Endoscopy 4 (1979) 259-262
CASE REPORT
Endoscopic Therapy of a Congenital Oesophageal Stricture Division of Gastroenterology, Department of Internal Medicine, Division of Pediatrics and Division of Pediatric Surgery, Medizinische Hochschule Hannover
Summary
A sixteen months old girl with Down's-Syndrome suffered from congenital oesophageal membrane and segmental oesophageal stricture. In the neonatal period, two other associated malformations of the gastrointestinal tract (malrotation of gut and duodenal web) had been operated success-
fully. The first endoscopic examination of the eosophagus was performed at the age of sixteen months because of increasing dysphagia. A solid membrane in the lower part of the oesophagus was incised by diathermy. The associated segmental stenosis necessitated repeated bougienages. After this treatment the child developed well without
Zusammenfassung Es wird über ein Mädchen mit M. Down berichtet,
das im Alter von 16 Monaten erstmals Zeichen einer Dysphagie entwickelte. Im Neugeborenenal-
ter hatte bereits eine Malrotation mit Duodenalatresie operiert werden miissen. Bei der Ösophagoskopie stellte sich im unteren Drittel des Osophagus eine derbe Membran dar. Sie wurde mit einer Diathermiesonde durchtrennt. Die zusätzlich vorhandene segmentale Ösophagusstenose mußte wiederholt bougiert werden. Nach dieser Behandlung gedeiht das Kind gut, ohne erneute Zeichen einer Dysphagie aufzuweisen.
clinical signs of dysphagia.
Key-Words: Congenital oesophageal web, oesophageal stenosis, endoscopic therapy
Congenital oesophageal stenosis is a rare malformation (2, 4). The main symptom is dysphagia. In most of the patients, vomiting occurs only after feeding a solid food. Up to now, two methods have been applied for treatment of this malformation: surgical resection during bougienage (3, 5).
an open operation and
The development of gastrointestinal fiberscopes suitable for infants and children
enabled a third way of treatment, which was performed in our patient: the membrane was incised endoscopically, whereas the segmental 0013-726 X/79
1600-0259 $ 02.00 © 1979
stenosis was treated by consecutive dilatation with bougies.
Case Report N. C. (18. 05. 76) is the first child of apparently healthy parents aged 28 and 26 years. At birth, she presented the stigmata of DownSyndrome and the clinical symptoms of high
intestinal obstruction. During operation on the third day of life in an outside hospital duodenal web and malrotation of the bowel were found and treated by resection, end to end
anastomosis
Georg Thieme Publishers
and
fixation.
After
Downloaded by: National University of Singapore. Copyrighted material.
H. Huchzermeyer, M. Burdelski, M. Hruby
Fig. 1 a
H. Huchzermeyer, M. Burdelski, M. Hruby
Oesophagogram showing a congenital
web near the junction of middle and distal thirds of
the oesophagus with segmental stenosis distally. The proximal oesophagus is dilated.
operation, a barium meal performed by
nasogastric tube revealed normal duodenal passage. The child recovered well and was in good condition up to the sixteenth month of life. At this time, severe vomiting started. The barium swallow showed an oesophageal web and a segmental stenosis in the lower third of the oesophagus (Fig. 1 a).
Endoscopic findings and treatment
The endoscopic examination was performed with a pediatric gastrointestinal fiberscope (GIF-P2, Olympus) under general anesthesia. At a distance of 20 cm from the mouth, the oesophageal lumen was ob-
structed by a thick, non-transparent membrane with an excentric perforation, 2-3 mm wide (Fig. 2 a). Although the biopsy forceps could pass the perforation (Fig. 2b), the membrane was too strong for dilatation or rupturing. Biopsy specimens from above, in
and below the membrane showed normal oesophageal mucosa.
Fig. 1 b
The same area after endoscopic elec-
troincision of the web and after bougienage of the stenosis.
