- Email: [email protected]
Spontaneous rupture of the esophagus in a newborn infant
S P O N T A N E O U S R U P T U R E OF T H E E S O P H A G U S IN A NEWBORN INFANT IIoLLIS J.
WISEMAN, FRED
M.D., E U G E N E 1~. CELANO, M.D., AND
C. H E S T E R III, M.D. MOBILE, ALA.
T is our put[pose to call attention to sudden spontaneous r u p t u r e of the esophagus with the secondary development of tension pneumothorax in a newborn infant. Since this condition is rare in the neonatal period and since immediate s u r g e r y can be lifesaving, a brief note in the literature is felt justified. The t e r m " s p o n t a n e o u s " is used with reservation since the etiology is totally obscure. Less t h a n 200 ease reports of patients with spontaneous r u p t u r e of tile esophagus have been recorded in the literature. The series reported b y Derbes and Mitchell 1 included a 2month-old child whose death resulted front a pylorie stenosis and in whom an esophageal r u p t u r e was demonstrated at autopsy. To our knowledge, this is the youngest patient previously repot"ted.
Ia
CASE REPORT
The i n f a n t was the sixth child born of a healthy 26-year-old Negro. The mother was seen twice during her p r e n a t a l course and the only complication was slight vaginal bleeding 1 month p r i o r to delivery. A t 6:17 a.z~. on J a n u a r y 14 a 6 pound, 14 ounce female i n f a n t was born spontaneously at term. Presentation was vertex, right occiput anterior, and the membranes r u p t u r e d just p r i o r to delivery. F r o m t h e D e p a r t m e n t s of S u r g e r y a n d Pediatrics a n d the Pediatric O u t - p a t i e n t Department, Mobile General Hospital. 207
The infant cried spontaneously and resuscitation was not required at any time. She was sent to the newborn infant n u r s e r y where slight i n s p i r a t o r y retractions were noted b y the nurse. At :10:30 A.M. the patient was first examined b y a staff physician and again slight retractions were noted. She was placed in humidified oxygen in an incubator and did f a i r l y well until the following day when the retractions became more severe. A t the end of 24 hours she was started on routine feedings and tetracycline b y mouth. Later in the second d a y a chest film was llq&d~.
H e r breathing" continued to be shallow and irregular; eyanosis was relieved b y administration of oxygen, and she required occasional stimulation with caffeine. E x a m i n a t i o n revealed decreased breath sounds over t h e right chest. Penicillin given intramuscularly was added to the t h e r a p y during the second night. The chest film was not seen due to a change in house officers until the following morning, the t h i r d d a y of life. E x a m i n a t i o n at this time revealed a seriously ill i n f a n t with obvious emphysema. She was quite cyanotic and respirations were shallow and rapid. B r e a t h sounds were diminished throughout the r i g h t chest. F i n e rNes were present posteriorly at the left base. The x-ray taken the previous d a y revealed t h a t the h e a r t and mediastinum were shifted to the left. There was increased radioluceney throughout the right hemithorax and scattered oval and linear densities
208
THE
JOURNAL
(Fig. 1). It was felt that these findings represented a congenital lobar emphysema or a large cyst. I m m e d i a t e surgery was felt mandatory. SURGICAL
TREATMENT
A routine right thoracotomy incision was made. Upon opening tile chest, a mixture of milk and mucus poured out. A f t e r removing' a p p r o x i m a t e l y
OF P E D I A T R I C S
This tube was left in place and the perforation was closed with interr u p t e d sutures of 6-0 silk. The pleura was brought over the closure as a second layer. The chest was closed with three tubes in place, one situated posteriorly n e a r the site of the perforation. The procedure was well tolerated and tile infant was a p p a r e n t l y greatly improved.
A,
/0"ig. 1 . - - A , P r e o p e r a t i v e
]~.
c h e s t film s h o w i n g t e n s i o n p n e u m o t h o r a x taken 8 days postoperatively.
