H-Type Congenital Tracheoesophageal Fistula in a Saudi Child

2 downloads 8 Views 123KB Size Report
The skull was small with a wide anterior fontanelle, an open posterior ... thyroid was displaced upward, and after division of the inferior thyroid veins, the trachea ...

Case Reports

H-Type Congenital Tracheoesophageal Fistula in a Saudi Child Asal Y. Izzidien Al-Samarrai, FRCS, (Ed)* Zein Al-Abdeen Karrar, MRCP (UK), DCH(London), DTCH(Liverpool)† Ahmed Al-Muzrachi, FRCR††, Klaus Jessen, MD§ Mohamed Ahmed Abdulla, MRCP (UK), DCH(London), DTCH(Liverpool)|| * Professor of Pediatric Surgery; † Associate Professor ; Department of Pediatrics; †† Consultant Radiologist; Department of Medicine; § Assistant Professor; Department of Medicine; || Associate Professor; Department of Pediatrics; College of Medicine and King Khalid Hospital, King Saud University, P.O. Box 2925, Riyadh 11461, Saudi Arabia

AYI Al-Samarrai, Z Al-Abdeen Karrar, A Al-Muzrachi, K Jessen, MA Abdulla, H-Type Congenital Tracheoesophageal Fistula in a Saudi Child. 1986; 6(2): 129-132 MeSH KEYWORDS: Tracheoesophageal fistula, congenital

Introduction

TRACHEOESOPHAGEAL fistula without atresia is a rare entity, and is commonly referred to as “H-type” or “Ntype” and constitutes 1 to 4.2% of all tracheoesophageal fistulas, the incidence of which is about one per 4,000 births.1,2,3 The diagnosis of H-type fistula is difficult despite modern diagnostic tools, but should be suspected when an infant presents with a triad including bouts of coughing during feeding, sometimes followed by cyanosis, recurrent chest infections (pneumonitis), and gaseous abdominal distention. This paper documents the first H-type fistula reported in a Saudi child in association with other anomalies (VATER association). The diagnostic and management aspects of this rare type of fistula will be discussed. Case Report A 53-day-old Saudi male infant was referred to our care from The Childrens Hospital on the parents’ request. He was the product of a full-term, twin pregnancy, delivered by cesarean section because of a transverse lie. His female twin was normal. The parents are unrelated and have a two-year-old daughter who is normal. The patient’s birth weight was 3.2 kg, and he was found to have an imperforate anus with passage of meconium through the urethra. A right transverse colostomy was done for a high type anorectal malformation. He was noted to have choking attacks associated with bottle feeding, and developed recurrent episodes of aspiration pneumonia. Bottle feeding was stopped and he was fed through a nasogastric tube, but he persisted in having choking attacks with aspiration. His weight dropped by 500 gm over five weeks. Barium swallow was done twice but no abnormalities could be demonstrated. It was assumed that he had functional pharyngeal incoordination. On arrival at King Khalid University Hospital, examination revealed a wasted baby with mild respiratory distress. His respiratory rate was 52/ min, pulse rate 125/min, and blood pressure 80/50 mmHg. His weight, length and skull circumference were 2.83 kg, 52 cm and 32 cm, respectively. All were below the tenth percentile for his age. There were no dysmorphic features. The skull was small with a wide anterior fontanelle, an open posterior fontanelle and a metopic suture. There were no obvious skeletal malformations except for mild scoliosis in the thoracic region. The upper limbs and sacrum were clinically normal. The genitalia were normal, and the cardiovascular and central nervous system examinations were normal. Examination of the respiratory system showed tachypnea and signs of consolidation in the right upper lobe. Initial investigation showed a hypochromic microcytic anemia and a low serum albumin. A chest x-ray confirmed the presence of right upper lobe collapse and consolidation. The barium swallow with cinefluoroscopy and videotape recording showed the presence of an H-type fistula at the root of the neck (Figure 1).

H-Type Congenital Tracheoesophageal Fistula in a Saudi Child

Figure 1. Preoperative barium swallow showing the H-type fistula (see arrow); O = esophagus, T = trachea. Barium seen passing through the fistula into the trachea.

