Eric W. Fonkalsrud

Surgical Treatment of Gastroesophageal Reflux in Children: A Combined Hospital Study of 7467 Patients

Objective. To review retrospectively the combined clinical experience with the surgical treatment of persistently symptomatic gastroesophageal reflux (SGER) in childhood from seven large childrens surgery centers in the United States. Design. During the past 20 years, 7467 children <18 years of age underwent antireflux operations for SGER at the seven participating hospitals. Fifty-six percent were neurologically normal (NN) and 44% were neurologically impaired (NI). The most frequent diagnostic studies were upper gastrointestinal series (68%), esophageal pH monitoring (54%), gastric emptying study (32%), and esophagoscopy (25%). The age at operation was under 12 months in 40% and 1 to 10 years in 48%. The type of fundoplication was Nissen (64%), Thal (34%), and Toupet (1.5%). A gastric emptying procedure was performed on 11.5% of NN patients and 40% of NI patients. Laparoscopic fundoplication was performed on 2.6% of patients. Results. Good to excellent results were achieved in 95% of NN and 84.6% of NI patients. Major complications occurred in 4.2% of NN and 12.8% of NI patients. The most frequent complications were recurrent reflux attributable to wrap disruption (7.1%), respiratory (4.4%), gas bloat (3.6%), and intestinal obstruction (2.6%). Postoperative death occurred in 0.07% of NN and 0.8% of NI patients. Reoperation was performed in 3.6% of NN and 11.8% of NI patients. The results and complications were similar among the participating hospitals and did not seem related to the type of fundoplication used. Conclusion. The excellent results (94% cure) and low morbidity with gastroesophageal fundoplication with or without a gastric emptying procedure from a large combined hospital study indicate that operation should be used early for SGER in NN children and to facilitate enteral feedings and care in NI children.

Effects of chronic corticosteroids and vitamin a on the healing of intestinal anastomoses

The ability of vitamin A to reverse the inhibitory effects of chronic corticosteroids on cutaneous and fascial wound healing is well established. To investigate this in the unique low-collagen environment of the intestinal anastomosis, 35 rabbits received twice-daily injections of either saline (control), dexamethasone (0.1 mg/kg/day), dexamethasone plus low-dose vitamin A (1,000 IU/kg/day), or dexamethasone plus high-dose vitamin A (10,000 IU/kg/day) for a 2-week period. Animals then underwent creation of single-layer, inverting small and large intestine anastomoses. All injections were continued postoperatively. A fifth group received only dexamethasone preoperatively and dexamethasone plus high-dose vitamin A postoperatively. On postoperative day 7, animals underwent in situ assessment of anastomotic bursting pressure and subsequent histologic examination using a modified Ehrlich/Hunt scale. Corticosteroids significantly impaired the healing of small and large intestine anastomoses, with decreased bursting pressures and histologic parameters at 1 week. Only high-dose vitamin A significantly reversed this inhibitory effect, whether given preoperatively or only postoperatively.

Repair of pectus excavatum deformities : 30 years of experience with 375 patients

OBJECTIVE To review the surgical experience with pectus excavatum chest deformities at UCLA Medical Center during a 30-year period. BACKGROUND Pectus excavatum is a relatively common malformation that is often symptomatic; however, childrens physicians often do not refer patients for surgical correction. METHODS Hospital records from 375 patients who underwent repair of pectus excavatum deformities between 1969 and 1999 were reviewed. Decrease in stamina and endurance during exercise was reported by 67%; 32% had frequent respiratory infections, 8% had chest pain, and 7% had asthma. The mean pectus severity score (width of chest divided by distance between posterior surface of sternum and anterior surface of spine) was 4.65 (normal chest = 2.56). All patients had marked cardiac deviation into the left chest. Repair was performed with subperiosteal resection of the abnormal cartilages, transverse wedge osteotomy of the anterior sternum, and internal support with a steel strut for 6 months. Repair was performed on 177 children before age 11 years; 38 adults with severe symptoms underwent repair. RESULTS The mean hospital stay was 3.1 days. With a mean follow-up of 12.6 years, all patients with preoperative respiratory symptoms, exercise limitation, and chest pain experienced improvement. Vital capacity increased 11% (mean) within 9 months in 35 patients evaluated. There were no deaths. Complications included hypertrophic scar formation (35), atelectasis (12), pleural effusion (13), recurrent sternal depression (5), and pericarditis (3). More than 97% had a very good or excellent result. CONCLUSION Pectus excavatum deformities can be repaired with a low rate of complications, a short hospital stay, and excellent long-term physiologic and cosmetic results.