In a second examination, an attempt to dilate the perforation with a bougie under endoscopic control failed (Fig. 2c). Then, a pediatric gastrointestinal fiberscope with a normal seized instrumental channel was cho-
sen (TX-7, ACM). After a polyp snare had been inserted, the web was incised by diathermy. The procedure was completed by the insertion of a large gastrictube, which remained for one week. At this time, a barium swallow was normal (Fig. lb). Three months later, dysphagia and vomiting recurred. Endoscopically, a narrowing of the lower part of the oesophagus was discovered. Repeated bougienage succeeded in dilatation with as yet a good function. Discussion
Congenital oesophageal stenosis is a rare malformation which is observed with a relatively high incidence, as are other intestinal obstructions, in patients with Down-Syn-
Downloaded by: National University of Singapore. Copyrighted material.
260
Fig. 2a Endoscopic picture of congenital oesophageal web at 20 cm from the incisors.
261
Fig. 2b Only a biopsy forceps can pass the stenosis.
oesophagus (2, 3). A wide range of obstruction results in clinical symptoms varying from mild dysphagia to severe vomiting. The presentation of symptoms is often related to the start of solid foods (3). The diagnostic approach in patients with suspected oesophageal stenosis should be started with a barium svvallow. The barium should not be given with a gastric tube, as this can lead to false negative results as shown in our patient. The role of endoscopy is secondary. It helps to differentiate between primary, congenital malformations such as a web and achalasia, and secondary changes like inflammatory stenosis (1).
After the cause of the stenosis has been Fig. 2c A flexible rubber bougie is introduced beneath the fiberendoscope, but it failed to pass the stenosis. (Figs. Olympus-Instruments).
2ac were
made
with
drome (8). Three types of stenosis can be distinguished (2, 7): oesophageal web oesophageal segmental stenosis combination of oesophageal web and stenosis. The localization of the stenosis varies: most
of them presenting in the lower part of the
found a first attempt should be undertaken to remove the stenosis mechanically. The value of dilatation under fiberendoscopic control is obvious (6). If this method of treatment fails, the performance of electrocautery with a gastrointestinal fiberscope is justified in order to avoid intervention by thoracic surgery. The indication for this endoscopic treatment, however, should be restricted to patients with
clear anatomic situation in the poststenotic area. It should be emphasized, however, that optimal technical equipment and skilled endoscopic technique are essential, otherwise the risk of complications like perforation and mediastinitis is too high (6).
Downloaded by: National University of Singapore. Copyrighted material.
Endoscopic Therapy of a Congenital Oesophageal Stricture
262
H. Huchzermeyer: Endoscopic Therapy of a Congenital Oesophageal Stricture 6 Huchzermeyer, H., J. Freise, H. Becker: Dilatation of
References Burdelski, M.: Endoscopy in Pediatric Gastroenterology. Eur. J. Pediatr. 128 (1978) 33-39
2 Greenough, W. G.: Congenital oesophageal strictures. Amer. J. Roentgenol. 92 (1964) 994
3 Gross, R. E.: The Surgery of Infancy and Childhood. P. 103, W. B. Saunders Company, Philadelphia 1953
4 Hecker, W. Ch.: Osophagusstenosen im Kindesalter und ihre Therapie. Winch. Med. Wschr. 36 (1968) 2026 5 Holinger, P. H., K. C. Johnston, W. J. Potts: Congenital anomalies of oesophagus. Ann. Otol. Rhin. Laryng. 60
benign oesophageal strictures by peroral fiberendoscopic bougienage. Endoscopy 9 (1977) 207
7 Shamma'a, M. H., E. B. Benedict: Oesophageal webs. A report of 58 cases and an attempt at classification. New Engl. J. Med. 259 (1958) 378 8 Silverman, A.: Pediatric clinical gastroenterology. P. 47, The C. V. Mosby Company, Saint Louis 1971
(1951) 707
PD Dr. H. Huchzermeyer, Division of Gastroenterology, Department of Internal Medicine, Dr. M. Burdelski, Kinderklinik, Dr. M. Hruby, Kinderchirurgische Abteilung, Medizinische Hochschule Hannover, Karl-Wiechert-Allee 9, D-3000 Hannover 61
Downloaded by: National University of Singapore. Copyrighted material.
