats reduce symptoms and medication usage in mild gastrooesophageal reflux. ● other endoluminal therapies involve the injection of bulking materials submucosally into the lower oesophagus at the level of the sphincter to reduce gastro-oesophageal reflux. SPSE 1