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Laryngotracheoesophageal cleft in a newborn
hzternational Journal of Pediatric Otorhinolaryngology, 8 (1984) 61-71
61
Elsevier POR 00261 Case R e p o r t s
Laryngotracheoesophageal cleft in a n e w b o r n * J a m e s R. K a u t e n x, H o r s t R. K o n r a d 2 a n d K e i t h A. W i c h t e r m a n 3 t Department of Cardiothoracic Surgery, Emory Unioersity School of Medicine, Atlanta, GA, and Dioisions of 20tolaryngology and "~General Surgery, Southern Illinois Unioersity School of Medicine, Springfield, IL (U.S.A.)
(Received December28th, 1983) (Revised May 7th, 1984) (Accepted May 25th, 1984)
Key words: congenital foregut anomalies - laryngotracheoesophagealcleft - bronchobiliary fistula -
microgastria - esophageal diverticulum
Summa~ A case of laryngotracheoesophageal cleft in a newborn is presented. A newborn presenting with respiratory distress underwent contrast radiographic studies, direct laryngoscopy, bronchoscopy, and esophagoscopy, demonstrating a complete laryngotracheoesophageal cleft, esophageal diverticulum and microgastria. Attempts to maintain an adequate airway by ligation of the salivary ducts and ligation of the distal esophagus were unsuccessful. Continued biliary drainage per endotracheal tube postoperatively afforded a presumptive diagnosis of bronchobiliary fistula. The child expired on its fourth day of life. A review of the literature and modalities of therapy for these rare and unusual combinations of foregut anomalies is discussed. This is the first reported case of a newborn with the combination of laryngotracheoesophageal cleft, bronchobiliary fistula, microgastria and esophageal diverticulum.
Introduction There have been less than 90 laryngotracheoesophageal clefts and their variants reported in the past and recent literature [3,5,6,8,11,13-15,18,21,26,27,29,30,31]. Two recent publications, one by Narcy et al. and the other by Roth et al., describe * Presented paper, SENTAC Meeting, San Diego, CA, U.S.A., December4-6, 1983. Correspondence: H.R. Konrad,,Chairmar~ Division of Otorhinolaryngology,Southern Illinois University
School of Medicine, Springfield, IL, U.S.A.
62 84 and 82 cases respectively [23,34]. C o n c o m i t a n t foregut anomalies reported are tracheoesophageal fistula [11,21] and unilateral p u l m o n a r y hypoplasia [26]. The present report records a case of a newborn with multiple foregut malformations: laryngotracheoesophageal cleft, esophageal diverticulum, bronchobiliary fistula and micrOgastria.
Case Report A neonate was admitted to St. John's High Risk Nursery ( H R N ) on April 9, 1981, because of respiratory distress. The patient was a 20-min-old white female born to a 17-year-old primipara after a 20-week by dates gestation. Rupture of membranes occurred 13 days prior to delivery. Delivery was vertex vaginal with low forceps under pudendal and paracervical blocks. The A p g a r at 5 min was 7. Birthweight was 1247 g. Because of respiratory distress in the delivery room, the b a b y was intubated and transferred directly to the H R N . O n admission, the neonate was in moderate respiratory distress with a heart rate of 156 b e a t s / m i n , respiratory rate of 40 b r e a t h s / m i n and blood pressure of 4 0 / 3 2
Fig. 1. Anteroposterior and lateral X-rays of the chest and abdomen showing an abnormal collection of air in the mediastinum and a paucity of intra-abdominal bowel gas. The esophagus appears to contain the endotracheal tube.
63
Fig. 2. Barium swallow showing cgntrast in the tracheobronchial tree, dilation of the distal esophagus, and a small tubular stomach.
64 m m Hg. Gross e x a m i n a t i o n of the larynx disclosed a laryngeal cleft. T h e trachea was in the midline, a n d bilateral rhonchi were heard in the lung fields. The heart rate was regular, a n d n o m u r m u r s were heard. The umbilical cord c o n t a i n e d 3 vessels; the
Fig. 3. Barium swallow demonstrating a cervical esophageal diverticulum.
