Congenital Atresia of the
Duodenum
With Gastric Perforation Case
Report
and Review of the Literature
Jun Takebayashi, MD; Kenzo Asada, MD; Kanji Tokura, MD; Akira Ohokita, MD; Masahiro Okuno, MD; Katsusuke Satake, MD \s=b\ Gastric perforation associated with duodenal atresia is a very rare condition. We are able to cure an infant with this complicated disease by using a two-stage operation. Of interest is the occurrence of massive subcutaneous emphysema in our
patient. (Am J Dis Child 129:1227-1228, 1975) duodenal atresia
Congenitalby gastric perforationcompli¬
is a condition. Until now, 11 very cases have been reported in the litera¬ ture.111 We encountered a patient with congenital duodenal atresia cated rare
complicated by gastric perforation that was treated successfully by a two-stage operation.
The purpose of this article is to re¬ port our case and review the litera¬ ture.
REPORT OF A CASE A boy was born on March 3,1966, from a 22-year-old primigrávida mother, follow¬ ing a 36-week gestation associated with hydramnion. Immediately after birth, the baby ap¬ peared normal and had a normal bowel movement.
The birth weight was 2,550 gm (5.6 lb). Seven hours after birth, the upper part of the abdomen was slightly distended and the baby vomited a small amount of dark blood. At the same time, subcutaneous em¬ physema was noted in the scrotum and right lower part of the abdomen. Received for publication Dec 13,1973; accepted Feb 4, 1975. From the First Department of Surgery, Osaka
City University, Japan. Reprint requests to Department of Surgery, Osaka City University Medical School, 5-7, Asahimachi 1 chome, Abeno-ku, Osaka, 545, Japan (Dr. Rakebayashi).
Forty hours after birth, the abdominal distention had progressed and the subcu¬ taneous emphysema had extended to the midchest level. The patient had difficulty in breathing and was cyanosed; he was trans¬ ferred to our surgical clinic about 42 hours after birth. Physical examination showed a neonate in acute distress. His temperature was 38 C (101 F) and weight was 2,250 gm (6.0 lb). Cyanosis and dyspnea were clearly appar¬ ent. The heart and respiration rates were severely elevated. Abdominal examination showed substantial abdominal distention and absence of bowel sounds in all four
quadrants. The abdominal roentgenogram disclosed massive pneumoperitoneum with the "dou¬ ble bubble" sign and severe abdominothoracic subcutaneous emphysema (Fig¬ ure). An exploratory laparotomy was performed following the diagnosis of duodenal obstruction with gastric perfora¬ tion. At operation, a large amount of free air was found in the peritoneal cavity. The stomach and entire duodenum were se¬ verely dilated and the distal part of the small intestine and colon were collapsed. A large amount of milky fluid containing pus was found in the peritoneal cavity. A gas¬ tric perforation of about 3 mm in diame¬ ter, the edge of which was necrotic, was found on the lesser curvature. The perfora¬ tion was closed by two-layer suture. We found no other abnormalities in the ab¬ dominal cavity. As the patient's condition was deteriorating because of progressive cyanosis, the operation had to be discontin¬ ued. On the third day postoperatively, the subcutaneous emphysema had decreased to a great degree and the cyanosis had cleared gradually. This improvement con¬ tinued except for vomiting and weight loss
(300
gm
Three
[0.7 lb]). days later, the second operation
Downloaded From: http://archpedi.jamanetwork.com/ by a University of Iowa User on 06/19/2015
performed. The suture line on the lesser curvature of the stomach was found to be intact, but the wall of duodenum proximal to the obstruction was distinctly hypertrophied and dilated. There were ad¬ hesions around the antrum of the stomach and the transverse colon, and an atresia of the duodenum was found near the liga¬ ment of Treitz. A side-to-side antecolic duodenojejunostomy was performed. The patient was given fluids parenterally for the first four days, and then he received tube feedings. Oral feeding was started on the sixth day and later the tube was re¬ moved. The baby cried well, had good suck¬ ing reflex and normal bowel movements, and was finally discharged from hospital on the 31st postoperative day on a routine infant feeding schedule. An upper-gas¬ trointestinal series of roentgenograms two months later showed normal passage of barium to the small intestine. The patient developed satisfactorily and weighed 4,550 gm (10.0 lb) two months after operation. At the age of 8 years, he has continued to was
develop normally. COMMENT
Neonatal gastric perforation sec¬ ondary to or associated with duodenal atresia has been reported to date on only 11 occasions (Table). In 1940, Brody1 reported the first case of gas¬ tric perforation associated with duodenal atresia. This was an autopsy
finding.
