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Case report: Complete laryngotracheoesophageal cleft: CT diagnosis and associated abnormalities
ClinicalRadiology(1990) 41, 437 438
Case Report: Complete Laryngotracheoesophageal Cleft: CT Diagnosis and Associated Abnormalities A. G. W I L K I N S O N , S. M A C K E N Z I E a n d G. M. A. H E N D R Y
Department of Radiology, Royal Hospital for Sick Children, Edinburgh
A case of complete laryngotracheoesophageal cleft is described in a male infant in which bronchoscopy could not be performed due to a narrow tracheal orifice. CT scan of the thorax demonstrated a common tracheal and oesophageal lumen extending inferiorly to the carina, with hypoplasia of the right lung and cardiac dextroposition. Contrast studies demonstrated a hypoplastic intrathoracic stomach. Chromosome analysis revealed satellites on the Y chromosome with karyotype 46XYqs.
T r a c h e o e s o p h a g e a l cleft is a rare congenital a b n o r m a l i t y which has been classified (Evans, 1985) into type I if the defect is confined to the supraglottic larynx, type II if the cricoid l a m i n a is involved a n d type I I I if the defect extends into the thoracic trachea. Complete l a r y n g o t r a cheoesophageal cleft extending inferiorly to the c a r i n a is the least c o m m o n form o f the a b n o r m a l i t y with o n l y 14 cases in the literature. Diagnosis is usually suspected o n clinical findings of cyanosis from birth, c h o k i n g o n feeding a n d aspiration p n e u m o n i a . There is often a history of p o l y h y d r a m n i o s . C o n t r a s t e x a m i n a t i o n shows free spillage o f c o n t r a s t from oesophagus to trachea a n d c o n f i r m a t i o n o f the diagnosis is usually by endoscopy. W e describe a case in which b r o n c h o s c o p y was impossible because o f a n a r r o w tracheal orifice a n d in which c o n f i r m a t i o n of the diagnosis was m a d e by c o m p u t e d tomography.
CT scan of the thorax was performed to demonstrate the lower limit of the cleft (Fig. 3). A common tracheoesophageal channel containing both the endotracheal and nasogastric tubes was identified extending down to the carina. A normal left main bronchus was identified,but the right bronchi were hypoplastic with hypoplasia and consolidation of the lung. Ultrasound examination of the abdomen confirmed absence of an intra-abdominal stomach, but no other abnormality of the abdominal viscera was seen. As a preliminary to corrective surgery, intubation of the left main bronchus was attempted without success. It was felt that the abnormalities were incompatible with life and corrective surgery was not undertaken. The child deteriorated and died of respiratory failure at the age of 11 days. Permission for autopsy was refused. Chromosomal analysis revealed the presence of satellites on the long arm of the Y chromosome; karyotype was 46XYqs.
DISCUSSION The diagnosis o f complete laryngo-tracheoesophageal cleft m a y be difficult to confirm even if it is clinically suspected. C o n t r a s t e x a m i n a t i o n shows free spillage of contrast from the oesophagus to the trachea b u t often fails to d e m o n s t r a t e the extent o f the cleft. E n d o s c o p y m a y fail to reveal the a b n o r m a l i t y because the edges of the cleft tend to a p p r o x i m a t e ; posterior pressure o n the b r o n c h o s c o p e ( D o n o h o e a n d Gee, 1984) or oesophagoscopy, with a n e n d o t r a c h e a l t u b e in situ to separate the edges of the cleft ( C o t t o n a n d Schreiber, 1981), have been suggested to i m p r o v e the diagnostic yield o f endoscopy. N o case has previously been reported in which c o m p u t e d t o m o g r a p h y has been used to confirm the diagnosis a n d d e m o n s t r a t e the extent o f the cleft.