200 c.c. of this material, the ateleetatic right l u n g was identified and rotated anteriorly. A 1 era. longitudinal p e r f o r a t i o n witl~, ragged and friable edges was discovered in the midportion of the esophagus on the right side. The patency of the esophagus was demonstrated by passing a polyvinyl catheter into the stomach.
on r i g h t .
B,
Chest
film
POSTOPERATIVE TREAT1V[ENT A chest x-ray on the first postoperative day showed only a slight amount of fluid in the right chest, with complete expansion of the right lung. The thoracotomy tubes were removed on the second postoperative day and an x-ray showed the chest to be clearing" at this time.
W I S E M A N E T AL. :
R U P T U R E OF E S O P H A G U S
Penicillin and oxytetracycline were used to combat infection. Glucose water was started b y Levin tube on the fourth day and intravenous fluids were discontinued on the fifth day. On the sixth postoperative day an evaporated milk formula was begun b y gastric tube, and the tube was removed on the seventh day. Weight gain occurred 12 days postoperatively and on the twenty-first day the i n f a n t was disc h a r g e d to be followed in the clinic. Contrast studies of the esophagus performed before discharge were entirely noITnal. The patient has been seen twice in the clinic, the most recent visit being at 21~ months of age, at which time she weighed 9 pounds, 51~ ounces. She was having no difficulties at that time. DISCUSSION
Considerable attention in the literature has been devoted to spontaneous r u p t u r e of the esophagus since the original description by Boerhaave in 1724. E a r l y recognition has become increasingly i m p o r t a n t since improvements in thoracic s u r g e r y have made survival possible. According to Derbes and MitehelP only 35 per cent of the patients survive for 24 hours if surgery is not p e r f o r m e d promptly. The characteristic clinical and roentgenologic features occurring in older children and adults have been adequately described. ~-* The usual pattern of violent vomiting, followed by intense substernal pain, and associated with subcutaneous or mediastinal emphysema were, of course, absent in this patient. I t h ~ been stated that if a p n e u m o t h o r a x or hydropneumothorax develops, subcutaneous emphysema is absent or late in appearance. Tension p n e u m o t h o r a x which has previously been reported in association with r u p t u r e of the esophagus '~ was an unusual feature in this patient.
209
A number of etiologie factors have been suggested. 1, a, s Forceful vomiting and other conditions which could increase intraesophageal pressure such as defecation, epilepsy, etc., have been precipitating factors. I n this patient, in whom there was no vomiting, none of these would seem applicable. In a newborn infant intubation or pressure resuseitation would seem to be the most likely cause of a r u p t u r e d esophagus. This child, however, breathed spontaneously and was not resuscitated in a n y fashion. All personnel concerned were diligently questioned immediately following surgery and it is certain tha.t intubation was not performed. I t is highly probable t h a t the catastrophic event occurred at or shortly a f t e r delivery since this b a b y was noted to have some r e s p i r a t o r y distress at a few hours of age. I t is possible t h a t an esophagus filled with amniotie fluid could be r u p t u r e d b y sudden extrathoracic pressure such as strong uterine contractions. Two p r e m a t u r e infants recently seen in this hospital were operated on for spontaneous r u p t u r e of the stomach. One of these was subsequently autopsied and found to have a congenital defect of the gastric museularis. 7 Theoretically, such a congenital anomaly could occur in the esophagus. Since this child survived and no sections were obtained, this is sheer speculation. One f u r t h e r unusual feature in this patient was the location of the perforation. I t has been demonstrated experimentally that the weakest section of the esophagus is just above the eardia on the left side. This has been confirmed clinically as the most likely spot for rupture. This lesion occurred on. the right side in the middle third
210
THE JOURNAL OF PEDIATRICS
of the esophagus. Subsequent contrast studies have demonstrated no stricture or other abnormality in this area. Immediate surgery with closure of the defect is the obvious treatment. E a r l y recognition is mandatory. Since our diagnosis was delayed, it is felt that calling attention to this possibility in a newborn infant may alert others to earlier diagnosis. The importance of early roentgenographic examination in any newborn infant with unexplained respiratory distress should be emphasized. SUMMARY
Idiopathic r u p t u r e of the esophagus with a tension pneumothorax in a newborn infant is reported. Immediate surgery with closure of the defect is the only possible therapy. Possible etiologic factors are briefly commented upon.