In 1956, Kiesewetter2 attempted the first operative repair of this com¬ plicated condition, but unfortunately the infant died six days after oper¬ ation. Since then, several cases have been reported, but the patients in all of these died either following oper¬ ation or spontaneously.
As far as we know, our patient is the second cured by operation, and it is interesting to note that mas¬ sive subcutaneous emphysema was an important clinical finding. Kiesewetter2 reported massive subcutaneous emphysema from gastric perforation in a patient with inguinal hernia, and he suspected that the cause was air infiltration from an inguinal hernia. In our case, an inguinal hernia was not found and the cause was not clear. However, the possibility re¬ mains that air may have infiltrated the subcutaneous tissue of the trunk, scrotum, and an inguinal region from the peritoneal cavity. The treatment usually recom¬ mended for duodenal obstruction or atresia is retrocolic duodenojejunos¬ tomy. In our case, antecolic duodeno¬ jejunostomy was performed because of an adhesion due to the first oper¬ ation. The two-stage operation was well tolerated and the subsequent progress of this child was normal. It is suggested that this complicated condition can be cured by good care of the patient, together with either a
only
one-stage View of abdomen of infant in upright position, showing "double bubble" appearance of distended stomach and duodenum, with massive pneumoperitoneum.
Congenital Atresia of the Duodenum Author & Year
Age (Days) & Sex
Brody,1 1940 Kiesewetter,21956 Ishida 8 Saito,3 1962
6, F 5, M 2, M
Yamazaki,41964 Ogawa,6 1966 Ueda,7 1968 Takahashi,81971 Sakoguchi,'1971 Nishioka,101971
4, M 3, F 3, M
Kaku,"
1973
The first successful
2, IVI 8, Ivi 5, F
With Gastric Perforation
Coexisting Anomalies
Operation
Duodenojejunostomy Gastrostomy
S
je-
junal intubation
laparotomy ? Duodenoduodenostomy Gastrojejunostomy Laparotomy ? Laparotomy Gastrojejunostomy Gastrojejunostomy Laparotomy ?
operation
was
reported by Ogawa8 in 1966. In his case, atresia of the second part of the duodenum and a membranous septum in the lumen of the duodenum just above the level of the Treitz ligament were found. The gastric perforation was closed and end-to-end duodenoduodenostomy after removal of the
Mongoloid facies
Esophageal
atresia
Scleroderma
or
two-stage operation.
References
Brody H: Ruptured diverticulum of the a newborn infant, associated with congenital membrane occluding the duodenum. Arch Pathol 29:125-128, 1940. 2. Kiesewetter WB: Spontaneous rupture of 1.
stomach in
Result Died Died Died Died Recovered Died Died Died Died Died Died
membranous septum was performed. Gastric perforation and duodenal atresia each have high mortality. It is obvious that the combination of these two diseases has a higher mortality than either of the single diseases. In addition, babies with this condition are usually premature and have other congenital abnormalities.
Downloaded From: http://archpedi.jamanetwork.com/ by a University of Iowa User on 06/19/2015
the stomach in the newborn. Am J Dis Child 91:162-167, 1956. 3. Ishida M, Saito S: Perforation and rupture of the stomach in the newborn. Operation 16:887, 1962. 4. Yamazaki K: A case of stomach perforation in a newborn infant. J Tokyo Women's Med College 34:776, 1964. 5. Shaw A, Blanc WA, Santulli TV, et al: Spontaneous rupture of the stomach in the newborn: A clinical and experimental study. Surgery 58:561-571, 1965. 6. Ogawa M: A survival case of gastric perforation in the newborn. J Jap Soc Pediatr Surg 2:74, 1966. 7. Ueda S: Three cases of the neonatal gastric perforation and rupture. Surg Diagnosis Treatment 10:1190, 1968. 8. Takahashi H: Congenital duodenal atresia with gastric rupture. J Jap Soc Pediatr Surg 7:238, 1971. 9. Sakoguchi Y: Three cases of gastric perforation in the newborn. J Jap Soc Pediatr Surg 7:247, 1971. 10. Nishioka T: Two cases of congenital duodenal atresia with gastric perforation. Ther Tokyo 5:101, 1971. 11. Kaku N: Ten cases of gastric perforation. J Jap Soc Pediatr Surg 9:633, 1973.
