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Upper gi endoscopy in cornelia de lange syndrome
PEDIATRIC ENDOSCOPY $145
"/'147
ERCP IN THE EVALUATION AND DIAGNOSIS OF ANOMALOUS PANCREATICOBILIARY JUNCTION IN CHILDHOOD M Guelmd, C Morera, M Rodriguez, Prados JG, Jaen D GI Unit and Pediatric G1 Unit, Hospital General del Oeste, Los Magallanes, Caracas, Venezuela. Anomalous pancrcaticobiliary Junction (APBJ) has been defined as a smgle communication of the common bile duct with the pancreatic duct that forms a long common channel located outside the duodenal wall and is therefore not under the influence of the sphincter of Oddi. In adults, APBJ is said to be present when the common channel outside the duodenal wall is greater than 6 mm This measurement is not applicable in children. In adults, APBJ may be associated with an increased risk of malignancy We report our experience in children with APBJ diagnosed by ERCP METHODS: ERCP was performed in neonates and young infants with a pediatric Olympus duodenoscope and a tapered cannula In children older than 3 years an adult duodenoscope was used. During the procedure the heart rate and oxygen saturation was continuously monitored. ERCP was performed on an ambulatory basis and children were discharged usually in a period of 2 to 4 hours. The mother was instructed to call in case of problems. RESULTS: In 294 consecutive cases of successful ERCP, APBJ was found in 66 (22.4%) patients with an age range of 30 days to 19 years (f47, m:19) Two patients group were identified in the basis of the age at which ERCP was performed Group 1 included 156 neonates or young infants in which ERCP was performed to evaluate neonatal cholestasis. Six (2.0%) patients had APBJ: one (0.3%) with neonatal hepatitis, three (1.0%) with biliary atresia, and two (0.7%) with choledochal cyst Group 2 included 138 children older than 1 year in which ERCP was performed to evaluate recurrent jaundice (48 patients), recurrent pancreatitis (11 patients), and pain with abdominal mass (1 patient). Sixty (20.4%) patients had APBJ: 57 (19.4%) with choledochal cyst, 2 (0.7%) with pancreas divisum associated with choledochal c~,st, and 3 (1.0%0) with no other associated abnormalities. After ERCP two patients had abdominal pain which disappeared completely without treatment. One neonate had mild duodenal erosions without clinical consequences. CONCLUSION: ERCP is of significant value in establishing the diagnosis of APBJ, and in defining anatomic and pathologqc relationships such as choledochal cyst. ERCP should be performed early in the evaluation of children with recurrent jaundice and recurrent pancreatitis,
UPPER G I ENDOSCOPY I N C O M L T ~ k D E
I~l~E
SMRM
T.S. Gunasekaran, J. Berman, J. Kraut. Division of Pediatric Gastroenterology, Lutheran General Children's Hospital, Park Ridge, IL. Cornelia de Lange Syndrome (CdLS) is a rare disorder characterized by multiple congenital anomalies, mental retardation and severe growth failure. Gastrointestinal malformation and malfunction, particularly of the upper GI tract are seen frequently in children with CDL syndrome. Methods: 6 children with CdLS, mean age 3.5 yr (0.5-8.0), weight 8.1 kg (5.0-13.5) were seen for gastrointestinal symptoms. They included vomiting (5), coughing/choking (4), and feeding difficulty requiring tube feedings (4). Previous surgery included pyloromyotomy in one and Nissen fundoplication in another. 5 patients had barium upper GI studies. 5 patients had upper GI endoscopy to the descending duodenum with esophageal and gastric antral biopsies. Results: Upper GI endoscopy findings included: esophageal stricture (2), hiatal hernia (2), erythema and/or erosions of the distal esophagus (4). Esophageal biopsies demonstrated esophagitis (4) and Barrett esophagus (i). In 3 patients the proximal esophagus was difficult to intubate. Barium upper GI studies showed esophageal stricture (2), hiatal hernia (I), aberrant subclavian artery (I), poor esophageal motility (I) and gastroesophageal reflux (4). Conclusion: Children with CdLS have a high incidence of complications of GE reflux including stricture and Earrett's esophagus. Abnormal anatomy of the oropharynx may make esophageal intubation more difficult.
