Pediatric Radiology

Pediatr. Radiol. 8, 65-69 (1979)

9 by Springer-Verlag1979

Originals Traumatic Pharyngeal Pseuflodiverticulum in Neonates and Infants Two Case Reports and Review of the Literature J. L u c a y a 1, M. H e r r e r a 1, a n d S. Salcedo 2 Departments of 1Radiology and 2pediatrics, Clinica Infantil de la Seguridad Social, Barcelona, Spain

Abstract. T h i r t y o n e cases of p h a r y n g e a l p s e u d o d i v e r t i c u l u m h a v e b e e n r e p o r t e d in the l i t e r a t u r e ; t w e n t y n i n e were d i a g n o s e d d u r i n g the n e o n a t a l period. R e s p i r a t o r y distress, i n c r e a s e d oral secretions, difficulty with f e e d i n g a n d the impossibility of passing a n a s o g a s t r i c c a t h e t e r w e r e the m o s t c o m m o n s y m p t o m s a n d / o r signs. P n e u m o m e d i a s t i n u m , p n e u m o t h o r a x , cervical e m p h y s e m a a n d ectopic l o c a t i o n of a f e e d i n g catheter, a l o n e or in c o m b i n a t i o n , were i d e n t i f i e d in the chest r o e n t g e n o g r a m s of 16 patients. E s o p h a g o g r a p h y a n d / o r e n d o s c o p y were the diagnostic m e t h o d s of choice. T h e exact l o c a t i o n of the p e r f o r a t i o n was i d e n t i f i e d in 18 patients. Most of the p e r f o r a t i o n s were in e i t h e r the p o s t e r i o r p h a r y n g e a l wall or in the p y r i f o r m sinuses. T h e survival rate was as g o o d a m o n g s t the m e d i c a l l y t r e a t e d p a t i e n t s as in those w h o u n d e r w e n t surgery.

Key words: P h a r y n g e a l p e r f o r a t i o n - E s o p h a g e a l perforation - Tratmlatic pseudodiverticulum

Traumatic pharyngeal pseudodiverticulum (TPPD), s e c o n d a r y to p e r f o r a t i o n of e i t h e r the h y p o p h a r y n x or u p p e r esophagus, is a rare e n t i t y which was first r e p o r t e d b y E k l 6 f et al. i n 1969 [6]. T h e p u r p o s e of this p a p e r is to p r e s e n t two addit i o n a l cases of T P P D a n d the results of a review of the clinical a n d r o e n t g e n o l o g i c a l f e a t u r e s of the 31 cases which we have b e e n able to collect f r o m the l i t e r a t u r e [1, 2, 4 - 1 4 , 16, 17].

Case Reports Case 1. This boy, weighing 1370 Gms., was the product of a 30 week twin gestation and a breech delivery. The one minute Apgar score was one and he was subjected to some resuscitative manoeu-

vers. Shortly after birth he developed tachypnea, grunting respirations, nasal flaring, cyanosis and substernal retractions and was transferred to the Children's Hospital of Barcelona. The diagnosis on admission was idiopathic respiratory distress syndrome, confirmed by chest roentgenograms. An endotracheal tube was inserted and intermittent positive-pressure ventilation was begun. During intubation, copious bloody secretions were seen in his oropharynx. On the third day, a 5F polyvinyl nasogastric feeding tube was passed without apparent difficulty. A chest roentgenogram obtained a few hours later demonstrated that the distal tip of the tube was located in the retroperitoneal space (Fig. 1). The diagnosis of pharyngeal or esophageal perforation was then suspected. Water soluble contrast material was injected into the feeding catheter; it filled a channel which originated from the hypopharynx and extended across the diaphragm into the retroperitoneal space (Fig. 2). Contrast material was also noted to spill over into the esophagus and fill the stomach (Fig. 3). The catheter was retrieved and a nasogastric tube was introduced under direct vision. The patient was treated with antibiotics and intragastric feeds. On the seventh day he developed clinical signs of sepsis and disseminated intravascular coagulation and died two days later. A small perforation of the posterior pharyngeal wall, immediately above the cricopharyngeal muscle, was confirmed on autopsy. Case 2. This 3 month old boy underwent bilateral inguinal hernia repair at a rural hospital. Endotracheal intubation was reported to have been difficult. Shortly after surgery he developed respiratory difficulty and was transferred to the Children's Hospital of Barcelona. On physical examination there was severe respiratory difficulty with tachypnea, intercostal retractions, cyanosis and bilateral supraclavicular subcutaneous emphysema. Roentgenograms performed at the rural hospital showed right upper lobe atelectasis, pneumomediastinum, a small left sided pneumothorax and cervical emphysema. He was treated with assisted ventilation and improved rapidly. Twelve hours later the endotracheal tube was withdrawn. Forty eight hours after his admission he choked when attempting to feed by mouth and his respiratory difficulty worsened. A chest roentgenogram showed there was a prevertebral cervical water density mass with a small collection of air within it (Fig. 4). The trachea appeared slightly compressed and displaced anteriorly. An esophagogram was then performed. Both, the esophagus and a large oval structure located behind it were filled with contrast material (Fig. 5). At endoscopy a small perforation was identified in the posterior pharyngeal wall, inmediatly above the cricopharyngeus. The patient was treated with intragastric feeds