1
CASE REPORT
Endoscopic Therapy of a Congenital Oesophageal Stricture Division of Gastroenterology, Department of Internal Medicine, Division of Pediatrics and Division of Pediatric Surgery, Medizinische Hochschule Hannover
Summary
A sixteen months old girl with Down's-Syndrome suffered from congenital oesophageal membrane and segmental oesophageal stricture. In the neonatal period, two other associated malformations of the gastrointestinal tract (malrotation of gut and duodenal web) had been operated success-
fully. The first endoscopic examination of the eosophagus was performed at the age of sixteen months because of increasing dysphagia. A solid membrane in the lower part of the oesophagus was incised by diathermy. The associated segmental stenosis necessitated repeated bougienages. After this treatment the child developed well without
Zusammenfassung Es wird über ein Mädchen mit M. Down berichtet,
das im Alter von 16 Monaten erstmals Zeichen einer Dysphagie entwickelte. Im Neugeborenenal-
ter hatte bereits eine Malrotation mit Duodenalatresie operiert werden miissen. Bei der Ösophagoskopie stellte sich im unteren Drittel des Osophagus eine derbe Membran dar. Sie wurde mit einer Diathermiesonde durchtrennt. Die zusätzlich vorhandene segmentale Ösophagusstenose mußte wiederholt bougiert werden. Nach dieser Behandlung gedeiht das Kind gut, ohne erneute Zeichen einer Dysphagie aufzuweisen.
clinical signs of dysphagia.
Key-Words: Congenital oesophageal web, oesophageal stenosis, endoscopic therapy
Congenital oesophageal stenosis is a rare malformation (2, 4). The main symptom is dysphagia. In most of the patients, vomiting occurs only after feeding a solid food. Up to now, two methods have been applied for treatment of this malformation: surgical resection during bougienage (3, 5).
an open operation and
The development of gastrointestinal fiberscopes suitable for infants and children
enabled a third way of treatment, which was performed in our patient: the membrane was incised endoscopically, whereas the segmental 0013-726 X/79
1600-0259 $ 02.00 © 1979
stenosis was treated by consecutive dilatation with bougies.
Case Report N. C. (18. 05. 76) is the first child of apparently healthy parents aged 28 and 26 years. At birth, she presented the stigmata of DownSyndrome and the clinical symptoms of high
intestinal obstruction. During operation on the third day of life in an outside hospital duodenal web and malrotation of the bowel were found and treated by resection, end to end
anastomosis
Georg Thieme Publishers
and
fixation.
After
Downloaded by: National University of Singapore. Copyrighted material.
H. Huchzermeyer, M. Burdelski, M. Hruby
Fig. 1 a
H. Huchzermeyer, M. Burdelski, M. Hruby
Oesophagogram showing a congenital
web near the junction of middle and distal thirds of
the oesophagus with segmental stenosis distally. The proximal oesophagus is dilated.
operation, a barium meal performed by
nasogastric tube revealed normal duodenal passage. The child recovered well and was in good condition up to the sixteenth month of life. At this time, severe vomiting started. The barium swallow showed an oesophageal web and a segmental stenosis in the lower third of the oesophagus (Fig. 1 a).
Endoscopic findings and treatment
The endoscopic examination was performed with a pediatric gastrointestinal fiberscope (GIF-P2, Olympus) under general anesthesia. At a distance of 20 cm from the mouth, the oesophageal lumen was ob-
structed by a thick, non-transparent membrane with an excentric perforation, 2-3 mm wide (Fig. 2 a). Although the biopsy forceps could pass the perforation (Fig. 2b), the membrane was too strong for dilatation or rupturing. Biopsy specimens from above, in
and below the membrane showed normal oesophageal mucosa.
Fig. 1 b
The same area after endoscopic elec-
troincision of the web and after bougienage of the stenosis.
