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Congenital diverticulum of the pharynx simulating esophageal atresia
Congenital Diverticulum of the Pharynx Simulating Esophageal Atresia By A. MacKellar and J. C. Kennedy
I
T IS REASONABLE to make a presumptive diagnosis of esophageal atresia if an infant, shortly after birth, is unable to swallow secretions. There are, however, some less common lesions that may present similar signs, and these have been reported from time to time in the literature. In 19.~0 Brintnall and Kridelbaugh’ described two patients with congenital diverticula of the hypopharynx; in 1969 Girdany et a1.2 reported two infants with traumatic pseudodiverticula of the pharynx and Gwinn and Lee,3 in 1971, a further patient with a similar lesion. Eklof et a1.,4 in 1969, described three infants with submucosal perforation of the esophagus. All these patients presented in the newborn period with signs suggestive of esophageal atresia. CASE REPORT
A female was born at II :15 p.m. on July 5, 1971, by breech extraction following a normal pregnancy. Five minutes after birth the Apgar rating was 8. Two hours later excessive amounts of mucus were present in the mouth and pharynx and moist sounds were audible over both lungs. An unsuccessful attempt was made to pass a catheter through the mouth to the stomach, and the baby was transferred to the Princess Margaret Hospital for Children with a provisional diagnosis of esophageal atresia. The infant was in good condition and weighed 3.12 kg. Regular oropharyngeal suction prevented the accumulation of secretions. Radiological examination of the infant, after passage of a radioopaque catheter through the mouth, showed that the catheter was arrested at the level of the seventh thoracic vertebra, which was considerably lower than is usual in esophageal atresia (Fig. IA). For this reason a smaller catheter was passed through the nose and it continued without obstruction into the stomach. Barium was iniected as this catheter was removed and the esophagus appeared normal (Fig. 1B). The larger catheter was again passed through the mouth and entered a pouch situated behind the normal esophagus, the lower end of which was clearly seen opposite the body of the seventh thoracic vertebra (Fig. IC). Examination of the barium in the stomach of the infant showed the characteristic indentation of the antrum and elongation of the pyloric canal consistent with congenital pyloric stenosis. After 30 min no contrast medium had entered the duodenal loop, which was, therefore, not studied (Fig. ID). Small amounts of gas were present in the small bowel. A cinC film demonstrated the origins and radiological anatomy of the normal esophagus, the abnormal tube, and the absence of any fistulous communication with the bronchial tree. Examination of the pharynx confirmed the presence of two openings, the abnormal one lying above, to the left, and posterior to the normal esophageal opening. There was no evidence of trauma around the abnormal opening. Operation was performed 16 hr after birth. After induction of anesthesia and endotracheal intubation, catheters were introduced through the nose into the normal esophagus and through the mouth into the abnormal pharyngeal opening. Right lateral transpleural thoracotomy gave excellent exposure. Some From the Departments of Surgery and Radiology, Princess Margaret Hospital for Ckifdren, Perth, Western Australia. A. MacKellar, M.B.Ch.B., F.R.C.S.E., F.R.A.C.S.: Senior Surgeon, Department of Surgery, Princess Margaret Hospital for Children, Perth, Western Australia. J. C Kennedy, M.B.Ch.B., M.C.R.A., F.F.R.: Radiologist in Charge, Department of Radiology, Princess Margaret Hospital for Children, Perth, Western Australia. 408
Journal
of Pediatric
Surgery,
Vol. 7. No. 4 (August-September),
1972
CONGENITAL DIVERTICULUM OF PHARYNX
d Fig. 1. Radiographs of infant. (A) Catheter passed through mouth arrested at level of seventh thoracic vertebra. (B) Normal esophagus after injection of barium via the catheter passed through the nose. (C) Filling with barium of the normal esophagus and of abnormal tube. (D) Features suggestive of congenital hypertrophic pyloric stenosis.