65 abdominal examination was normal. The b a b y had normal female genitalia and a patent rectum. There were no neurologic abnormalities. Arterial blood gas on an inspiratory oxygen content of 100% was: PO2, 54 m m Hg; p C O 2, 80 m m Hg; and pH, 7.10. The chest X-ray (Fig. 1) revealed an abnormal mediastinal air collection and a paucity of intra-abdominal bowel gas. The tip of the endotracheal tube was at the level of the diaphragm and was felt to be within the esophagus. Therefore, the tube was withdrawn, and the b a b y reintubated. Bilious material was suctioned from the endotracheal tube. A n upper gastrointestinal series (Fig. 2) with cineradiography demonstrated barium in the tracheobronchial tree, dilation of the distal esophagus, and a small tubular stomach. A cervical esophageal diverticulum was also demonstrated (Fig. 3). Direct laryngoscopy, esophagoscopy and bronchoscopy were done and videofilmed. These studies demonstrated a complete laryngotracheoesophageal cleft as depicted in Fig. 4. Because of the baby's size and continued respiratory distress, a definitive reconstructive procedure was considered prohibitive; therefore, a palliative procedure was performed. The major salivary gland ducts were ligated and an exploratory laparot o m y was performed under local anesthesia. At laparotomy, there were several abnormalities noted (Fig. 5). The stomach was a narrow tubular structure in the normal location with no distinct differentiation into lesser or greater curvature, a n t r u m or cardia. The gallbladder emptied directly into the c o m m o n duct with no distinct cystic duct. The colon and small bowel were entirely normal. The distal esophagus was oversewn to prevent reflux of gastrointestinal contents into the airway, and a tube was placed in the jejunum to be used initially for
~ ~ Epiglottis~ J ~ ~ f1~,:,,~!~' ~ ~ V ° caI f°Ids~ ~ ' ~ r ~ II~'~,.~ ! ~ ~ A r y t e n o i d c a r t . ~ ~,~/~ t;',~"~,!,..~ ~ ' ~ Cricoidc a r t . - - ~ . ., ':~; , Cleft ,y,.
Fig. 4. Artist's drawing of the laryngotracheoesophageal cleft. The cleft extends to the level of the carina (complete cleft) and beyond into the right mainstream bronchus.
66
L
Esophagu~j "~-
Gallbladder~ " ~ ~ j
~
Stomach
J.//~,l.~'~-ldl~l']~,~ ¢ Spleen
co,oo
Fig. 5. Artist's drawing of findings at laparotomy.Explanationin text.
decompression and later for feedings. The abdomen was dosed, and the baby promptly returned to the HRN. Postoperatively, persistent bile-stained secretions were obtained from the endotracheal tube, suggesting a bronchobiliary fistula. There was progressive difficulty with oxygenation and maintenance of an adequate airway. The baby expired 24 h postoperatively.
Discussion Congenital foregut malformations consist of defects of embryogenesis from the buccopharyngeal membrane proximally to the liver bud distally. This group of anomalies includes tracheoesophageal fistula with and without atresia, bronchopulmonary sequestration, enteric duplication, esophageal diverticulum, neuroenteric and bronchogenic cyst, laryngeal cleft, and laryngotracheoesophageal cleft [17,28]. Reported rare malformations include microgastria and bronchobiliary fistula [2,7,9,10,12,16,20,24,25,32,35,36].
Laryngotracheoesophageal cleft Laryngotracheoesophageal cleft is the result of failure of fusion and rostral advancement of the lateral ridges of the tracheoesophageal groove [3,5]. This defect is rare, with 84 cases reported in the world literature as of 1983 [31]. Pettersson classified these defects into 3 types: Type I - - t h e laryngoesophageal cleft, Type I I - - t h e partial laryngotracheoesophageal deft, and the most severe form, Type III - - t h e complete laryngotracheoesophageal cleft [29]. The first reported case of laryngotracheoesophageal cleft was by Richter in 1792. Richter described an infant in whom, upon palpation with a finger in the infant's
67 throat, " t h e gullet and larynx were a common cavity" [33]. In 1949, Finlay reported the second case of the malformation [14]. A recent in-depth report has been published by Narcy [23]. The clinical manifestations of laryngotracheoesophageal cleft may vary in severity, often paralleling the extent of the defect. The most frequent symptoms are those of respiratory etiology: inspiratory stridor, respiratory distress or cyanosis.. Choking and excessive oral mucus can also be present. A toneless cry has also been described [14,30]. Polyhydramnios and prematurity are frequently associated [3]. Tracheoesophageal fistula and pulmonary hypoplasia have also been reported to coexist with laryngotracheoesophageal cleft [11,21,26]. The diagnosis of laryngeal clefts can be difficult and has been missed only to be made at autopsy. In the review by Burroughs and Leape, the diagnosis was not made in 14 of 33 (42%) patients until autopsy [6]. Felman stated that findings of persistent esophageal air, distended bowel, vertebral abnormalities, or pneumonia in a chest X-ray in early life are suggestive of abnormal tracheoesophageal connection; however, a normal chest X-ray does not exclude this [13]. Esophagography with dilute barium can delineate the anatomy; however, the appearance of contrast material in the tracheobronchial tree is not pathognomonic for laryngotracheoesophageal cleft. Other abnormalities, such as an