146
$148
ESOPHAGITIS FROM RETCHING AND VOMITING: A NEW ENDOSCOPIC FINDING TS Gunasekaran. J Berman, J Kraut. Lutheran General Children's Hospital, Park Ridge, 1L We describe a type of esophagitis seen in children with retching and vomiting, which has a distinct endoscopic appearance. Patients: Four children, three girls and one boy, were evaluated for recurrent vomiting. Mean age was 6.5 yrs. (5-10)and weight 29.05 kg (20-37.2). All had hematemesis at the time of evaluation. Methods: All patients had CBC, electrolytes, BUN, creatinine, liver function tests, amylase, urine analysis and bariurn upper GI series. Upper GI endoscopy was done within 24 hours of the bleeding. Two to three biopsies from descending duodenum, antrum and esophagus (3-4 cm above the Z line) were done. Results: The mean hemoglobin was 13.65 grrgdl (12.9-14.7). Barium upper GI series, urine analysis and blood tests were normal. Endoscopy in all showed the following changes in the distal half of the esophagus; confluent redness, with 4-6 "linear" whitish areas in between extending down to the Z-line. The proximal half of the esophagus, stomach and duodenum were normal. Biopsies of the esophagus showed changes of reflux esophagitis and were negative for fungus (including brushings) and inclusion bodies. Patients were treated with ranitidine for two weeks. Twenty four hour esophageal pH monitoring was done in two patients, three weeks following the vomiting episode and was normal. Follow-up endoscopies were done six to eight weeks later in two patients and were normal. Follow-up up to 30 months showed recurrence of vomiting six to eight months later without bleeding in two patients and no vomiting in one. Conclusion: Peptic or infectious esophagitis, the commonest causes of esophagitis in children, produces redness, erosions or ulcers, but have not been shown to have an endoscopic appearance similar to that described here. Severe retching and vomiting produce this distinct endoscopic appearance of the distal half of the esophagus.
LONGTERM OUTCOME OF VERTICAL LINES IN THE (DISTAL) ESOPHAGEAL MUCOSA (VLEM) AND EOSINOPHILIC ESOPHAGITIS: A PROSPECTIVE STUDY. SK Gupta, JF Fitzgerald, SKF Chong, JM Croffie, ME Ellett. Div of Pediatric Gastroenterology, Indiana Univ School of Medicine, Indianapolis. Introduction- Last year we reported a previously unpublished endoscopic finding in children with eosinophilic/peptic esophagitis, viz VLEM (Gastrointest Endoscopy 1995;41:338). Aim: We now present data from a prospective study of this possible association and report on it's response to therapy. Methods: Endoscopists recorded the findings for each esophagogastroduodenoscopy (EGD) (n322) performed between 6/1/95-11/30/95 on a standard form. Histological assessments of esophageal mucosal biopsies were recorded. Results: VLEM were observed in 22 patients (pts) (ages 3mo-17 yrs) (12 new pts) who underwent 27 EGDs during the study period. Esophageal mucosal biopsies were obtained for histological study during each of these EGDs. VLEM were observed on 25 EGDs. 23/25 (92%; specificity 0.96, sensitivity 0.54) cases had eosinophilic esophagitis. 20/23 (87%) were subjectively graded moderate or severe by the pathologists. 14/22 pts underwent ~ 2 EGDs. VLEM and histological findings were unchanged in 10/14 pts on follow up (f/u) (mean 6.7mo, range 2-19 mo) and improved/resolved in 3/14 on f/u (mean 12.3mo, range 5-27 mo). In one pt, VLEM appeared on repeat EGD (2 mo later) while the histology improved. All pts had received similar therapy (promotility agent and aggressive acidsuppression) and 11/14 reported symptomatic improvement. Conclusions: In this prospective study, we have confirmed our earlier retrospective observation of the positive correlation between VLEM and histological eosinophilic/peptic esophagitis. Additionally, the data suggests that VLEM and the associated histological abnormalities are generally slow to respond to therapy inspite of symptomatic improvement. Thus, prolonged therapy with endoscopic monitoring may be indicated. The long term implication(s) of these observations awaits further study.