In a second examination, an attempt to dilate the perforation with a bougie under endoscopic control failed (Fig. 2c). Then, a pediatric gastrointestinal fiberscope with a normal seized instrumental channel was cho-
sen (TX-7, ACM). After a polyp snare had been inserted, the web was incised by diathermy. The procedure was completed by the insertion of a large gastrictube, which remained for one week. At this time, a barium swallow was normal (Fig. lb). Three months later, dysphagia and vomiting recurred. Endoscopically, a narrowing of the lower part of the oesophagus was discovered. Repeated bougienage succeeded in dilatation with as yet a good function. Discussion
Congenital oesophageal stenosis is a rare malformation which is observed with a relatively high incidence, as are other intestinal obstructions, in patients with Down-Syn-
Downloaded by: National University of Singapore. Copyrighted material.
260
Fig. 2a Endoscopic picture of congenital oesophageal web at 20 cm from the incisors.
261
Fig. 2b Only a biopsy forceps can pass the stenosis.
oesophagus (2, 3). A wide range of obstruction results in clinical symptoms varying from mild dysphagia to severe vomiting. The presentation of symptoms is often related to the start of solid foods (3). The diagnostic approach in patients with suspected oesophageal stenosis should be started with a barium svvallow. The barium should not be given with a gastric tube, as this can lead to false negative results as shown in our patient. The role of endoscopy is secondary. It helps to differentiate between primary, congenital malformations such as a web and achalasia, and secondary changes like inflammatory stenosis (1).
After the cause of the stenosis has been Fig. 2c A flexible rubber bougie is introduced beneath the fiberendoscope, but it failed to pass the stenosis. (Figs. Olympus-Instruments).
2ac were
made
with
drome (8). Three types of stenosis can be distinguished (2, 7): oesophageal web oesophageal segmental stenosis combination of oesophageal web and stenosis. The localization of the stenosis varies: most
of them presenting in the lower part of the
found a first attempt should be undertaken to remove the stenosis mechanically. The value of dilatation under fiberendoscopic control is obvious (6). If this method of treatment fails, the performance of electrocautery with a gastrointestinal fiberscope is justified in order to avoid intervention by thoracic surgery. The indication for this endoscopic treatment, however, should be restricted to patients with
clear anatomic situation in the poststenotic area. It should be emphasized, however, that optimal technical equipment and skilled endoscopic technique are essential, otherwise the risk of complications like perforation and mediastinitis is too high (6).
Downloaded by: National University of Singapore. Copyrighted material.
Endoscopic Therapy of a Congenital Oesophageal Stricture
262
H. Huchzermeyer: Endoscopic Therapy of a Congenital Oesophageal Stricture 6 Huchzermeyer, H., J. Freise, H. Becker: Dilatation of
References Burdelski, M.: Endoscopy in Pediatric Gastroenterology. Eur. J. Pediatr. 128 (1978) 33-39
2 Greenough, W. G.: Congenital oesophageal strictures. Amer. J. Roentgenol. 92 (1964) 994
3 Gross, R. E.: The Surgery of Infancy and Childhood. P. 103, W. B. Saunders Company, Philadelphia 1953
4 Hecker, W. Ch.: Osophagusstenosen im Kindesalter und ihre Therapie. Winch. Med. Wschr. 36 (1968) 2026 5 Holinger, P. H., K. C. Johnston, W. J. Potts: Congenital anomalies of oesophagus. Ann. Otol. Rhin. Laryng. 60
benign oesophageal strictures by peroral fiberendoscopic bougienage. Endoscopy 9 (1977) 207
7 Shamma'a, M. H., E. B. Benedict: Oesophageal webs. A report of 58 cases and an attempt at classification. New Engl. J. Med. 259 (1958) 378 8 Silverman, A.: Pediatric clinical gastroenterology. P. 47, The C. V. Mosby Company, Saint Louis 1971
(1951) 707
PD Dr. H. Huchzermeyer, Division of Gastroenterology, Department of Internal Medicine, Dr. M. Burdelski, Kinderklinik, Dr. M. Hruby, Kinderchirurgische Abteilung, Medizinische Hochschule Hannover, Karl-Wiechert-Allee 9, D-3000 Hannover 61
Downloaded by: National University of Singapore. Copyrighted material.
1