MAC KELLAR AND KENNEDY
410
edema of the mediastinum was already present with transudation from an abnormal tube-like structure lying 0.5 cm behind and to the left of the esophagus and completely separate from it. This tube-like structure was gently dissected out and a thin strand of tissue passing down to the diaphragm from the lower end was divided. The dissection was carried upward as far as the root of the neck and the thoracic wound was temporarily closed. The tube was then approached from the left side of the neck by division of the sternomastoid and omohyoid muscles and the inferior thyroid artery. The main vessels in the neck and the thyroid gland were retracted forward and dissection of the tube-like structure was continued. At this stage it proved impossible to retain the structure intact because of the friable nature of its wall, and it was removed piecemeal in the upper neck. The opening between the neck and thorax was closed with interrupted catgut sutures. The muscles were repaired and a drain was left in the lateral wall of the pharynx. Both wounds were then closed, an underwater seal drain being left in the right pleural cavity. The baby’s general condition did not allow further operative procedure at this stage. During the next 48 hr intravenous therapy was continued. Methicillin and kanamycin were given to control infection and the stomach was kept empty by nasogastric suction. Intermittent positive pressure ventilation through a portex nasotracheal tube was necessary. TWO days later, at a second operation, the abdomen was opened through a right upper transverse incision. There was a small effusion in the peritoneal cavity and some edema in the region of the lesser sac, but no evidence of the suspected pyloric stenosis. However, a malrotation of the small intestine was present, with bands passing across the duodenum from the right paracolic gutter. These bands were divided, a gastrostomy established, and a feeding tube passed through the pylorus into the third part of the duodenum. As it had proved difficult to control the collection of air in the right pleural cavity with the intercostal catheter, the wound of the neck was reopened and the opening between the neck and thorax was firmly packed with ribbon gauze soaked in parafin. A tube drain was then reinserted in the left lateral wall of the pharynx, no further attempt being made to close that opening. The neck wound was then sutured. The infant tolerated the second procedure well. After 36 hr, feeding through the duodenostomy tube was begun and all feeds were absorbed. The baby required intermittent positive pressure ventilation through a nasotracheal tube for 4 days, gradually tolerating longer periods without artificial ventilation, and the tube was finally removed after 6 days. The lateral pharyngostomy drain was shortened daily from the fourth day until it came out on the eighth day. Discharge from the wound then gradually ceased. The pack between the neck and thorax was also removed gradually between the eighth and 15th days. The intercostal catheter was then taken out and oral feeding commenced. One week later the gastrostomy and enterostomy tubes were removed. All feeds were then taken without difficulty by mouth. A barium swallow showed normal anatomy of the pharynx and some reduction of peristaltic activity of the lower esophagus. Gastroesophageal reflux was noted on several occasions but the stomach appeared normal and emptied without delay. The baby was discharged from the hospital on July 30 weighing 3.65 kg, and when last seen in the outpatient department on December 30, 1971, weighed 6.8 kg and was thriving. Microscopic examination of the tissue removed at operation showed compressed and distorted striated muscle, fat, connective tissue, and moderate-sized vessels in the wall of the structure, which was lined by inflamed epithelium. It was difficult to be certain of the nature of this epithelium owing to the necrosis caused by the diathermy dissection of the tissues. The striated muscle bundles were not arranged in coats as is seen in the upper esophagus and there was no other suggestion of normal muscle coats. The appearances were those of a very thin tube of mucous membrane surrounded by connective tissue with some blood vessels and a few striated muscle bundles. DISCUSSION The abnormality found in this baby is similar to that first described by Brintnall and Kridelbaugh in 19.50.’ Their two patients were seen at a late stage and succumbed to purulent mediastinitis. One of these children also had congenital hypertrophic pyloric stenosis.