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J. Lucaya et al.: Traumatic Pharyngeal Pseudodiverticulum

Fig. 1, Patient 1. Newborn infant with respiratory distress syndrome. The distaI tip of the feeding catheter appears located in the retroperitoneal space Fig. 2. Patient 1. The injection of contrast material into the feeding tube fills a large channel which ends in the retroperitoneal space

Fig. 3. Patient 1. Injection of contrast material after partial withdrawal of the tube. The false channel crosses the diaphragm and runs behind the esophagus. Contrast material also filled the esophagus and the trachea

J. Lucaya et al.: Traumatic Pharyngeal Pseudodiverticulum

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Fig. 4. Patient 2. A large water density mass with a large collection of air within it may be seen in the mediastinum. There is compression and marked anterior displacement of the trachea. Patchy atelectasis may be identified in both upper lobes

Fig. S. Patient 2. Esophagogram. The middle third of the esophagus appears compressed and anteriorly displaced by a large contrast filled cavity. Both the upper and lower esophageal thirds have a normal size and configuration

antibiotics and surgical drainage of the TPPD. He made an uneventful recovery. Fifteen days later a repeated esophagogram showed no abnormality.

Discussion

T P P D in children were first reported in 1969 by Ekl6f et al. [6] who considered t h e m to be of traumatic origin. Our review and data support this view. Preceeding the onset of s y m p t o m s caused by T P P D , 27 of the 33 reviewed patients were known to have suffered some sort of instrumental injury to their oropharynx. Twenty four had been either intubated and/or subjected to vigorous aspiration of their oropharyngeal secretions. The remaining three patients [8, 14] had had digital manipulation of their o r o -

pharynx, aimed either to dislodge an impacted foreign body, or to r e m o v e thick secretions, or as used by the obstetrician while delivering the head of a breech presentation. In 1969, Girdany and co-workers [8] suggested that injury to the posterior pharyngeal wall m a y cause cricopharyngeal spasm and signs of high esophageal obstruction and that subsequent attempts to pass a nasogastric catheter m a y lead to perforation of the pharyngeal wall and produce the traumatic pharyngeal pseudodiverticulum. The observation by the same authors of two newborn infants with cricopharyngeal spasm without T P P D who were thought to have esophageal atresia because they salivated excesively, vomited feedings and in w h o m a nasogastric tube could not be passed, supported their former suggestion. However, later reports [11, 16] on patients

68 with TPPD who had never had symptoms and/or signs of upper esophageal obstruction raised some doubts on the certainty of Girdany's hypothesis. We believe that the role of cricopharyngeal spasm as a primary cause of the TPPD remains to be proved. Twenty nine patients, 15 of whom were full-term and 14 prematurely born infants, were diagnosed within the first nine days of life. The relatively high incidence of TPPD in premature infants may be related to the frequent use of nasogastnc mtubatlon in these patients. The remaining 4 patients were diagnosed between 1 and 18 months of age. In the oldest patient the TPPD was diagnosed following the retrieval of a foreign body which had become impacted in his oropharynx [14]. Respiratory distress, increased oral secretions and difficulty with feeding (choking, vomiting and/or cyanosis) were the most common symptoms. Nineteen patients presented with respiratory difficulty which, in 10, was attributable to their associated respiratory distress syndrome. Increased oral secretions, occasionally bloody or mucopurulent, and difficulty with feeding occurred in 17 and 10 patients, respectively. Inability to pass a feeding catheter into the stomach was the most common sign. The tube could not be advanced in 15 patients, most of whom were thought to have esophageal atresia. The roentgenographic features of the chest were specified in 22 patients. The examination was considered normal in only one of them [6]. Eleven patients presented pulmonary lesions, which were typical of hyaline membrane disease in nine and interpreted as aspiration pneumonia in 2 [4, 5]. Pneumomediastinum was identified in the films of 11 patients. [1, 7-10, 12, 14, 16, 17], 5 of whom also had cervical emphysema [7, 12, 16, 17]. Pneumothorax, either bilateral or right sided, was identified in 3 patients [11]. A cervical prevertebral soft tissue mass and/or ectopic air at the same level were seen in the films of 3 patients [5, 14]. Pneumopericardium [16] and retroperitoneal air [12] were each seen once. An aberrant position of the feeding catheter was seen in the roentgenograms of 8 of those 13 patients whose nasogastric intubation was thought to have been succesfully accomplished [11, 12, 16]. The tube was found to be in the right pleural space in 5 cases [11, 16], within the retroperitoneum in 2 [12] and in the pericardial cavity in one [16]. It should be pointed out that all those patients whose feeding catheters were found to have followed an anomalous course were premature infants with severe idiopathic respiratory distress syndrome, who never presented symptoms of high esophageal obstruction. As a consequence of this, and also because