H-type tracheoesophageal fistula, can produce similar findings. Direct visualization of the defect by endoscopic techniques is the most accurate means of diagnosis. The use of m o d e m endoscopic telescopes improves the quality of visual observation. Obtaining a videofilm of the endoscopic findings gives a permanent record which can be reviewed after the procedure. Despite careful endoscopy, the diagnosis has been missed [6]. One author [32] reported that, during endoscopy, the diagnosis could not be made until air was inflated into the esophagus via an esophageal catheter while viewing from the trachea. The first successful surgical correction of a partial laryngotracheoesophageal cleft was performed by Pettersson in 1955 [30]. He repaired a cleft extending to the lower margin of the thyroid gland through a cervical approach. The repair was performed by separating the trachea from the esophagus, then closing each individually with interrupted sutures. Hendren proposed a modification of the repair described by Pettersson whereby he interposed a vascularized shernohyoid muscle pedicle flap between two suture lines [18]. His repair was for a short cleft. Novoselac described a technique of left hypopharyngotomy with formation of two overlapping mucosal flaps to close a short cleft in two layers [27]. Jahrsdoerfer et al. reported the repair of a cleft trachea to the level of the second tracheal ring. The repair was performed via an anterior laryngofissure approach [19]. This approach was detailed further by Bell et al. and Montgomery and Smith [1,22]. Access can be made through a thyreotomy or a cricotracheo-fissure [31]. An advantage of this approach is that the nerve supply to the larynx is not damaged; a disadvantage of this approach is that an already damaged larynx can be made unstable [34]. The repair of a complete laryngotracheoesophageal cleft has not been widely
68 reported, as this is an uncommon and usually fatal variant. In the review by Burroughs and Leape, 7 of 33 (21%) cases were clefts to the level of the carina [7]. Four of these patients had surgical repair; three did not. There were no survivors. Pettit and associates reported the repair of a complete laryngotracheoesophageal cleft using a combined cervical and thoracic approach. A right thoracotomy was done first, repairing the cleft to the level of the thoracic inlet. A low horizontal collar incision was made, repairing the cervical portion. This patient survived 10 days postsurgery, expiring because of a mucous plug in the tracheostomy tube. On postmortem examination, the surgical repair was intact [31]. Severe respiratory distress in infants with complete clefts often precludes major definitive surgery. A technique, and the one used in our case, is to isolate the gastrointestinal tract from the respiratory system by oversewing the distal esophagus, ligating the major salivary ducts, and placing an enteral tube for decompression. A definitive repair is then performed when the baby is able to undergo major reconstructive surgery. A similar technique has been reported by Cotton and Schreiber [8]. The prognosis of a complete laryngotracheoesophageal cleft is poor. No long-term survivors of a complete laryngotracheoesophageal cleft have been reported.
Bronchobiliary fistula Bronchobiliary fistula is rare. The embryology of bronchobiliary fistula has been postulated to be the result of two possible mechanisms. Neuhauser and co-workers found the major portion of their fistulas represented esophagus histologically, therefore representing a duplication of the upper gastrointestinal tract between the laryngotracheal outgrowth and the hepatic diverticulum [25]. Bremer proposed a different mechanism, suggesting the anomaly resulted from the joining of an accessory mediastinal bronchus with an aberrant hepatic duct [4]. In 1952, Neuhauser et al. reported the first congenital fistula between the respiratory tree and the biliary tract [25]. The first successful surgical management was described in 1958 by Haight and Groves [16]. Cuadros, in 1974, summarized a total of 8 cases in the world literature [9]. Anatomically, there is a communication between the right mainstem bronchus and the left hepatic ductal system [36]. The histology of these lesions is fairly consistent. The proximal portion consists of cartilaginous rings and glandular epithelium representing bronchus with transition to squamous epithelium distally representing biliary epithelium [36]. Signs and symptoms are of respiratory origin, with recurrent bronchopulmonary infections and aspiration pneumonitis early in fife [36]. Expectoration of bile-stained sputum may also be present. The diagnosis has been made in several ways. A bronchogram demonstrated the fistula in the case presented by Haight and Graves [16]. Enjoji and associates diagnosed their fistula with an operative fistulocholangiogram [12]. Weitzmann and associates reported bilious drainage from an anomalous bronchus on bronchoscopy [36].
69 Definitive treatment has consisted of resection of the intrathoracic fistula with no attempt at excision of the short intra-abdominal segment [16,36]. The anomaly bears a good prognosis, as in the 8 cases summarized by Cuadros, 6 (75%) survived. The 2 (25%) non-survivors did not have definitive surgical treatment [9].