Further work-up to rule out other malformations included skull and abdominal ultrasound followed by an intravenous pyelogram, micturating cystourethrogram, and isotope renogram studies. These revealed a nonfunctioning, small right kidney, and a functioning, normal left kidney with bilateral dilatation of both ureters and calyceal systems. There was no obstructive lesion, and there was bilateral, grade III vesicoureteric reflux on voiding. A skeletal survey, echocardiogram, and electroencephalogram all were normal. There was partial sacral agenesis on sacral x-ray. Urine cultures grew Escherichia coli and Proteus mirabilis. Our final diagnosis was compatible with a VATER association (vertebral defects, imperforate anus, tracheoesophageal fistula, and radial and renal dysplasia) with failure to thrive because of feeding difficulty, respiratory and renal tract infections. He was nursed upright and tolerated gradual increments of full-strength milk through a nasogastric tube. His caloric intake was increased by adding Caloreen to the feeds. He received regular chest physiotherapy. His pneumonia and urinary tract infections resolved on appropriate antibiotics. Five weeks later, his weight was 4.2 kg and he was judged to be fit for surgery. Operative Report Endoscopy was done prior to exploration, and this confirmed the presence of H-type fistula. An anterior transcervical approach through a low transverse incision was made, exposing both the thyroid and the trachea. The thyroid was displaced upward, and after division of the inferior thyroid veins, the trachea was displaced laterally, exposing the fistula and a tape was passed around it. The fistula was then divided and sutured, after which pretracheal fascia and fat were placed between the trachea and the esophagus, covering the site of the fistula. His postoperative course was uneventful. He required total parenteral nutrition for seven days. A repeat barium swallow showed no evidence of fistula or reflux, and he was started on bottle feeding with full-strength milk following an initial trial of feeding with glucose and electrolyte solution. He tolerated bottle feeding well and gained weight steadily. His weight on discharge, seven weeks after admission, was 4.8 kg. Discussion The H-type tracheoesophageal fistula is embryologically the least severe form of larynogotracheoesophageal cleft.4 The normal development of the trachea and esophagus begins at about the fourth week of gestation, when a median laryngotracheal groove appears in the ventral wall of the foregut. As this groove deepens and the foregut elongates, ridges appear on the lateral walls, beginning at the caudal end, and then they fuse to separate the larynogotracheal tube from the esophagus.5Failure of fusion of the lateral wall, which normally forms a septum between the trachea and esophagus, will lead to H-type fistula. The failure of fusion is variable in location and extent.4 It was reported that 24% were cervical, 54% were high thoracic, and 22% near the carina.6 Andrassy, et al,7 found 70% of 23 tracheoesophageal fistulas were within 2 cm above or below the sternal notch, and could be

Annals of Saudi Medicine, Vol 6 No. 2; 1986

H-Type Congenital Tracheoesophageal Fistula in a Saudi Child

repaired through a cervical incision. Although the first description of isolated tracheoesophageal fistula was by Lamb in 1873,8 it was not until 1939 when Imperatori9 reported the first surgical repair. Since then over 200 cases have been reported in the literature. 4,621

In this anomaly, the esophagus and trachea are quite normal, except that there is a very short fistula between the two, which runs from the esophagus upward to the trachea. Because of this upward course, swallowing is not always associated with aspiration into the trachea.21 These children usually will present with a clinical triad of paroxysms of coughing precipitated by feeding with occasional cyanosis during some feeds, gaseous distention of the gastrointestinal tract, and repeated attacks of pneumonia. It is known that some of these cases remain undiagnosed until adulthood, the oldest reported case being 63 years of age.22-23 The diagnosis of these fistulas should be made by barium swallow with cinefluoroscopy and video recording, as they can be missed by the conventional methods such as esophagography. 24 Tracheobronchoscopy in conjunction with instillation of methylene blue into the esophagus to identify the tracheoesophageal fistula was recommended by Abbott.25 Measurement of intragastric oxygen concentration was described in diagnosing these fistulas where the intragastric oxygen concentration increased and declined in response to the successive endotracheal insufflation of 100% oxygen and room air.26 Endoscopy seems to be a superior diagnostic tool and is considered to be more reliable and safer than radiologic examination of the esophagus. 17 It also allows comprehensive evaluation of any other congenital anomalies in the tracheobronchial tree and esophagus. 27 For that reason, endoscopy was performed in our patient but demonstrated only one anomaly. No further fistula could be seen, although double and multiple fistulas have been reported.2, 4 It is important to stop oral feeding, start nasogastric tube feeding, and to treat the chest problem as soon as the diagnosis is made. The usual operative approach has been a right cervical approach. 2 We have found that the anterior transcervical approach, as described by Killen, et al,12gives better exposure. We share the view of Ein, et al,19 that it is not necessary to isolate and surround with tapes both the trachea and esophagus, as well as the fistula, as this increases the risk of recurrent laryngeal nerve injury and postoperative tracheal edema. We found one tape around the fistula to be sufficient. The other associated anomalies in this child are compatible with the description of similar cases reported in the literature as the VATER association.28 Our patient had his fistula repaired and will be followed regarding his urinary and anal anomalies. REFERENCES 1. Haight C. Some observations on esophageal and tracheoesophageal fistulas of congenital origin. J Thorac Surg 1957; 34:14172. 2. Esophageal atresia and tracheoesophageal fistula. In: Raffensperger JG, ed. Swenson’s Pediatric Surgery 4th ed. New York: Appleton-Century-Crofts, 1980:650-72. 3. Holder TM, Cloud DT, Lewis JE Jr, Pilling GP 4th. Esophageal atresia and tracheoesophageal fistula. A survey of its members by the Surgical Section of the American Academy of Pediatrics. Pediatrics 1964; 34:542-9. 4. LaSalle AJ, Andrassy RJ, Ver Steeg K, Ratner I. Congenital tracheoesophageal fistula without esophageal atresia. J Thorac Cardiovasc Surg 1979; 78(4):583-8. 5. Cudmore RE: Oesophageal atresia and trachea-oesophageal fistula. In: Rickham PP, Lister J, Irving IM, eds; Neonatal surgery. London: Butterworth, 1980:190-1. 6. Lynn HB, Davis LA. Tracheo-esophageal fistula without atresia of the esophagus. Surg Clin North Am 1961; 41:871-82. 7. Andrassy RJ, Ko P, Hanson BA, et al. Congenital tracheoesophageal fistula without esophageal atresia. A 22-year experience. Am J Surg 1980; 140(6):731-3. 8. Lamb DS. A fatal case of congenital tracheo-esophageal fistula. Philadelphia Med Times 1873; 3:705. 9. Imperatori CJ. Congenital tracheo-esophageal fistulae without atresia of the esophagus. Arch Otolaryng 1939; 30:352-9. 10. Ware GW, Cross LL. Congenital tracheo-esophageal fistula without atresia of the esophagus. Pediatrics 1954; 14:254-61. 11. Schneider KM, Becker JM. The “H-type” tracheoesophageal fistula in infants and children. Surgery 1962;51:667-86. 12. Killen DA, Greenlee HB. Transcervical repair of H-type congenital tracheo-esophageal fistula: review of the literature. Ann Surg 1965; 162:145-50.