We wish to express our appreciation to Dr. Rex Perkins, Medical College, University of Alabama, for his comments and surgical advice. REFERENCES 1. Derbes, V. J , and ~ i t c h e E , R. E.: Rupture of the Esophagus~ Surgery 39: 688~ and 39: 865~ 1956. 2. Naclerio, E. A.: The " V " Sign in the Diagnosis of Spontaneous Rupture of the Esophagus (An E a r l y Roentgen Clue), Am. J. Surg. 93: 291, 1957. 3. Ware, G. W , Shnider, B. I., and Davis, E. tI.: Spontaneous Rupture of the Esophagus: Report of a Successful Case W i t h Cardiac A r r e s t a n d a Review of the Literature~ A. M. A. Arch. Surg. 65: 723, ]952. 4. Christoforides~ A., and Nelson~ S. W.: Spontaneous Rupture of the Esophagus W i t h Emphasis on the Roentgenologic Diagnosis, Am. J. Roentgenol. 78: 574, 1957. 5. Pembleton, W. E.: Rupture of the Esophagus Causing Tension Pneumothorax, Virginia M. Monthly 73: 331, ]946. 6. Kirby, C. D.: Spontaneous Rupture of the Esophagus, Am. J. !VI. Sc. 229: 199, 1955. 7. Meyer, J. L , I h Congenital Defect in Musculature of Stomach Resulting in Spontaneous Gastric P e r f o r a t i o n in Neon a t a l Perio'd: Report of Two Cases, J. PEDIAT. 51: 416, 1957.
WISEMAN, FRED
M.D., E U G E N E 1~. CELANO, M.D., AND
C. H E S T E R III, M.D. MOBILE, ALA.
T is our put[pose to call attention to sudden spontaneous r u p t u r e of the esophagus with the secondary development of tension pneumothorax in a newborn infant. Since this condition is rare in the neonatal period and since immediate s u r g e r y can be lifesaving, a brief note in the literature is felt justified. The t e r m " s p o n t a n e o u s " is used with reservation since the etiology is totally obscure. Less t h a n 200 ease reports of patients with spontaneous r u p t u r e of tile esophagus have been recorded in the literature. The series reported b y Derbes and Mitchell 1 included a 2month-old child whose death resulted front a pylorie stenosis and in whom an esophageal r u p t u r e was demonstrated at autopsy. To our knowledge, this is the youngest patient previously repot"ted.
Ia
CASE REPORT
The i n f a n t was the sixth child born of a healthy 26-year-old Negro. The mother was seen twice during her p r e n a t a l course and the only complication was slight vaginal bleeding 1 month p r i o r to delivery. A t 6:17 a.z~. on J a n u a r y 14 a 6 pound, 14 ounce female i n f a n t was born spontaneously at term. Presentation was vertex, right occiput anterior, and the membranes r u p t u r e d just p r i o r to delivery. F r o m t h e D e p a r t m e n t s of S u r g e r y a n d Pediatrics a n d the Pediatric O u t - p a t i e n t Department, Mobile General Hospital. 207
The infant cried spontaneously and resuscitation was not required at any time. She was sent to the newborn infant n u r s e r y where slight i n s p i r a t o r y retractions were noted b y the nurse. At :10:30 A.M. the patient was first examined b y a staff physician and again slight retractions were noted. She was placed in humidified oxygen in an incubator and did f a i r l y well until the following day when the retractions became more severe. A t the end of 24 hours she was started on routine feedings and tetracycline b y mouth. Later in the second d a y a chest film was llq&d~.