J. Lucayaet al.: Traumatic PharyngealPseudodiverticulum feeding tubes had been easily passed, the diagnosis of their TPPD had not been suspected prior to obtaining the roentgenograms. An esophagogram was done in 28 patients. The pseudodiverticulum filled in 27. The borders of the anomalous channel were irregular in 15 and smooth in the remaining 12 patients. Its caliber was equal to that of a normal esophagus in 10, wider in 6 and narrower in 11. The distal border of the pseudodiverticulum was at the cervical level in 4, between T1 and T3 in 5, between T4 and T9 in 10, at the level of the diaphragm in 5 and below it in 3 patients. With the exception of one case the TPPD was never found to impinge nor to displace the trachea. Contrast medium overspilled into the tracheobronchial tree in 10 patients. Failure to retrieve the injected contrast material from the pseudodiverticulum occurred in 9 patients. In 21 of the 28 patients, some contrast material entered the esophagus which was always located anterior to the pseudodiverticulum. Lack of esophageal filling, as observed in 7 patients, was probably due to persistent cricopharyngeal spasm. It is precisely in such cases were the roentgenological features of the TPPD may simulate those of esophageal atresia. A repeated esophagogram, one week to four months after the initial one, was performed in 19 patients. It was normal in 17 of them. A small dimple at the site of the previous perforation was observed once [2]. Another patient was found to have mild cricopharyngeal spasm and some swallowing difficulties with occasional aspiration of barium into the tracheobronchial tree [8]. The exact location of the perforation was identified at endoscopy in 13 patients, during surgery in four and at autopsy once. Twelve were in the posterior pharyngeal wall, five in either of the pyriform sinuses and one in the cervical esophagus. In the remaining 13 patients, the perforation site could not be determined exactly, but the roentgenological studies suggested they were either pharyngeal or at the level of the cervical esophagus. Esophageal atresia, true pharyngeal diverticuli and esophageal duplication have to be considered in the differential diagnosis of TPPD. The location of the pseudodiverticular channel away from the trachea, the frequent irregularity of its contours, its usual long length and narrow diameter are important features. Once contrast medium has entered the channel it has remained there. And there are those abnormalities associated with perforation, namely pneumothorax, pneumomediastinum and cervical emphysema. Such are the radiological features of TPPD which will have to be looked for in order to differentiate it from esophageal atresia.

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J. Lucaya et al.: Traumatic Pharyngeal Pseudodiverticulum I n 1949, B r i n t n a l l a n d K r i d e l b a u g h [3] r e p o r t e d t w o p a t i e n t s with t r u e p h a r y n g e a l d i v e r t i c u l i w h o s e r o e n t g e n o l o g i c a l findings w e r e i n d i s t i n g u i s h a b l e f r o m t h o s e of T P P D . W h i l e t h e f o r m e r d o persist, t h e l a t t e r t e n d to r e g r e s s s p o n t a n e o u s l y as r e c o v e r y o c curs. T h e r e f o r e , f o l l o w - u p e x a m i n a t i o n s a r e m o s t h e l p f u l in d e t e r m i n i n g t h e e x a c t n a t u r e of the p r e viously encountered diverticulum. Fifteen patients were treated conservatively with n a s o g a s t r i c f e e d s a n d antibiotics, a n d 18 surgically. E i g h t p a t i e n t s d i e d b u t in o n l y t w o of t h e m [4, 8] t h e c a u s e of d e a t h c o u l d b e a t t r i b u t e d to t h e i r p h a r y n g e a l diverticuli. T h e survival r a t e in t h e g r o u p of cases w i t h o u t a s s o c i a t e d life t h r e a t e n i n g c o n d i t i o n s , was as g o o d a m o n g s t t h e c o n s e r v a t i v e l y t r e a t e d p a t i e n t s as in t h o s e w h o u n d e r w e n t surgery. E k l 6 f et al. [6] first a d v o c a t e d t h e c o n s e r v a t i v e t r e a t m e n t of p a t i e n t s with T P P D . O u r d a t a s u p p o r t s this o p i n i o n . A s L y n c h [13] p o i n t e d out, s u r g e r y s h o u l d b e r e s e r v e d to t r e a t t h e r a r e c o m p l i c a t i o n s of T P P D such as m e d i a s t i n i t i s , cervical a b s c e s s e s o r p n e u m o p e r i c a r d i u m with c a r d i a c t a m p o n a d e .