Microgastria Microgastria is a rare caudal foregut anomaly. It represents one of the more primitive stages o f the embryogenesis of the stomach. There is an arrest of the normal axial rotation and formation of the greater or lesser curvatures [20]. As a result, the small tubular stomach remains with its long axis in the mid-sagittal plane, without differentiation into parts [7]. Dide reported in 1894 the first case of microgastria in an adult [2]. Caffey, in 1956, reported the first case in a child [7]. A number of cases subsequently have been reported in the literature [2,7,10,20,24,32,35]. Patients can present at birth or later with a history of feeding difficulties, vomiting, recurrent pneumonitis, or failure to thrive. These symptoms are consistent with the loss of gastric storage which is assumed by the esophagus. Treatment may be medical or operative. Medical management consists of multiple small feedings. A major complication, aspiration pneumonitis, can still occur. Neifield et al. reported successful management with a formation of a Hunt-Lawrence pouch anastomosed to the stomach near the pylorus [24]. Prognosis with recent and more aggressive surgical management is good.
Conclusion Patients with complete laryngotracheoesophageal cleft have not survived in the past. Better diagnosis with present-day roentgenographic studies and endoscopy and improved perioperative and postoperative care should lead to a greater chance of survival in the future. Several approaches to the immediate care of an infant with laryngotracheoesophageal cleft are possible. The ideal method is immediate primary repair through a right thoracotomy and right cervical incision. The majority of these children, however, have extreme respiratory difficulty and are poor risks for major reconstructive surgery. One method of managing these critically ill infants would be with a staged repair, as attempted here; the initial stage being the isolation of the gastrointestinal tract from the pulmonary system, and the second being definitive repair of the cleft at a later date.
References 1 Bell, D.W., Christiansen, T.,~., Smith, T.E. and Strucker, F.J., Laryngotracheoesophagealcleft: the anterior approach, Ann. Otol., 86 (1977) 616-622.
70 2 Blank, E. and Chisolm, A.J., Congenital microgastria: a case report with a 26-year follow-up. Pediatrics, 51 (1973) 1037-1041. 3 Blumberg. J.B., Stevenson, J.K. and Lemire, R.J. et al., Laryngotracheoesophageal cleft, the embryologic implications: review of the literature, Surgery, 57 (1965) 559-566. 4 Bremer, J.L., Congenital Anomalies of the Viscera: Their Embryological Basis, Cambridge, Harvard University Press, 1957. 5 Broek, P. van den and Brinkman, W.F.T., Congenital laryngeal defects, Int. J. pediat, otorhinolaryng.,1 (1979) 71-78. 6 Burroughs, N. and Leape, L.L., Laryngotracheoesophageal cleft: report of a case successfully treated and review of the literature, Pediatrics, 53 (1974) 516-522. 7 Caffey, J., Pediatric X-ray diagnosis. In: A Textbook for Students and Practitioners of Pediatrics. Surgery, and Radiology (3rd edn), Year Book Medical Publishers, Chicago, 1956, pp. 496-498. 8 Cotton, R.T. and Schreiber, J.T., Management of laryngotracheoesophageal cleft, Ann. otol., 90 (1981) 401-405. 9 Cuadros, J., Aransay, A. and Nistal, M. et al., Fistula traqueo-biliar congenita, An. esp. Pediat.. 7 (1974) 256-261. 10 Dide, M., Sur en estomac d'adulte h type foetal, Bull. soc. anat., 69 (1894) 669-670. 11 Donahoe, P.K. and Hendren. W.H., The surgical management of laryngotracheoesophageal cleft with tracheoesophageal fistula and esophageal atresia, Surgery, 71 (1972) 363-368. 12 Enjoji, M., Watanabe. H. and Nakamura, Y., A case report: congenital biliotracheal fistula with trifurcation of bronchi, Ann. paediat., 200 (1963) 321. 13 Felman, A.H. and Talbert, J.L., Laryngotracheoesophagcal cleft, description of a combined laryngoscopic and roentgenographic diagnostic technique and report of two patients, Radiology, 103 (1972) 641-644. 14 Finlay, H.V.L., Familial congenital stridor, Arch. Dis Child., 24 (1949) 219-223. 15 Fuzesi, K. and Young, D.G., Congenital laryngotracheoesophageal cleft, J. pediat. Surg., 11 (1976) 933-937. 