CASE REPORT A male infant was transferred to the Royal Hospital for Sick Children 12 h after birth for investigation of suspected oesophageal atresia and tracheoesophageal fistula. He had been born by Caesarean section for transverse lie at 36 weeks gestation. Birth weight was 2.09 kg. Pregnancy had been complicated by polyhydramnios and an ultrasound scan at 32 weeks had shown dextroposition of the fetal heart with failure to visualise the fetal stomach. The child was cyanosed at birth, but improved with artificial ventilation. At intubation the larynx was thought to be swollen but no other abnormality was detected. A nasogastric tube could not be passed below the diaphragm. The chest radiograph (Fig. 1) showed opacifieation of the right hemithorax with cardiac dextroposition and hypoplasia of the right lung. No stomach bubble was seen below the diaphragm and the tip of the nasogastric tube lay above the diaphragm. No cardiac abnormality was detected at echocardiography. The baby was extubated and was able to breathe spontaneously. At reintubation, prior to contrast studies, a laryngeal cleft was noted. Iso-osmolar water soluble contrast injected into the nasogastric tube spilled freely into the trachea at all levels (Fig. 2). The stomach was hypoplastic and intrathoracic with a tortuous duodenal loop. Laryngoscopy showed complete cleft of the posterior larynx with wide separation of the arytenoids and continuation of the cleft into the trachea. The tracheal orifice was narrow and could not be entered with a 3 mm bronchoseope making it impossibleto determine the lower limit of the deft. Correspondence to: Dr G. M. A. Hendry, Department of Radiology, Royal Hospital for SickChildren, SciennesRoad, Edinburgh EH9 1LF.
Fig. 1 - Chest radiograph showing cardiac dexti'oposition and hypoplasia of the right lung.
438
CLINICAL RADIOLOGY
(a~
(b) Fig. 3 - (a) CT scan at the level of the first thoracic vertebra showing common tracheoeosophageal lumen containing both the nasogastric and endotracheal tubes. (b) CT scan at the level of the carina showing common tracheoesophageal lumen and hypoplastic right lung. Note nasogastric tube within lumen.
Fig. 2 - Contrast study showing free spillage of contrast from oesophagus to trachea.
A s s o c i a t e d c o n g e n i t a l a b n o r m a l i t i e s are c o m m o n : dext r o c a r d i a a n d m a l r o t a t i o n o f the c a e c u m was r e p o r t e d b y Pettit et al. (1979) a n d E v a n s (1985) described three cases o f which, one h a d a b s e n c e o f tracheal cartilage with b r o n c h i arising f r o m the o e s o p h a g u s a n d two h a d b i f u r c a t i o n o f the t r a c h e a in the neck. O n e o f the nine cases o f c o m p l e t e t r a c h e o e s o p h a g e a l cleft reviewed b y B u r r o u g h s a n d L e a p e (1974) h a d multiple c o n g e n i t a l abnormalities. O n l y one case o f c o m p l e t e l a r y n g o - t r a c h e o e s o p h a g e a l cleft has been r e p o r t e d ( D o n o h o e a n d Gee, 1984) in which there has been surgical r e p a i r a n d survival b e y o n d the i m m e d i a t e p o s t - o p e r a t i v e period. A Y - s h a p e d e n d o t r a c h e o b r o n c h i a l tube was m a d e to p r o t e c t the a i r w a y a n d a cuff o f o e s o p h a g u s was used to c o m p l e t e the t r a c h e a via t h o r a c i c a n d cervical a p p r o a c h e s .
C o m p u t e d t o m o g r a p h y is a useful m o d a l i t y for diagnosing cases such as this where e n d o s c o p y is difficult b e c a u s e o f o t h e r c o n g e n i t a l a n o m a l i e s o f the b r o n c h i a l tree. E a r l y accurate diagnosis is essential for successful m a n a g e m e n t , b u t in this case m u l t i p l e a n o m a l i e s were i n c o m p a t i b l e with life.
REFERENCES
Burroughs, N & Leape, LL (1974). Laryngotracheoesophageal deft: report of a case successfully treated and review of the literature. Pediatrics, 53, 516-222. Cotton, RT & Schreiber, JT (1981). Management of laryngotracheosophageal cleft. Annals of Otology, Rhinology and Laryngology, 90, 401-405. Donohoe, PK & Gee, PE (1984). Complete laryngotracheoesophageal cleft: management and repair. Journal of Paediatric Surgery, 19, 143-148. Evans, JNG (1985). Management of the cleft larynx and tracheoesophageal clefts. Annals of Otology, Rhinology and Laryngology, 94, 627-630. Pettit, PN Jnr, Butcher, RB, Bethea, MC & King, TT (1979). Surgical correction of complete tracheoesophageal cleft. Laryngoscope, 89, 804 811.