CONGENITAL DIVERTICULUM OF PHARYNX
411
A somewhat similar abnormality is said to have been produced by the obstetrician’s finger in the baby’s mouth during the course of breech extraction. This lesion is a traumatic pseudodiverticulum and was reported by Girdany
et a1.2 and by Gwinn
evidence
suggestive
of trauma
and Lee.3 Examination to the posterior
of their patients
pharyngeal
wall. There
revealed was no
sign of damage to the pharyngeal wall of our patient and the length of the sac and its well-defined outline both at radiological examination and at operation so soon after birth made this etiology unlikely. The sac was completely separate from the normal esophagus and did not, therefore, conform to the criteria laid down for a duplication of the esophagus. There was no damage to the esophageal mucosa as would have been expected if this lesion had been caused by a submucosal perforation of the esophagus. The abnormal opening was situated in the pharynx just to the left of the midline above the cricopharyngeus muscle. Lateral pharyngeal diverticula of congenital origin represent remnants of the second, third, or fourth branchial pouches. The site of the internal opening and the position of this diverticulum suggest that it may have arisen from the fourth branchial pouch. The presence of mediastinal edema 24 hr after birth in this patient indicates how rapidly mediastinitis may develop as a result of transudation through the thin wall of the diverticulum. Early recognition and surgical correction of this condition are essential, therefore, if the infant is to survive. SUMMARY
Inability of a newborn infant to swallow secretions is usually due to esophageal atresia, but there are other, less common causes. Our patient suffered from a congenital diverticulum of the pharynx, demonstrated by radiography and at operation, and had an associated intestinal malrotation. She was successfully managed by a two-stage procedure with preliminary excision of the lesion from the chest and neck and later correction of the intestinal obstruction. REFERENCES 1. Brintnall, E. S., and Kridelbaugh, W. W. : Congenital diverticulum of the posterior hypopharynx simulating atresia of the eso-
3. Gwinn, J. L., and Lee, F. A.: Radiological case of the month: Pseudodiverticulum of the pharynx. Amer. J. Dis. Child.
phagus. Ann. Surg. 131:564, 1950. 2. Girdany, B. R., Sieber, W. K., and Osman, M. Z. : Traumatic pseudodiverticulum of the pharynx in newborn infants. New Eng. J. Med. 280:237, 1969.
121:329, 1971. 4. Eklof, O., Lohr, G., and Okmian, L.: Submucosal perforation of the esophagus in the neonate. Acta Radiol. 8:193, 1969.
I
T IS REASONABLE to make a presumptive diagnosis of esophageal atresia if an infant, shortly after birth, is unable to swallow secretions. There are, however, some less common lesions that may present similar signs, and these have been reported from time to time in the literature. In 19.~0 Brintnall and Kridelbaugh’ described two patients with congenital diverticula of the hypopharynx; in 1969 Girdany et a1.2 reported two infants with traumatic pseudodiverticula of the pharynx and Gwinn and Lee,3 in 1971, a further patient with a similar lesion. Eklof et a1.,4 in 1969, described three infants with submucosal perforation of the esophagus. All these patients presented in the newborn period with signs suggestive of esophageal atresia. CASE REPORT
A female was born at II :15 p.m. on July 5, 1971, by breech extraction following a normal pregnancy. Five minutes after birth the Apgar rating was 8. Two hours later excessive amounts of mucus were present in the mouth and pharynx and moist sounds were audible over both lungs. An unsuccessful attempt was made to pass a catheter through the mouth to the stomach, and the baby was transferred to the Princess Margaret Hospital for Children with a provisional diagnosis of esophageal atresia. The infant was in good condition and weighed 3.12 kg. Regular oropharyngeal suction prevented the accumulation of secretions. Radiological examination of the infant, after passage of a radioopaque catheter through the mouth, showed that the catheter was arrested at the level of the seventh thoracic vertebra, which was considerably lower than is usual in esophageal atresia (Fig. IA). For this reason a smaller catheter was passed through the nose and it continued without obstruction into the stomach. Barium was iniected as this catheter was removed and the esophagus appeared normal (Fig. 1B). The larger catheter was again passed through the mouth and entered a pouch situated behind the normal esophagus, the lower end of which