16 Haight, C. and Graves, W.H., Personal communication with Weitzman, J.J. 17 Heithoff, K.B., Sane, S.M. and Williams. H.G. et al., Bronchopulmonary foregut malformations: a unifying etiological concept, Amer. j. roentgenol., 126 (1976) 46-55. 18 Hendren. W.H., Repair of a laryngotracheoesophageal cleft using interposition of a strap muscle, J. pediat. Surg., 11 (1976) 425-429. 19 Jahrsdoerfer, R.A., Kirchner, J.A. and Thaler. S.U.. Cleft larynx. Arch. Otolaryng., 86 (1967) 108-113. 20 Kessler, H. and Smulewicz, J.J., Microgastia associated with agenesis of the spleen, Radiology, 107 (1973) 393-396. 21 Mahout, G.H., Cohen, S.R. and Woolley, M.M.. Laryngotracheoesophageal cleft associated with esophageal atresia and multiple tracheoesophageal fistulas in a twin, J. thorac, cardiovasc. Surg., 65 (1973) 223-226. 22 Montgomery. W.W. and Smith, S.A., Congenital laryngeal defects in the adult, Ann. otol., 85 (1976) 491-497. 23 Narcy, P., Andrieu-Guitrancourt. J., Beauvillain de Montreuil, C. and Dcsnos. J. et al., Le larynx de l'enfant, Paris, Library Annette, 1979. 24 Neifeld, J.P., Berman, W.F. and Lawrence, W. et al.. Management of congenital microgastria with a jejunal reservoir pouch, J. pediat. Surg.. 15 (1980) 882-885. 25 Neuhauser, E.B.D., Elkin. M. and Landing, B., Congenital direct communication between biliary system and respiratory tract. Amer. J. Dis Child., 83 (1952) 654-659. 26 Novak, R.W.. Laryngotracheoesophageal cleft and unilateral pulmonary, hypoplasia in twins, Pediatrics. 67 (1981) 732-734, 27 Novoselac, M.. Dangel, P. and Fisch. U., Laryngotracheoesophageal cleft, J pediat. Surg., 8 (1973) 963-964. 28 O'Connell, D.J. Kelleher, J., Congenital intrathoracic bronchopulmonary foregut malformations in childhood, J. Canad. Assoc Radiol., 30 (1979) 103-108. 29 Pettersson, G., Laryngo-tracheo-esophageal cleft, Z. Kinderchir., 7 (1969) 43-48. 30 Pettersson, Ga., Inhibited separation of larynx and the upper part of trachea from oesophagus in a newborn, report of a case successfully operated upon, Acta chir. stand. 110 (1955l 250-254.
71 31 Pettit, P.N., Butcher, R.B. and Bethea, M.C. et. al., Surgical correction of complete tracheoesophageal cleft, Laryngoscopy, 89 (1979) 804-811. 32 Putschar, W.G.J. and Manion, W.C., Congenital absence of the spleen and associated anomalies, Amer. j. clin. path., 26 (1956) 429-470. 33 Richter, C.F., Dissertatio medico de infanticido in artis obstetriciae, Thesis for degree of doctor of medicine, 1972. 34 Roth, B., Rose, K-G, Benz-Bohm and Gunther, H., Laryngotracheo-oesophageal cleft, clinical features, diagnosis and therapy. Europ. J. Pedlar., 140 (1983) 41-46. 35 Shackelford, G.D., McAlister, N.H., Brodeur, A.E., et al., Congenital microgastria, Amer. J Roentgenol., 118 (1973) 72-76. 36 Weitzman, J.J., Cohen, S.R.. Woods, L.O., et al., Congenital bronchobiliary fistula, J. pediat., 73 (1968) 329-334.
61
Elsevier POR 00261 Case R e p o r t s
Laryngotracheoesophageal cleft in a n e w b o r n * J a m e s R. K a u t e n x, H o r s t R. K o n r a d 2 a n d K e i t h A. W i c h t e r m a n 3 t Department of Cardiothoracic Surgery, Emory Unioersity School of Medicine, Atlanta, GA, and Dioisions of 20tolaryngology and "~General Surgery, Southern Illinois Unioersity School of Medicine, Springfield, IL (U.S.A.)
(Received December28th, 1983) (Revised May 7th, 1984) (Accepted May 25th, 1984)
Key words: congenital foregut anomalies - laryngotracheoesophagealcleft - bronchobiliary fistula -
microgastria - esophageal diverticulum
Summa~ A case of laryngotracheoesophageal cleft in a newborn is presented. A newborn presenting with respiratory distress underwent contrast radiographic studies, direct laryngoscopy, bronchoscopy, and esophagoscopy, demonstrating a complete laryngotracheoesophageal cleft, esophageal diverticulum and microgastria. Attempts to maintain an adequate airway by ligation of the salivary ducts and ligation of the distal esophagus were unsuccessful. Continued biliary drainage per endotracheal tube postoperatively afforded a presumptive diagnosis of bronchobiliary fistula. The child expired on its fourth day of life. A review of the literature and modalities of therapy for these rare and unusual combinations of foregut anomalies is discussed. This is the first reported case of a newborn with the combination of laryngotracheoesophageal cleft, bronchobiliary fistula, microgastria and esophageal diverticulum.