Duodenum
With Gastric Perforation Case
Report
and Review of the Literature
Jun Takebayashi, MD; Kenzo Asada, MD; Kanji Tokura, MD; Akira Ohokita, MD; Masahiro Okuno, MD; Katsusuke Satake, MD \s=b\ Gastric perforation associated with duodenal atresia is a very rare condition. We are able to cure an infant with this complicated disease by using a two-stage operation. Of interest is the occurrence of massive subcutaneous emphysema in our
patient. (Am J Dis Child 129:1227-1228, 1975) duodenal atresia
Congenitalby gastric perforationcompli¬
is a condition. Until now, 11 very cases have been reported in the litera¬ ture.111 We encountered a patient with congenital duodenal atresia cated rare
complicated by gastric perforation that was treated successfully by a two-stage operation.
The purpose of this article is to re¬ port our case and review the litera¬ ture.
REPORT OF A CASE A boy was born on March 3,1966, from a 22-year-old primigrávida mother, follow¬ ing a 36-week gestation associated with hydramnion. Immediately after birth, the baby ap¬ peared normal and had a normal bowel movement.
The birth weight was 2,550 gm (5.6 lb). Seven hours after birth, the upper part of the abdomen was slightly distended and the baby vomited a small amount of dark blood. At the same time, subcutaneous em¬ physema was noted in the scrotum and right lower part of the abdomen. Received for publication Dec 13,1973; accepted Feb 4, 1975. From the First Department of Surgery, Osaka
City University, Japan. Reprint requests to Department of Surgery, Osaka City University Medical School, 5-7, Asahimachi 1 chome, Abeno-ku, Osaka, 545, Japan (Dr. Rakebayashi).
Forty hours after birth, the abdominal distention had progressed and the subcu¬ taneous emphysema had extended to the midchest level. The patient had difficulty in breathing and was cyanosed; he was trans¬ ferred to our surgical clinic about 42 hours after birth. Physical examination showed a neonate in acute distress. His temperature was 38 C (101 F) and weight was 2,250 gm (6.0 lb). Cyanosis and dyspnea were clearly appar¬ ent. The heart and respiration rates were severely elevated. Abdominal examination showed substantial abdominal distention and absence of bowel sounds in all four
quadrants. The abdominal roentgenogram disclosed massive pneumoperitoneum with the "dou¬ ble bubble" sign and severe abdominothoracic subcutaneous emphysema (Fig¬ ure). An exploratory laparotomy was performed following the diagnosis of duodenal obstruction with gastric perfora¬ tion. At operation, a large amount of free air was found in the peritoneal cavity. The stomach and entire duodenum were se¬ verely dilated and the distal part of the small intestine and colon were collapsed. A large amount of milky fluid containing pus was found in the peritoneal cavity. A gas¬ tric perforation of about 3 mm in diame¬ ter, the edge of which was necrotic, was found on the lesser curvature. The perfora¬ tion was closed by two-layer suture. We found no other abnormalities in the ab¬ dominal cavity. As the patient's condition was deteriorating because of progressive cyanosis, the operation had to be discontin¬ ued. On the third day postoperatively, the subcutaneous emphysema had decreased to a great degree and the cyanosis had cleared gradually. This improvement con¬ tinued except for vomiting and weight loss
(300
gm
Three
[0.7 lb]). days later, the second operation
Downloaded From: http://archpedi.jamanetwork.com/ by a University of Iowa User on 06/19/2015
performed. The suture line on the lesser curvature of the stomach was found to be intact, but the wall of duodenum proximal to the obstruction was distinctly hypertrophied and dilated. There were ad¬ hesions around the antrum of the stomach and the transverse colon, and an atresia of the duodenum was found near the liga¬ ment of Treitz. A side-to-side antecolic duodenojejunostomy was performed. The patient was given fluids parenterally for the first four days, and then he received tube feedings. Oral feeding was started on the sixth day and later the tube was re¬ moved. The baby cried well, had good suck¬ ing reflex and normal bowel movements, and was finally discharged from hospital on the 31st postoperative day on a routine infant feeding schedule. An upper-gas¬ trointestinal series of roentgenograms two months later showed normal passage of barium to the small intestine. The patient developed satisfactorily and weighed 4,550 gm (10.0 lb) two months after operation. At the age of 8 years, he has continued to was
develop normally. COMMENT
Neonatal gastric perforation sec¬ ondary to or associated with duodenal atresia has been reported to date on only 11 occasions (Table). In 1940, Brody1 reported the first case of gas¬ tric perforation associated with duodenal atresia. This was an autopsy
finding.