Annals of Saudi Medicine, Vol 6 No. 2; 1986

H-Type Congenital Tracheoesophageal Fistula in a Saudi Child

13. Johnston PW, Hastings N. Congenital tracheoesophageal fistula without esophageal atresia. Am J Surg 1966; 112:233-40. 14. Kappelman MM, Dorst J, Haller JA, Stambler A. H-type tracheo-esophageal fistula. Diagnostic and operative management. Am J Dis Child 1969; 118:568-75. 15. Bedard P, Girvan DP, Shandling B. Congenital H-type tracheo-esophageal fistula. J Pediatr Surg 1974; 9:663-8. 16. Gans SL, Johnson RO. Diagnosis and surgical management of “H-type” tracheoesophageal fistula in infants and children. J Pediatr Surg 1977; 12:233-6. 17. Benjamin B. Endoscopy in congenital tracheal anomalies. J Pediatr Surg 1980; 15(2): 164-71. 18. Hicks LM, Mansfield PB. Esophageal atresia and tracheoesophageal fistula. Review of thirteen years experience. J Thorac Cardiovasc Surg 1981 ; 81(3) :358-63. 19. Ein SH, Friedberg J. Esophageal atresia and tracheoesophageal fistula: review and update. Otolaryngol Clin North Am 1981; 14(l):219-49. 20. Yazbeck S, Dubuc M. Congenital tracheoesophageal fistula without esophageal atresia. Can J Surg 1983; 26(3):239-41. 21. Louhimo I, Lindahl H. Esophageal atresia: primary results of 500 consecutively treated patients. J Pediatr Surg 1983; 18(3):217-29. 22. Olivet RT, Payne WS. Congenital H-type tracheoesophageal fistula complicated by achalasia in an adult: report of a case. Mayo Clin Proc 1975; 50(8) :464-8. 23. Stephens RW, Lingeman RE, Lawson LJ. Congenital tracheoesophageal fistulas in adults. Ann Otol Rhinol Laryngol 1976; 85(5 Pt.l):613-7. 24. Mancrief JA, Randolph JG. Congenital tracheoesophageal fistula without atresia of the esophagus. A method for diagnosis and surgical correction. J Thorac Cardiovasc Surg 1966; 51:434-42. 25. Abbott OA. Abnormal esophageal communication. Their type, diagnosis and therapy. J Thorac Surg 1945; 14:382-92. 26. Korones SB, Evans LJ. Measurement of intragastric oxygen concentration for the diagnosis of H-type tracheoesophageal fistula. Pediatrics 1977; 60(4):450-2. 27. Holinger PH, Brown WT, Maurizi DG. Endoscopic aspects of post-surgical management of congenital esophageal atresia and tracheoesophageal fistula. J Thorac Cardiovasc Surg 1965; 49:22-32. 28. Weber TR, Smith W, Grosfeld JL. Surgical experience in infants with the VATER association. J Pediatr Surg 1980; 15(6): 849-54.

Annals of Saudi Medicine, Vol 6 No. 2; 1986

Suggest Documents