Introduction There have been less than 90 laryngotracheoesophageal clefts and their variants reported in the past and recent literature [3,5,6,8,11,13-15,18,21,26,27,29,30,31]. Two recent publications, one by Narcy et al. and the other by Roth et al., describe * Presented paper, SENTAC Meeting, San Diego, CA, U.S.A., December4-6, 1983. Correspondence: H.R. Konrad,,Chairmar~ Division of Otorhinolaryngology,Southern Illinois University
School of Medicine, Springfield, IL, U.S.A.
62 84 and 82 cases respectively [23,34]. C o n c o m i t a n t foregut anomalies reported are tracheoesophageal fistula [11,21] and unilateral p u l m o n a r y hypoplasia [26]. The present report records a case of a newborn with multiple foregut malformations: laryngotracheoesophageal cleft, esophageal diverticulum, bronchobiliary fistula and micrOgastria.
Case Report A neonate was admitted to St. John's High Risk Nursery ( H R N ) on April 9, 1981, because of respiratory distress. The patient was a 20-min-old white female born to a 17-year-old primipara after a 20-week by dates gestation. Rupture of membranes occurred 13 days prior to delivery. Delivery was vertex vaginal with low forceps under pudendal and paracervical blocks. The A p g a r at 5 min was 7. Birthweight was 1247 g. Because of respiratory distress in the delivery room, the b a b y was intubated and transferred directly to the H R N . O n admission, the neonate was in moderate respiratory distress with a heart rate of 156 b e a t s / m i n , respiratory rate of 40 b r e a t h s / m i n and blood pressure of 4 0 / 3 2
Fig. 1. Anteroposterior and lateral X-rays of the chest and abdomen showing an abnormal collection of air in the mediastinum and a paucity of intra-abdominal bowel gas. The esophagus appears to contain the endotracheal tube.
63
Fig. 2. Barium swallow showing cgntrast in the tracheobronchial tree, dilation of the distal esophagus, and a small tubular stomach.
64 m m Hg. Gross e x a m i n a t i o n of the larynx disclosed a laryngeal cleft. T h e trachea was in the midline, a n d bilateral rhonchi were heard in the lung fields. The heart rate was regular, a n d n o m u r m u r s were heard. The umbilical cord c o n t a i n e d 3 vessels; the
Fig. 3. Barium swallow demonstrating a cervical esophageal diverticulum.
65 abdominal examination was normal. The b a b y had normal female genitalia and a patent rectum. There were no neurologic abnormalities. Arterial blood gas on an inspiratory oxygen content of 100% was: PO2, 54 m m Hg; p C O 2, 80 m m Hg; and pH, 7.10. The chest X-ray (Fig. 1) revealed an abnormal mediastinal air collection and a paucity of intra-abdominal bowel gas. The tip of the endotracheal tube was at the level of the diaphragm and was felt to be within the esophagus. Therefore, the tube was withdrawn, and the b a b y reintubated. Bilious material was suctioned from the endotracheal tube. A n upper gastrointestinal series (Fig. 2) with cineradiography demonstrated barium in the tracheobronchial tree, dilation of the distal esophagus, and a small tubular stomach. A cervical esophageal diverticulum was also demonstrated (Fig. 3). Direct laryngoscopy, esophagoscopy and bronchoscopy were done and videofilmed. These studies demonstrated a complete laryngotracheoesophageal cleft as depicted in Fig. 4. Because of the baby's size and continued respiratory distress, a definitive reconstructive procedure was considered prohibitive; therefore, a palliative procedure was performed. The major salivary gland ducts were ligated and an exploratory laparot o m y was performed under local anesthesia. At laparotomy, there were several abnormalities noted (Fig. 5). The stomach was a narrow tubular structure in the normal location with no distinct differentiation into lesser or greater curvature, a n t r u m or cardia. The gallbladder emptied directly into the c o m m o n duct with no distinct cystic duct. The colon and small bowel were entirely normal. The distal esophagus was oversewn to prevent reflux of gastrointestinal contents into the airway, and a tube was placed in the jejunum to be used initially for
~ ~ Epiglottis~ J ~ ~ f1~,:,,~!~' ~ ~ V ° caI f°Ids~ ~ ' ~ r ~ II~'~,.~ ! ~ ~ A r y t e n o i d c a r t . ~ ~,~/~ t;',~"~,!,..~ ~ ' ~ Cricoidc a r t . - - ~ . ., ':~; , Cleft ,y,.
Fig. 4. Artist's drawing of the laryngotracheoesophageal cleft. The cleft extends to the level of the carina (complete cleft) and beyond into the right mainstream bronchus.