Current Management of Pectus Excavatum

Pectus excavatum (PE) is one of the most common anomalies of childhood. It occurs in approximately 1 in every 400 births, with males afflicted 5 times more often than females. PE is usually recognized in infancy, becomes much more severe during adolescent growth years, and remains constant throughout adult life. Symptoms are infrequent during early childhood, but become increasingly severe during adolescent years with easy fatigability, dyspnea with mild exertion, decreased endurance, pain in the anterior chest, and tachycardia. The heart is deviated into the left chest to varying degrees causing reduction in stroke volume and cardiac output. Pulmonary expansion is confined, causing a restrictive defect. Repair is recommended for patients who are symptomatic and who have a markedly elevated pectus severity index as determined by chest X-ray or computed tomography scan. Repair using the highly modified Ravitch technique is usually performed after the age of 8 years. The optimal age for repair is between 12 and 16 years. Repair can be performed on adults with similar good results. Recent modifications in the Ravitch technique remove minimal cartilage and routinely use a temporary internal support bar for 6 months. Operation rarely takes more than 3 hours, and hospitalization rarely exceeds 3 days. Pain is mild and complications are rare, with 97% of patients experiencing a good to excellent result. The new minimally invasive Nuss repair avoids cartilage resection and takes less operating time, but is associated with more severe pain, longer hospitalization and a higher complication rate, with the bar remaining for 2 or more years. This technique is less applicable to older patients and those with asymmetric deformities. Long-term follow-up will be necessary to determine which operation may be best for any specific patient.

Gastroesophageal reflux in childhood

Gastroesophageal reflux (GER) is one of the most frequent symptomatic clinical disorders affecting the gastrointestinal tract of infants and children. During the past 2 decades, GER has been recognized more frequently because of an increased awareness of the condition and also because of the more sophisticated diagnostic techniques that have been developed for both identifying and quantifying the disorder. Gastroesophageal fundoplication is currently one of the three most common major operations performed on infants and children by pediatric surgeons in the United States. Normal gastroesophageal function is a complex mechanism that depends on effective esophageal motility, timely relaxation and contractility of the lower esophageal sphincter, the mean intraluminal pressure in the stomach, the effectiveness of contractility in emptying of the stomach, and the ease of gastric outflow. More than one of these factors are often abnormal in the same child with symptomatic GER. In addition, in patients with GER disease, and particularly in those patients with neurologic disorders, there appears to be a high prevalence of autonomic neuropathy in which esophagogastric transit and gastric emptying are frequently delayed, producing a somewhat complex foregut motility disorder. GER has a different course and prognosis depending on the age of onset. The incompetent lower esophageal sphincter mechanism present in most newborn infants combined with the increased intraabdominal pressure from crying or straining commonly becomes much less frequent as a cause of vomiting after the age of 4 months. Chalasia and rumination of infancy are self-limited and should be carefully separated from symptomatic GER, which requires treatment. The most frequent complications of recurrent GER in childhood are failure to thrive as a result of caloric deprivation and recurrent bronchitis or pneumonia caused by repeated pulmonary aspiration of gastric fluid. Children with GER disease commonly have more refluxing episodes when in the supine position, particularly during sleep. The reflux of acid into the mid or upper esophagus may stimulate vagal reflexes and produce reflex laryngospasm, bronchospasm, or both, which may accentuate the symptoms of asthma. Reflux may also be a cause of obstructive apnea in infants and possibly a cause of recurrent stridor, acute hypoxia, and even the sudden infant death syndrome. Premature infants with respiratory distress syndrome have a high incidence of GER. Esophagitis and severe dental carries are common manifestations of GER in childhood. Barretts columnar mucosal changes in the lower esophagus are not infrequent in adolescent children with chronic GER, particularly when Heliobacter pylori is present in the gastric mucosa. Associated disorders include esophageal dysmotility, which has been recognized in approximately one third of children with severe GER. Symptomatic GER is estimated to occur in 30% to 80% of infants who have undergone repair of esophageal atresia malformations. Neurologically impaired children are at high risk for having symptomatic GER, particularly if nasogastric or gastrostomy feedings are necessary. Delayed gastric emptying (DGE) has been documented with increasing frequency in infants and children who have symptoms of GER, particularly those with neurologic disorders. DGE may also be a cause of gas bloat, gagging, and breakdown or slippage of a well-constructed gastroesophageal fundoplication. The most helpful test for diagnosing and quantifying GER in childhood is the 24-hour esophageal pH monitoring study. Miniaturized probes that are small enough to use easily in the newborn infant are available. This study is 100% accurate in diagnosing reflux when the esophageal pH is less than 4.0 for more than 5% of the total monitored time.