Case Report: Complete Laryngotracheoesophageal Cleft: CT Diagnosis and Associated Abnormalities A. G. W I L K I N S O N , S. M A C K E N Z I E a n d G. M. A. H E N D R Y
Department of Radiology, Royal Hospital for Sick Children, Edinburgh
A case of complete laryngotracheoesophageal cleft is described in a male infant in which bronchoscopy could not be performed due to a narrow tracheal orifice. CT scan of the thorax demonstrated a common tracheal and oesophageal lumen extending inferiorly to the carina, with hypoplasia of the right lung and cardiac dextroposition. Contrast studies demonstrated a hypoplastic intrathoracic stomach. Chromosome analysis revealed satellites on the Y chromosome with karyotype 46XYqs.
T r a c h e o e s o p h a g e a l cleft is a rare congenital a b n o r m a l i t y which has been classified (Evans, 1985) into type I if the defect is confined to the supraglottic larynx, type II if the cricoid l a m i n a is involved a n d type I I I if the defect extends into the thoracic trachea. Complete l a r y n g o t r a cheoesophageal cleft extending inferiorly to the c a r i n a is the least c o m m o n form o f the a b n o r m a l i t y with o n l y 14 cases in the literature. Diagnosis is usually suspected o n clinical findings of cyanosis from birth, c h o k i n g o n feeding a n d aspiration p n e u m o n i a . There is often a history of p o l y h y d r a m n i o s . C o n t r a s t e x a m i n a t i o n shows free spillage o f c o n t r a s t from oesophagus to trachea a n d c o n f i r m a t i o n o f the diagnosis is usually by endoscopy. W e describe a case in which b r o n c h o s c o p y was impossible because o f a n a r r o w tracheal orifice a n d in which c o n f i r m a t i o n of the diagnosis was m a d e by c o m p u t e d tomography.
CT scan of the thorax was performed to demonstrate the lower limit of the cleft (Fig. 3). A common tracheoesophageal channel containing both the endotracheal and nasogastric tubes was identified extending down to the carina. A normal left main bronchus was identified,but the right bronchi were hypoplastic with hypoplasia and consolidation of the lung. Ultrasound examination of the abdomen confirmed absence of an intra-abdominal stomach, but no other abnormality of the abdominal viscera was seen. As a preliminary to corrective surgery, intubation of the left main bronchus was attempted without success. It was felt that the abnormalities were incompatible with life and corrective surgery was not undertaken. The child deteriorated and died of respiratory failure at the age of 11 days. Permission for autopsy was refused. Chromosomal analysis revealed the presence of satellites on the long arm of the Y chromosome; karyotype was 46XYqs.
DISCUSSION The diagnosis o f complete laryngo-tracheoesophageal cleft m a y be difficult to confirm even if it is clinically suspected. C o n t r a s t e x a m i n a t i o n shows free spillage of contrast from the oesophagus to the trachea b u t often fails to d e m o n s t r a t e the extent o f the cleft. E n d o s c o p y m a y fail to reveal the a b n o r m a l i t y because the edges of the cleft tend to a p p r o x i m a t e ; posterior pressure o n the b r o n c h o s c o p e ( D o n o h o e a n d Gee, 1984) or oesophagoscopy, with a n e n d o t r a c h e a l t u b e in situ to separate the edges of the cleft ( C o t t o n a n d Schreiber, 1981), have been suggested to i m p r o v e the diagnostic yield o f endoscopy. N o case has previously been reported in which c o m p u t e d t o m o g r a p h y has been used to confirm the diagnosis a n d d e m o n s t r a t e the extent o f the cleft.