66
L
Esophagu~j "~-
Gallbladder~ " ~ ~ j
~
Stomach
J.//~,l.~'~-ldl~l']~,~ ¢ Spleen
co,oo
Fig. 5. Artist's drawing of findings at laparotomy.Explanationin text.
decompression and later for feedings. The abdomen was dosed, and the baby promptly returned to the HRN. Postoperatively, persistent bile-stained secretions were obtained from the endotracheal tube, suggesting a bronchobiliary fistula. There was progressive difficulty with oxygenation and maintenance of an adequate airway. The baby expired 24 h postoperatively.
Discussion Congenital foregut malformations consist of defects of embryogenesis from the buccopharyngeal membrane proximally to the liver bud distally. This group of anomalies includes tracheoesophageal fistula with and without atresia, bronchopulmonary sequestration, enteric duplication, esophageal diverticulum, neuroenteric and bronchogenic cyst, laryngeal cleft, and laryngotracheoesophageal cleft [17,28]. Reported rare malformations include microgastria and bronchobiliary fistula [2,7,9,10,12,16,20,24,25,32,35,36].
Laryngotracheoesophageal cleft Laryngotracheoesophageal cleft is the result of failure of fusion and rostral advancement of the lateral ridges of the tracheoesophageal groove [3,5]. This defect is rare, with 84 cases reported in the world literature as of 1983 [31]. Pettersson classified these defects into 3 types: Type I - - t h e laryngoesophageal cleft, Type I I - - t h e partial laryngotracheoesophageal deft, and the most severe form, Type III - - t h e complete laryngotracheoesophageal cleft [29]. The first reported case of laryngotracheoesophageal cleft was by Richter in 1792. Richter described an infant in whom, upon palpation with a finger in the infant's
67 throat, " t h e gullet and larynx were a common cavity" [33]. In 1949, Finlay reported the second case of the malformation [14]. A recent in-depth report has been published by Narcy [23]. The clinical manifestations of laryngotracheoesophageal cleft may vary in severity, often paralleling the extent of the defect. The most frequent symptoms are those of respiratory etiology: inspiratory stridor, respiratory distress or cyanosis.. Choking and excessive oral mucus can also be present. A toneless cry has also been described [14,30]. Polyhydramnios and prematurity are frequently associated [3]. Tracheoesophageal fistula and pulmonary hypoplasia have also been reported to coexist with laryngotracheoesophageal cleft [11,21,26]. The diagnosis of laryngeal clefts can be difficult and has been missed only to be made at autopsy. In the review by Burroughs and Leape, the diagnosis was not made in 14 of 33 (42%) patients until autopsy [6]. Felman stated that findings of persistent esophageal air, distended bowel, vertebral abnormalities, or pneumonia in a chest X-ray in early life are suggestive of abnormal tracheoesophageal connection; however, a normal chest X-ray does not exclude this [13]. Esophagography with dilute barium can delineate the anatomy; however, the appearance of contrast material in the tracheobronchial tree is not pathognomonic for laryngotracheoesophageal cleft. Other abnormalities, such as an H-type tracheoesophageal fistula, can produce similar findings. Direct visualization of the defect by endoscopic techniques is the most accurate means of diagnosis. The use of m o d e m endoscopic telescopes improves the quality of visual observation. Obtaining a videofilm of the endoscopic findings gives a permanent record which can be reviewed after the procedure. Despite careful endoscopy, the diagnosis has been missed [6]. One author [32] reported that, during endoscopy, the diagnosis could not be made until air was inflated into the esophagus via an esophageal catheter while viewing from the trachea. The first successful surgical correction of a partial laryngotracheoesophageal cleft was performed by Pettersson in 1955 [30]. He repaired a cleft extending to the lower margin of the thyroid gland through a cervical approach. The repair was performed by separating the trachea from the esophagus, then closing each individually with interrupted sutures. Hendren proposed a modification of the repair described by Pettersson whereby he interposed a vascularized shernohyoid muscle pedicle flap between two suture lines [18]. His repair was for a short cleft. Novoselac described a technique of left hypopharyngotomy with formation of two overlapping mucosal flaps to close a short cleft in two layers [27]. Jahrsdoerfer et al. reported the repair of a cleft trachea to the level of the second tracheal ring. The repair was performed via an anterior laryngofissure approach [19]. This approach was detailed further by Bell et al. and Montgomery and Smith [1,22]. Access can be made through a thyreotomy or a cricotracheo-fissure [31]. An advantage of this approach is that the nerve supply to the larynx is not damaged; a disadvantage of this approach is that an already damaged larynx can be made unstable [34]. The repair of a complete laryngotracheoesophageal cleft has not been widely
68 reported, as this is an uncommon and usually fatal variant. In the review by Burroughs and Leape, 7 of 33 (21%) cases were clefts to the level of the carina [7]. Four of these patients had surgical repair; three did not. There were no survivors. Pettit and associates reported the repair of a complete laryngotracheoesophageal cleft using a combined cervical and thoracic approach. A right thoracotomy was done first, repairing the cleft to the level of the thoracic inlet. A low horizontal collar incision was made, repairing the cervical portion. This patient survived 10 days postsurgery, expiring because of a mucous plug in the tracheostomy tube. On postmortem examination, the surgical repair was intact [31]. Severe respiratory distress in infants with complete clefts often precludes major definitive surgery. A technique, and the one used in our case, is to isolate the gastrointestinal tract from the respiratory system by oversewing the distal esophagus, ligating the major salivary ducts, and placing an enteral tube for decompression. A definitive repair is then performed when the baby is able to undergo major reconstructive surgery. A similar technique has been reported by Cotton and Schreiber [8]. The prognosis of a complete laryngotracheoesophageal cleft is poor. No long-term survivors of a complete laryngotracheoesophageal cleft have been reported.