In 1956, Kiesewetter2 attempted the first operative repair of this com¬ plicated condition, but unfortunately the infant died six days after oper¬ ation. Since then, several cases have been reported, but the patients in all of these died either following oper¬ ation or spontaneously.
As far as we know, our patient is the second cured by operation, and it is interesting to note that mas¬ sive subcutaneous emphysema was an important clinical finding. Kiesewetter2 reported massive subcutaneous emphysema from gastric perforation in a patient with inguinal hernia, and he suspected that the cause was air infiltration from an inguinal hernia. In our case, an inguinal hernia was not found and the cause was not clear. However, the possibility re¬ mains that air may have infiltrated the subcutaneous tissue of the trunk, scrotum, and an inguinal region from the peritoneal cavity. The treatment usually recom¬ mended for duodenal obstruction or atresia is retrocolic duodenojejunos¬ tomy. In our case, antecolic duodeno¬ jejunostomy was performed because of an adhesion due to the first oper¬ ation. The two-stage operation was well tolerated and the subsequent progress of this child was normal. It is suggested that this complicated condition can be cured by good care of the patient, together with either a
only
one-stage View of abdomen of infant in upright position, showing "double bubble" appearance of distended stomach and duodenum, with massive pneumoperitoneum.
Congenital Atresia of the Duodenum Author & Year
Age (Days) & Sex
Brody,1 1940 Kiesewetter,21956 Ishida 8 Saito,3 1962
6, F 5, M 2, M
Yamazaki,41964 Ogawa,6 1966 Ueda,7 1968 Takahashi,81971 Sakoguchi,'1971 Nishioka,101971
4, M 3, F 3, M
Kaku,"
1973
The first successful
2, IVI 8, Ivi 5, F
With Gastric Perforation
Coexisting Anomalies
Operation
Duodenojejunostomy Gastrostomy
S
je-
junal intubation
laparotomy ? Duodenoduodenostomy Gastrojejunostomy Laparotomy ? Laparotomy Gastrojejunostomy Gastrojejunostomy Laparotomy ?
operation
was
reported by Ogawa8 in 1966. In his case, atresia of the second part of the duodenum and a membranous septum in the lumen of the duodenum just above the level of the Treitz ligament were found. The gastric perforation was closed and end-to-end duodenoduodenostomy after removal of the
Mongoloid facies
Esophageal
atresia
Scleroderma
or
two-stage operation.
References
Brody H: Ruptured diverticulum of the a newborn infant, associated with congenital membrane occluding the duodenum. Arch Pathol 29:125-128, 1940. 2. Kiesewetter WB: Spontaneous rupture of 1.
stomach in
Result Died Died Died Died Recovered Died Died Died Died Died Died
membranous septum was performed. Gastric perforation and duodenal atresia each have high mortality. It is obvious that the combination of these two diseases has a higher mortality than either of the single diseases. In addition, babies with this condition are usually premature and have other congenital abnormalities.
Downloaded From: http://archpedi.jamanetwork.com/ by a University of Iowa User on 06/19/2015
the stomach in the newborn. Am J Dis Child 91:162-167, 1956. 3. Ishida M, Saito S: Perforation and rupture of the stomach in the newborn. Operation 16:887, 1962. 4. Yamazaki K: A case of stomach perforation in a newborn infant. J Tokyo Women's Med College 34:776, 1964. 5. Shaw A, Blanc WA, Santulli TV, et al: Spontaneous rupture of the stomach in the newborn: A clinical and experimental study. Surgery 58:561-571, 1965. 6. Ogawa M: A survival case of gastric perforation in the newborn. J Jap Soc Pediatr Surg 2:74, 1966. 7. Ueda S: Three cases of the neonatal gastric perforation and rupture. Surg Diagnosis Treatment 10:1190, 1968. 8. Takahashi H: Congenital duodenal atresia with gastric rupture. J Jap Soc Pediatr Surg 7:238, 1971. 9. Sakoguchi Y: Three cases of gastric perforation in the newborn. J Jap Soc Pediatr Surg 7:247, 1971. 10. Nishioka T: Two cases of congenital duodenal atresia with gastric perforation. Ther Tokyo 5:101, 1971. 11. Kaku N: Ten cases of gastric perforation. J Jap Soc Pediatr Surg 9:633, 1973.