Proficiency of Surgeons in Inguinal Hernia Repair Effect of Experience and Age

Objectives:We examined the influence of surgeon age and other factors on proficiency in laparoscopic or open hernia repair. Summary Background Data:In a multicenter, randomized trial comparing open and laparoscopic herniorrhaphies, conducted in Veterans Administration hospitals (CSP 456), we reported significant differences in recurrence rates (RR) for the laparoscopic procedure as a result of surgeons’ experience. We have also reported significant differences in RR for the open procedure related to resident postgraduate year (PGY) level. Methods:We analyzed data from unilateral laparoscopic and open herniorrhaphies from CSP 456 (n = 1629). Surgeons experience (experienced ≥250 procedures; inexperienced <250), surgeons age, median PGY level of the participating resident, operation time, and hospital observed-to-expected (O/E) ratios for mortality were potential independent predictors of RR. Results:Age was dichotomized into older (≥45 years) and younger (<45 years). Surgeons inexperience and older age were significant predictors of recurrence in laparoscopic herniorrhaphy. The odds of recurrence for an inexperienced surgeon aged 45 years or older was 1.72 times that of a younger inexperienced surgeon. For open repairs, although surgeons age and operation time appeared to be related to recurrence, only median PGY level of <3 was a significant independent predictor. Conclusion:This analysis demonstrates that surgeons age of 45 years and older, when combined with inexperience in laparoscopic inguinal herniorrhaphies, increases risk of recurrence. For open repairs, only a median PGY level of <3 was a significant risk factor.

Trophic effect of amniotic fluid on fetal gastrointestinal development

To determine if amniotic fluid or its constituent trophic factors influence fetal gastrointestinal tract development we developed models of fetal esophageal ligation to prevent swallowing of amniotic fluid and fetal esophageal cannulation with infusion of various substances to mimic fetal swallowing. A total of 43 fetuses was studied. Esophageal ligation resulted in a 32% reduction in gastric weight and a 40% reduction in serum gastrin level, compared to unoperated controls, whereas intestinal and liver weights were unchanged. Gastric acid concentration averaged 43.4 +/- 7.7 mumole/ml in control fetuses, but only 0.5 +/- 0.5 mumole/ml following esophageal ligation. Infusion of Ringers lactate solution intragastrically did not prevent the changes in gut development seen after esophageal ligation. In contrast, infusion of bovine amniotic fluid resulted in relatively normal gut development, with a gastric acid concentration of 28.5 +/- 6.9 mumole/ml and liver and gastric weights and serum gastrin levels no different from control. Epidermal growth factor had a potent trophic effect on both somatic and gastrointestinal fetal growth and resulted in a mean gastric acid concentration of 35.2 +/- 6.6 mumole/ml. In contrast, pentagastrin, although restoring gastric weight to control values, had no effect on gastric acid secretion, with a mean of 0.1 +/- 0.1 mumole/ml. We conclude that fetal swallowing of amniotic fluid is essential in fetal gastrointestinal development, possibly via luminal trophic actions of peptides such as epidermal growth factor and gastrin.