Bronchobiliary fistula Bronchobiliary fistula is rare. The embryology of bronchobiliary fistula has been postulated to be the result of two possible mechanisms. Neuhauser and co-workers found the major portion of their fistulas represented esophagus histologically, therefore representing a duplication of the upper gastrointestinal tract between the laryngotracheal outgrowth and the hepatic diverticulum [25]. Bremer proposed a different mechanism, suggesting the anomaly resulted from the joining of an accessory mediastinal bronchus with an aberrant hepatic duct [4]. In 1952, Neuhauser et al. reported the first congenital fistula between the respiratory tree and the biliary tract [25]. The first successful surgical management was described in 1958 by Haight and Groves [16]. Cuadros, in 1974, summarized a total of 8 cases in the world literature [9]. Anatomically, there is a communication between the right mainstem bronchus and the left hepatic ductal system [36]. The histology of these lesions is fairly consistent. The proximal portion consists of cartilaginous rings and glandular epithelium representing bronchus with transition to squamous epithelium distally representing biliary epithelium [36]. Signs and symptoms are of respiratory origin, with recurrent bronchopulmonary infections and aspiration pneumonitis early in fife [36]. Expectoration of bile-stained sputum may also be present. The diagnosis has been made in several ways. A bronchogram demonstrated the fistula in the case presented by Haight and Graves [16]. Enjoji and associates diagnosed their fistula with an operative fistulocholangiogram [12]. Weitzmann and associates reported bilious drainage from an anomalous bronchus on bronchoscopy [36].
69 Definitive treatment has consisted of resection of the intrathoracic fistula with no attempt at excision of the short intra-abdominal segment [16,36]. The anomaly bears a good prognosis, as in the 8 cases summarized by Cuadros, 6 (75%) survived. The 2 (25%) non-survivors did not have definitive surgical treatment [9].
Microgastria Microgastria is a rare caudal foregut anomaly. It represents one of the more primitive stages o f the embryogenesis of the stomach. There is an arrest of the normal axial rotation and formation of the greater or lesser curvatures [20]. As a result, the small tubular stomach remains with its long axis in the mid-sagittal plane, without differentiation into parts [7]. Dide reported in 1894 the first case of microgastria in an adult [2]. Caffey, in 1956, reported the first case in a child [7]. A number of cases subsequently have been reported in the literature [2,7,10,20,24,32,35]. Patients can present at birth or later with a history of feeding difficulties, vomiting, recurrent pneumonitis, or failure to thrive. These symptoms are consistent with the loss of gastric storage which is assumed by the esophagus. Treatment may be medical or operative. Medical management consists of multiple small feedings. A major complication, aspiration pneumonitis, can still occur. Neifield et al. reported successful management with a formation of a Hunt-Lawrence pouch anastomosed to the stomach near the pylorus [24]. Prognosis with recent and more aggressive surgical management is good.
Conclusion Patients with complete laryngotracheoesophageal cleft have not survived in the past. Better diagnosis with present-day roentgenographic studies and endoscopy and improved perioperative and postoperative care should lead to a greater chance of survival in the future. Several approaches to the immediate care of an infant with laryngotracheoesophageal cleft are possible. The ideal method is immediate primary repair through a right thoracotomy and right cervical incision. The majority of these children, however, have extreme respiratory difficulty and are poor risks for major reconstructive surgery. One method of managing these critically ill infants would be with a staged repair, as attempted here; the initial stage being the isolation of the gastrointestinal tract from the pulmonary system, and the second being definitive repair of the cleft at a later date.
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