H e r breathing" continued to be shallow and irregular; eyanosis was relieved b y administration of oxygen, and she required occasional stimulation with caffeine. E x a m i n a t i o n revealed decreased breath sounds over t h e right chest. Penicillin given intramuscularly was added to the t h e r a p y during the second night. The chest film was not seen due to a change in house officers until the following morning, the t h i r d d a y of life. E x a m i n a t i o n at this time revealed a seriously ill i n f a n t with obvious emphysema. She was quite cyanotic and respirations were shallow and rapid. B r e a t h sounds were diminished throughout the r i g h t chest. F i n e rNes were present posteriorly at the left base. The x-ray taken the previous d a y revealed t h a t the h e a r t and mediastinum were shifted to the left. There was increased radioluceney throughout the right hemithorax and scattered oval and linear densities
208
THE
JOURNAL
(Fig. 1). It was felt that these findings represented a congenital lobar emphysema or a large cyst. I m m e d i a t e surgery was felt mandatory. SURGICAL
TREATMENT
A routine right thoracotomy incision was made. Upon opening tile chest, a mixture of milk and mucus poured out. A f t e r removing' a p p r o x i m a t e l y
OF P E D I A T R I C S
This tube was left in place and the perforation was closed with interr u p t e d sutures of 6-0 silk. The pleura was brought over the closure as a second layer. The chest was closed with three tubes in place, one situated posteriorly n e a r the site of the perforation. The procedure was well tolerated and tile infant was a p p a r e n t l y greatly improved.
A,
/0"ig. 1 . - - A , P r e o p e r a t i v e
]~.
c h e s t film s h o w i n g t e n s i o n p n e u m o t h o r a x taken 8 days postoperatively.
200 c.c. of this material, the ateleetatic right l u n g was identified and rotated anteriorly. A 1 era. longitudinal p e r f o r a t i o n witl~, ragged and friable edges was discovered in the midportion of the esophagus on the right side. The patency of the esophagus was demonstrated by passing a polyvinyl catheter into the stomach.
on r i g h t .
B,
Chest
film
POSTOPERATIVE TREAT1V[ENT A chest x-ray on the first postoperative day showed only a slight amount of fluid in the right chest, with complete expansion of the right lung. The thoracotomy tubes were removed on the second postoperative day and an x-ray showed the chest to be clearing" at this time.
W I S E M A N E T AL. :
R U P T U R E OF E S O P H A G U S
Penicillin and oxytetracycline were used to combat infection. Glucose water was started b y Levin tube on the fourth day and intravenous fluids were discontinued on the fifth day. On the sixth postoperative day an evaporated milk formula was begun b y gastric tube, and the tube was removed on the seventh day. Weight gain occurred 12 days postoperatively and on the twenty-first day the i n f a n t was disc h a r g e d to be followed in the clinic. Contrast studies of the esophagus performed before discharge were entirely noITnal. The patient has been seen twice in the clinic, the most recent visit being at 21~ months of age, at which time she weighed 9 pounds, 51~ ounces. She was having no difficulties at that time. DISCUSSION
Considerable attention in the literature has been devoted to spontaneous r u p t u r e of the esophagus since the original description by Boerhaave in 1724. E a r l y recognition has become increasingly i m p o r t a n t since improvements in thoracic s u r g e r y have made survival possible. According to Derbes and MitehelP only 35 per cent of the patients survive for 24 hours if surgery is not p e r f o r m e d promptly. The characteristic clinical and roentgenologic features occurring in older children and adults have been adequately described. ~-* The usual pattern of violent vomiting, followed by intense substernal pain, and associated with subcutaneous or mediastinal emphysema were, of course, absent in this patient. I t h ~ been stated that if a p n e u m o t h o r a x or hydropneumothorax develops, subcutaneous emphysema is absent or late in appearance. Tension p n e u m o t h o r a x which has previously been reported in association with r u p t u r e of the esophagus '~ was an unusual feature in this patient.