VOLUME 43, NO. 4, 1996
GASTROINTESTINAL ENDOSCOPY 327
"/'147
ERCP IN THE EVALUATION AND DIAGNOSIS OF ANOMALOUS PANCREATICOBILIARY JUNCTION IN CHILDHOOD M Guelmd, C Morera, M Rodriguez, Prados JG, Jaen D GI Unit and Pediatric G1 Unit, Hospital General del Oeste, Los Magallanes, Caracas, Venezuela. Anomalous pancrcaticobiliary Junction (APBJ) has been defined as a smgle communication of the common bile duct with the pancreatic duct that forms a long common channel located outside the duodenal wall and is therefore not under the influence of the sphincter of Oddi. In adults, APBJ is said to be present when the common channel outside the duodenal wall is greater than 6 mm This measurement is not applicable in children. In adults, APBJ may be associated with an increased risk of malignancy We report our experience in children with APBJ diagnosed by ERCP METHODS: ERCP was performed in neonates and young infants with a pediatric Olympus duodenoscope and a tapered cannula In children older than 3 years an adult duodenoscope was used. During the procedure the heart rate and oxygen saturation was continuously monitored. ERCP was performed on an ambulatory basis and children were discharged usually in a period of 2 to 4 hours. The mother was instructed to call in case of problems. RESULTS: In 294 consecutive cases of successful ERCP, APBJ was found in 66 (22.4%) patients with an age range of 30 days to 19 years (f47, m:19) Two patients group were identified in the basis of the age at which ERCP was performed Group 1 included 156 neonates or young infants in which ERCP was performed to evaluate neonatal cholestasis. Six (2.0%) patients had APBJ: one (0.3%) with neonatal hepatitis, three (1.0%) with biliary atresia, and two (0.7%) with choledochal cyst Group 2 included 138 children older than 1 year in which ERCP was performed to evaluate recurrent jaundice (48 patients), recurrent pancreatitis (11 patients), and pain with abdominal mass (1 patient). Sixty (20.4%) patients had APBJ: 57 (19.4%) with choledochal cyst, 2 (0.7%) with pancreas divisum associated with choledochal c~,st, and 3 (1.0%0) with no other associated abnormalities. After ERCP two patients had abdominal pain which disappeared completely without treatment. One neonate had mild duodenal erosions without clinical consequences. CONCLUSION: ERCP is of significant value in establishing the diagnosis of APBJ, and in defining anatomic and pathologqc relationships such as choledochal cyst. ERCP should be performed early in the evaluation of children with recurrent jaundice and recurrent pancreatitis,
UPPER G I ENDOSCOPY I N C O M L T ~ k D E
I~l~E
SMRM
T.S. Gunasekaran, J. Berman, J. Kraut. Division of Pediatric Gastroenterology, Lutheran General Children's Hospital, Park Ridge, IL. Cornelia de Lange Syndrome (CdLS) is a rare disorder characterized by multiple congenital anomalies, mental retardation and severe growth failure. Gastrointestinal malformation and malfunction, particularly of the upper GI tract are seen frequently in children with CDL syndrome. Methods: 6 children with CdLS, mean age 3.5 yr (0.5-8.0), weight 8.1 kg (5.0-13.5) were seen for gastrointestinal symptoms. They included vomiting (5), coughing/choking (4), and feeding difficulty requiring tube feedings (4). Previous surgery included pyloromyotomy in one and Nissen fundoplication in another. 5 patients had barium upper GI studies. 5 patients had upper GI endoscopy to the descending duodenum with esophageal and gastric antral biopsies. Results: Upper GI endoscopy findings included: esophageal stricture (2), hiatal hernia (2), erythema and/or erosions of the distal esophagus (4). Esophageal biopsies demonstrated esophagitis (4) and Barrett esophagus (i). In 3 patients the proximal esophagus was difficult to intubate. Barium upper GI studies showed esophageal stricture (2), hiatal hernia (I), aberrant subclavian artery (I), poor esophageal motility (I) and gastroesophageal reflux (4). Conclusion: Children with CdLS have a high incidence of complications of GE reflux including stricture and Earrett's esophagus. Abnormal anatomy of the oropharynx may make esophageal intubation more difficult.