was clearly seen opposite the body of the seventh thoracic vertebra (Fig. IC). Examination of the barium in the stomach of the infant showed the characteristic indentation of the antrum and elongation of the pyloric canal consistent with congenital pyloric stenosis. After 30 min no contrast medium had entered the duodenal loop, which was, therefore, not studied (Fig. ID). Small amounts of gas were present in the small bowel. A cinC film demonstrated the origins and radiological anatomy of the normal esophagus, the abnormal tube, and the absence of any fistulous communication with the bronchial tree. Examination of the pharynx confirmed the presence of two openings, the abnormal one lying above, to the left, and posterior to the normal esophageal opening. There was no evidence of trauma around the abnormal opening. Operation was performed 16 hr after birth. After induction of anesthesia and endotracheal intubation, catheters were introduced through the nose into the normal esophagus and through the mouth into the abnormal pharyngeal opening. Right lateral transpleural thoracotomy gave excellent exposure. Some From the Departments of Surgery and Radiology, Princess Margaret Hospital for Ckifdren, Perth, Western Australia. A. MacKellar, M.B.Ch.B., F.R.C.S.E., F.R.A.C.S.: Senior Surgeon, Department of Surgery, Princess Margaret Hospital for Children, Perth, Western Australia. J. C Kennedy, M.B.Ch.B., M.C.R.A., F.F.R.: Radiologist in Charge, Department of Radiology, Princess Margaret Hospital for Children, Perth, Western Australia. 408
Journal
of Pediatric
Surgery,
Vol. 7. No. 4 (August-September),
1972
CONGENITAL DIVERTICULUM OF PHARYNX
d Fig. 1. Radiographs of infant. (A) Catheter passed through mouth arrested at level of seventh thoracic vertebra. (B) Normal esophagus after injection of barium via the catheter passed through the nose. (C) Filling with barium of the normal esophagus and of abnormal tube. (D) Features suggestive of congenital hypertrophic pyloric stenosis.
MAC KELLAR AND KENNEDY
410
edema of the mediastinum was already present with transudation from an abnormal tube-like structure lying 0.5 cm behind and to the left of the esophagus and completely separate from it. This tube-like structure was gently dissected out and a thin strand of tissue passing down to the diaphragm from the lower end was divided. The dissection was carried upward as far as the root of the neck and the thoracic wound was temporarily closed. The tube was then approached from the left side of the neck by division of the sternomastoid and omohyoid muscles and the inferior thyroid artery. The main vessels in the neck and the thyroid gland were retracted forward and dissection of the tube-like structure was continued. At this stage it proved impossible to retain the structure intact because of the friable nature of its wall, and it was removed piecemeal in the upper neck. The opening between the neck and thorax was closed with interrupted catgut sutures. The muscles were repaired and a drain was left in the lateral wall of the pharynx. Both wounds were then closed, an underwater seal drain being left in the right pleural cavity. The baby’s general condition did not allow further operative procedure at this stage. During the next 48 hr intravenous therapy was continued. Methicillin and kanamycin were given to control infection and the stomach was kept empty by nasogastric suction. Intermittent positive pressure ventilation through a portex nasotracheal tube was necessary. TWO days later, at a second operation, the abdomen was opened through a right upper transverse incision. There was a small effusion in the peritoneal cavity and some edema in the region of the lesser sac, but no evidence of the suspected pyloric stenosis. However, a malrotation of the small intestine was present, with bands passing across the duodenum from the right paracolic gutter. These bands were divided, a gastrostomy established, and a feeding tube passed through the pylorus into the third part of the duodenum. As it had proved difficult to control the collection of air in the right pleural cavity with the intercostal catheter, the wound of the neck was reopened and the opening between the neck and thorax was firmly packed with ribbon gauze soaked in parafin. A tube drain was then reinserted in the left lateral wall of the pharynx, no further attempt being made to close that opening. The neck wound was then sutured. The infant tolerated the second procedure well. After 36 hr, feeding through the duodenostomy tube was begun and all feeds were absorbed. The baby required intermittent positive