CASE REPORT A male infant was transferred to the Royal Hospital for Sick Children 12 h after birth for investigation of suspected oesophageal atresia and tracheoesophageal fistula. He had been born by Caesarean section for transverse lie at 36 weeks gestation. Birth weight was 2.09 kg. Pregnancy had been complicated by polyhydramnios and an ultrasound scan at 32 weeks had shown dextroposition of the fetal heart with failure to visualise the fetal stomach. The child was cyanosed at birth, but improved with artificial ventilation. At intubation the larynx was thought to be swollen but no other abnormality was detected. A nasogastric tube could not be passed below the diaphragm. The chest radiograph (Fig. 1) showed opacifieation of the right hemithorax with cardiac dextroposition and hypoplasia of the right lung. No stomach bubble was seen below the diaphragm and the tip of the nasogastric tube lay above the diaphragm. No cardiac abnormality was detected at echocardiography. The baby was extubated and was able to breathe spontaneously. At reintubation, prior to contrast studies, a laryngeal cleft was noted. Iso-osmolar water soluble contrast injected into the nasogastric tube spilled freely into the trachea at all levels (Fig. 2). The stomach was hypoplastic and intrathoracic with a tortuous duodenal loop. Laryngoscopy showed complete cleft of the posterior larynx with wide separation of the arytenoids and continuation of the cleft into the trachea. The tracheal orifice was narrow and could not be entered with a 3 mm bronchoseope making it impossibleto determine the lower limit of the deft. Correspondence to: Dr G. M. A. Hendry, Department of Radiology, Royal Hospital for SickChildren, SciennesRoad, Edinburgh EH9 1LF.
Fig. 1 - Chest radiograph showing cardiac dexti'oposition and hypoplasia of the right lung.
438
CLINICAL RADIOLOGY
(a~
(b) Fig. 3 - (a) CT scan at the level of the first thoracic vertebra showing common tracheoeosophageal lumen containing both the nasogastric and endotracheal tubes. (b) CT scan at the level of the carina showing common tracheoesophageal lumen and hypoplastic right lung. Note nasogastric tube within lumen.
Fig. 2 - Contrast study showing free spillage of contrast from oesophagus to trachea.
A s s o c i a t e d c o n g e n i t a l a b n o r m a l i t i e s are c o m m o n : dext r o c a r d i a a n d m a l r o t a t i o n o f the c a e c u m was r e p o r t e d b y Pettit et al. (1979) a n d E v a n s (1985) described three cases o f which, one h a d a b s e n c e o f tracheal cartilage with b r o n c h i arising f r o m the o e s o p h a g u s a n d two h a d b i f u r c a t i o n o f the t r a c h e a in the neck. O n e o f the nine cases o f c o m p l e t e t r a c h e o e s o p h a g e a l cleft reviewed b y B u r r o u g h s a n d L e a p e (1974) h a d multiple c o n g e n i t a l abnormalities. O n l y one case o f c o m p l e t e l a r y n g o - t r a c h e o e s o p h a g e a l cleft has been r e p o r t e d ( D o n o h o e a n d Gee, 1984) in which there has been surgical r e p a i r a n d survival b e y o n d the i m m e d i a t e p o s t - o p e r a t i v e period. A Y - s h a p e d e n d o t r a c h e o b r o n c h i a l tube was m a d e to p r o t e c t the a i r w a y a n d a cuff o f o e s o p h a g u s was used to c o m p l e t e the t r a c h e a via t h o r a c i c a n d cervical a p p r o a c h e s .
C o m p u t e d t o m o g r a p h y is a useful m o d a l i t y for diagnosing cases such as this where e n d o s c o p y is difficult b e c a u s e o f o t h e r c o n g e n i t a l a n o m a l i e s o f the b r o n c h i a l tree. E a r l y accurate diagnosis is essential for successful m a n a g e m e n t , b u t in this case m u l t i p l e a n o m a l i e s were i n c o m p a t i b l e with life.
REFERENCES
Burroughs, N & Leape, LL (1974). Laryngotracheoesophageal deft: report of a case successfully treated and review of the literature. Pediatrics, 53, 516-222. Cotton, RT & Schreiber, JT (1981). Management of laryngotracheosophageal cleft. Annals of Otology, Rhinology and Laryngology, 90, 401-405. Donohoe, PK & Gee, PE (1984). Complete laryngotracheoesophageal cleft: management and repair. Journal of Paediatric Surgery, 19, 143-148. Evans, JNG (1985). Management of the cleft larynx and tracheoesophageal clefts. Annals of Otology, Rhinology and Laryngology, 94, 627-630. Pettit, PN Jnr, Butcher, RB, Bethea, MC & King, TT (1979). Surgical correction of complete tracheoesophageal cleft. Laryngoscope, 89, 804 811.