146
$148
ESOPHAGITIS FROM RETCHING AND VOMITING: A NEW ENDOSCOPIC FINDING TS Gunasekaran. J Berman, J Kraut. Lutheran General Children's Hospital, Park Ridge, 1L We describe a type of esophagitis seen in children with retching and vomiting, which has a distinct endoscopic appearance. Patients: Four children, three girls and one boy, were evaluated for recurrent vomiting. Mean age was 6.5 yrs. (5-10)and weight 29.05 kg (20-37.2). All had hematemesis at the time of evaluation. Methods: All patients had CBC, electrolytes, BUN, creatinine, liver function tests, amylase, urine analysis and bariurn upper GI series. Upper GI endoscopy was done within 24 hours of the bleeding. Two to three biopsies from descending duodenum, antrum and esophagus (3-4 cm above the Z line) were done. Results: The mean hemoglobin was 13.65 grrgdl (12.9-14.7). Barium upper GI series, urine analysis and blood tests were normal. Endoscopy in all showed the following changes in the distal half of the esophagus; confluent redness, with 4-6 "linear" whitish areas in between extending down to the Z-line. The proximal half of the esophagus, stomach and duodenum were normal. Biopsies of the esophagus showed changes of reflux esophagitis and were negative for fungus (including brushings) and inclusion bodies. Patients were treated with ranitidine for two weeks. Twenty four hour esophageal pH monitoring was done in two patients, three weeks following the vomiting episode and was normal. Follow-up endoscopies were done six to eight weeks later in two patients and were normal. Follow-up up to 30 months showed recurrence of vomiting six to eight months later without bleeding in two patients and no vomiting in one. Conclusion: Peptic or infectious esophagitis, the commonest causes of esophagitis in children, produces redness, erosions or ulcers, but have not been shown to have an endoscopic appearance similar to that described here. Severe retching and vomiting produce this distinct endoscopic appearance of the distal half of the esophagus.
LONGTERM OUTCOME OF VERTICAL LINES IN THE (DISTAL) ESOPHAGEAL MUCOSA (VLEM) AND EOSINOPHILIC ESOPHAGITIS: A PROSPECTIVE STUDY. SK Gupta, JF Fitzgerald, SKF Chong, JM Croffie, ME Ellett. Div of Pediatric Gastroenterology, Indiana Univ School of Medicine, Indianapolis. Introduction- Last year we reported a previously unpublished endoscopic finding in children with eosinophilic/peptic esophagitis, viz VLEM (Gastrointest Endoscopy 1995;41:338). Aim: We now present data from a prospective study of this possible association and report on it's response to therapy. Methods: Endoscopists recorded the findings for each esophagogastroduodenoscopy (EGD) (n322) performed between 6/1/95-11/30/95 on a standard form. Histological assessments of esophageal mucosal biopsies were recorded. Results: VLEM were observed in 22 patients (pts) (ages 3mo-17 yrs) (12 new pts) who underwent 27 EGDs during the study period. Esophageal mucosal biopsies were obtained for histological study during each of these EGDs. VLEM were observed on 25 EGDs. 23/25 (92%; specificity 0.96, sensitivity 0.54) cases had eosinophilic esophagitis. 20/23 (87%) were subjectively graded moderate or severe by the pathologists. 14/22 pts underwent ~ 2 EGDs. VLEM and histological findings were unchanged in 10/14 pts on follow up (f/u) (mean 6.7mo, range 2-19 mo) and improved/resolved in 3/14 on f/u (mean 12.3mo, range 5-27 mo). In one pt, VLEM appeared on repeat EGD (2 mo later) while the histology improved. All pts had received similar therapy (promotility agent and aggressive acidsuppression) and 11/14 reported symptomatic improvement. Conclusions: In this prospective study, we have confirmed our earlier retrospective observation of the positive correlation between VLEM and histological eosinophilic/peptic esophagitis. Additionally, the data suggests that VLEM and the associated histological abnormalities are generally slow to respond to therapy inspite of symptomatic improvement. Thus, prolonged therapy with endoscopic monitoring may be indicated. The long term implication(s) of these observations awaits further study.
VOLUME 43, NO. 4, 1996
GASTROINTESTINAL ENDOSCOPY 327