The role of amniotic fluid in fetal nutrition.

The contribution of amniotic fluid to fetal growth and gastrointestinal tract development was studied in a rabbit model. In the fetal rabbit, at 23 days gestation, 3 conditions were surgically produced: (1) prevention of swallowing of amniotic fluid by esophageal ligation (n = 8); (2) esophageal ligation but insertion of an esophageal cannula distally to allow continuous infusion into the stomach of bovine amniotic fluid to mimic fetal swallowing (n = 7); and (3) sham operation (n = 7). Fetuses were delivered by Caesarean section at 28 days gestation. Esophageal ligation resulted in significant reductions of birth weight and crown-rump length and a trend to decreased liver weight when compared to sham operated controls. Additionally, marked reductions in gastric and intestinal tissue weight and gastric acidity were found following esophageal ligation. These reductions in both somatic and gastrointestinal tract growth and gastric function were reversed by infusion of amniotic fluid intragastrically. We conclude that amniotic fluid provides 10% to 14% of the nutritional requirements of the normal fetus, and that amniotic fluid contains a potent and as yet undefined gastrointestinal tract trophic factor.

Endorectal ileoanal anastomosis with isoperistaltic ileal reservoir after colectomy and mucosal proctectomy.

Forty-nine patients with chronic ulcerative colitis refractory to medical therapy and four with multiple polyposis have undergone total colectomy, mucosal protectomy, and endorectal ileal pull-through with ileoanal anastomosis at the UCLA Medical Center during the past 12 years (mean age, 19.4 years). Thirty-eight patients underwent second-stage closure of the ileostomy with construction of a side-to-side isoperistaltic ileal reservoir (mean, 6 months) after the ileal pullthrough operation. The anastomosis extended over a 20–30 cm distance and the lower end was placed within 6–8 cm of the ileoanal anastomosis. Transient reservoir inflammation, which occurred in half of the patients, was reduced by the use of oral metranidazole and was rarely found 6 months after operation. No patients died during the early or late postoperative periods. Cuff abscess in two patients and obstruction of the ileal reservoir outlet have required takedown of the reservoir (two patients) or temporary ileostomy (three patients). Of the 38 patients who have undergone lateral ileal reservoir construction, 33 have achieved a good to excellent result with complete continence and an average of five stools per 24 hours after 6 months. At least 12 patients now participate in competitive athletics; normal sexual activity has been achieved in all but one patient. Seven patients await construction of the reservoir. Although a technically difficult operation, the long-term results (mean, 19.4 months) indicate that the pullthrough operation is a good alternative to standard proctocolectomy.

Management of extrahepatic portal hypertension in children.

Among 69 patients with PVT, 338 variceal bleeding episodes occurred. Only two patients died from bleeding, and both lived in remote communities and were inaccessible to medical care. Fifty-three children underwent 164 operations for the management of PVT. Once operative management was undertaken, subsequent operations frequently were necessary. Nonoperative measures controlled acute variceal hemorrhage in most instances during the past 10 years. Almost all patients who underwent splenectomy alone, variceal ligation, gastric division, splenic transposition, or makeshift shunts subsequently rebled. These operations are rarely indicated in the current management of children with PVT. Portal venography is essential to define the portal venous circulation before a shunt operation is attempted. Cavomesenteric or central splenorenal shunts prevented further bleeding in eight of 15 patients and are the most reliable operations to control bleeding in patients with PVT. Emergency operation is rarely necessary to control bleeding. Sixteen patients (average age 14.6 years) with PVT did not undergo any operations, and are alive. Each of the six patients with PVT who died from complications of portal hypertension did so within nine months of an operation. Four of these patients had previous splenectomy and died with sepsis as one of the major factors. Bleeding episodes became less frequent as the patients increased in age. Patients who underwent shunts under unfavorable circumstances or who received various other operations to treat portal hypertension appeared to have a higher risk of morbidity and mortality than those managed nonoperatively.