209
A number of etiologie factors have been suggested. 1, a, s Forceful vomiting and other conditions which could increase intraesophageal pressure such as defecation, epilepsy, etc., have been precipitating factors. I n this patient, in whom there was no vomiting, none of these would seem applicable. In a newborn infant intubation or pressure resuseitation would seem to be the most likely cause of a r u p t u r e d esophagus. This child, however, breathed spontaneously and was not resuscitated in a n y fashion. All personnel concerned were diligently questioned immediately following surgery and it is certain tha.t intubation was not performed. I t is highly probable t h a t the catastrophic event occurred at or shortly a f t e r delivery since this b a b y was noted to have some r e s p i r a t o r y distress at a few hours of age. I t is possible t h a t an esophagus filled with amniotie fluid could be r u p t u r e d b y sudden extrathoracic pressure such as strong uterine contractions. Two p r e m a t u r e infants recently seen in this hospital were operated on for spontaneous r u p t u r e of the stomach. One of these was subsequently autopsied and found to have a congenital defect of the gastric museularis. 7 Theoretically, such a congenital anomaly could occur in the esophagus. Since this child survived and no sections were obtained, this is sheer speculation. One f u r t h e r unusual feature in this patient was the location of the perforation. I t has been demonstrated experimentally that the weakest section of the esophagus is just above the eardia on the left side. This has been confirmed clinically as the most likely spot for rupture. This lesion occurred on. the right side in the middle third
210
THE JOURNAL OF PEDIATRICS
of the esophagus. Subsequent contrast studies have demonstrated no stricture or other abnormality in this area. Immediate surgery with closure of the defect is the obvious treatment. E a r l y recognition is mandatory. Since our diagnosis was delayed, it is felt that calling attention to this possibility in a newborn infant may alert others to earlier diagnosis. The importance of early roentgenographic examination in any newborn infant with unexplained respiratory distress should be emphasized. SUMMARY
Idiopathic r u p t u r e of the esophagus with a tension pneumothorax in a newborn infant is reported. Immediate surgery with closure of the defect is the only possible therapy. Possible etiologic factors are briefly commented upon.
We wish to express our appreciation to Dr. Rex Perkins, Medical College, University of Alabama, for his comments and surgical advice. REFERENCES 1. Derbes, V. J , and ~ i t c h e E , R. E.: Rupture of the Esophagus~ Surgery 39: 688~ and 39: 865~ 1956. 2. Naclerio, E. A.: The " V " Sign in the Diagnosis of Spontaneous Rupture of the Esophagus (An E a r l y Roentgen Clue), Am. J. Surg. 93: 291, 1957. 3. Ware, G. W , Shnider, B. I., and Davis, E. tI.: Spontaneous Rupture of the Esophagus: Report of a Successful Case W i t h Cardiac A r r e s t a n d a Review of the Literature~ A. M. A. Arch. Surg. 65: 723, ]952. 4. Christoforides~ A., and Nelson~ S. W.: Spontaneous Rupture of the Esophagus W i t h Emphasis on the Roentgenologic Diagnosis, Am. J. Roentgenol. 78: 574, 1957. 5. Pembleton, W. E.: Rupture of the Esophagus Causing Tension Pneumothorax, Virginia M. Monthly 73: 331, ]946. 6. Kirby, C. D.: Spontaneous Rupture of the Esophagus, Am. J. !VI. Sc. 229: 199, 1955. 7. Meyer, J. L , I h Congenital Defect in Musculature of Stomach Resulting in Spontaneous Gastric P e r f o r a t i o n in Neon a t a l Perio'd: Report of Two Cases, J. PEDIAT. 51: 416, 1957.