pressure ventilation through a nasotracheal tube for 4 days, gradually tolerating longer periods without artificial ventilation, and the tube was finally removed after 6 days. The lateral pharyngostomy drain was shortened daily from the fourth day until it came out on the eighth day. Discharge from the wound then gradually ceased. The pack between the neck and thorax was also removed gradually between the eighth and 15th days. The intercostal catheter was then taken out and oral feeding commenced. One week later the gastrostomy and enterostomy tubes were removed. All feeds were then taken without difficulty by mouth. A barium swallow showed normal anatomy of the pharynx and some reduction of peristaltic activity of the lower esophagus. Gastroesophageal reflux was noted on several occasions but the stomach appeared normal and emptied without delay. The baby was discharged from the hospital on July 30 weighing 3.65 kg, and when last seen in the outpatient department on December 30, 1971, weighed 6.8 kg and was thriving. Microscopic examination of the tissue removed at operation showed compressed and distorted striated muscle, fat, connective tissue, and moderate-sized vessels in the wall of the structure, which was lined by inflamed epithelium. It was difficult to be certain of the nature of this epithelium owing to the necrosis caused by the diathermy dissection of the tissues. The striated muscle bundles were not arranged in coats as is seen in the upper esophagus and there was no other suggestion of normal muscle coats. The appearances were those of a very thin tube of mucous membrane surrounded by connective tissue with some blood vessels and a few striated muscle bundles. DISCUSSION The abnormality found in this baby is similar to that first described by Brintnall and Kridelbaugh in 19.50.’ Their two patients were seen at a late stage and succumbed to purulent mediastinitis. One of these children also had congenital hypertrophic pyloric stenosis.
CONGENITAL DIVERTICULUM OF PHARYNX
411
A somewhat similar abnormality is said to have been produced by the obstetrician’s finger in the baby’s mouth during the course of breech extraction. This lesion is a traumatic pseudodiverticulum and was reported by Girdany
et a1.2 and by Gwinn
evidence
suggestive
of trauma
and Lee.3 Examination to the posterior
of their patients
pharyngeal
wall. There
revealed was no
sign of damage to the pharyngeal wall of our patient and the length of the sac and its well-defined outline both at radiological examination and at operation so soon after birth made this etiology unlikely. The sac was completely separate from the normal esophagus and did not, therefore, conform to the criteria laid down for a duplication of the esophagus. There was no damage to the esophageal mucosa as would have been expected if this lesion had been caused by a submucosal perforation of the esophagus. The abnormal opening was situated in the pharynx just to the left of the midline above the cricopharyngeus muscle. Lateral pharyngeal diverticula of congenital origin represent remnants of the second, third, or fourth branchial pouches. The site of the internal opening and the position of this diverticulum suggest that it may have arisen from the fourth branchial pouch. The presence of mediastinal edema 24 hr after birth in this patient indicates how rapidly mediastinitis may develop as a result of transudation through the thin wall of the diverticulum. Early recognition and surgical correction of this condition are essential, therefore, if the infant is to survive. SUMMARY
Inability of a newborn infant to swallow secretions is usually due to esophageal atresia, but there are other, less common causes. Our patient suffered from a congenital diverticulum of the pharynx, demonstrated by radiography and at operation, and had an associated intestinal malrotation. She was successfully managed by a two-stage procedure with preliminary excision of the lesion from the chest and neck and later correction of the intestinal obstruction. REFERENCES 1. Brintnall, E. S., and Kridelbaugh, W. W. : Congenital diverticulum of the posterior hypopharynx simulating atresia of the eso-
3. Gwinn, J. L., and Lee, F. A.: Radiological case of the month: Pseudodiverticulum of the pharynx. Amer. J. Dis. Child.
phagus. Ann. Surg. 131:564, 1950. 2. Girdany, B. R., Sieber, W. K., and Osman, M. Z. : Traumatic pseudodiverticulum of the pharynx in newborn infants. New Eng. J. Med. 280:237, 1969.
121:329, 1971. 4. Eklof, O., Lohr, G., and Okmian, L.: Submucosal perforation of the esophagus in the neonate. Acta Radiol. 8:193, 1969.