References 1. Armstrong, R.G., Lindberg, E. F., Stanford, W., Takamoto, R. M., Wolfe, R. D., Dietz, J. W.: Traumatic pseudodiverticulum of the esophagus in the newborn infant. Surgery 67, 844 (1970) 2. Astley, R., Roberts, K.D.: Intubation perforation of the esophagus in the newborn baby. Br. J. Radiol. 43, 219 (1970) 3. BrintnaU, E. S., Kridelbaugh, W. W.: Congenital diverticulum of the posterior hypopharynx simulating atresia of the esophagus. Ann. Surg. 131, 564 (1950) 4. Ducharme, J.C., Bertrand, R., Debie, J.: Perforation of the pharynx in the newborn. A condition mimicking esophageal atresia. Can. Med. Assoc. J. 104, 785 (1971) 5. Edison, B., Holinger, P.: Traumatic pharyngeal pseudodiverticulum in the newborn infant. J. Pediatr. 82, 483 (1973)

6. Ekl6f, O., Lohr, G., Okmian, L.: Submucosal perforation of the esophagus in the neonate. Acta Radiol. [Diagn.] (Stockh.) 8, 187 (1969) 7. Espinosa, H., Garcia de Paredes, C.: Traumatic perforation of the pharynx in a newborn baby. J. Pediatr. Surg. 9, 247 (1974) 8. Girdany, B. R., Sieber, W. K., Osman, M. Z.: Traumatic pseudodiverticulum of the pharynx in newborn infants. N. Engl. J. Med. 280, 237 (1969) 9. Gwinn, J. L., Lee, F. A.: Radiological case of the month. Pseudodiverticulum of the pharynx. Am. J. Dis. Child 121, 329 (1971) 10. Heller, R.M., Kirchner, S.G., O'Neill, J.A.: Perforation of the pharynx in the newborn: A near look alike for esophageal atresia. Am. J. Roentgenol. 129, 335 (1977) 11. Kassner, E. G., Baumstark, A., Balsam, D., Haller, J. O.: Passage of feeding catheters into the pleural space: A radiographic sign of trauma to the pharynx and esophagus in the newborn. Am. J. Roentgenol. 128, 19 (1977) 12. Lee, S. B., Kuhn, J. P.: Esophageal perforation in the neonate. Am. J. Dis. Child. 130, 325 (1976) 13. Lynch, F. P., Coran, A. G., Seymour, R. C., Lee, F. A.: Traumatic esophageal pseudodiverticula in the newborn. J. Pediatr. Surg. 9, 675 (1974) 14. Osman, M. Z., Girdany, B. R.: Traumatic pseudodiverticulum of the pharynx in infants and children. Ann. Radiol. (Paris) 16, 143 (1973) 15. Sidaway, M.: Duplication of the esophagus. Ann. Radiol. (Paris) 7, 400 (1964) 16. Toulokian, R.J., Beardsley, G.P., Ablow, R.C., Effmann, E.L.: Traumatic perforation of the pharynx in the newborn. Pediatrics 59, 1019 (1977) 17. Wells, S.D., Leonidas, J.C., Conkle, D., Holder, T.M., Amoury, R. A., Ashcraft, K. W.: Traumatic prevertebral pharyngoesophageal pseudodiverticulum in the newborn infant. J. Pediatr. Surg. 9, 217 (1974)

Date of final acceptance: October 10, 1978 Dr. Javier Lucaya Clinica Infantil de la Seguridad Social P. Valle de Hebr6n Barcelona 32 Spain