Livaditis myotomy in long-gap esophageal atresia

Livaditis myotomy in long-gap esophageal atresia

Livaditis Myotomy in Long-Gap Esophageal Atresia By Harry Lindahl and Ilmo Louhimo Helsinki, Finland (}During 1973 to 1983, Livaditis m y o t o m y wa...

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Livaditis Myotomy in Long-Gap Esophageal Atresia By Harry Lindahl and Ilmo Louhimo Helsinki, Finland (}During 1973 to 1983, Livaditis m y o t o m y was performed on 12 esophageal atresia patients in w h o m anastomosis otherwise would have been impossible. Ten patients had the usual malformation with a distal fistula, while t w o had an isolated atresia. Four patients belonged to W a t e r ston's risk group A, 4 to group B, and 4 to group C. In 11 cases anastomosis was permitted by myotomy. In one patient anastomosis was impossible even after myotomy. This baby weighed 1,380 g and she also had trisomy 18 with severe cardiac and renal anomalies leading to death. One patient had a refistula and died of pneumonia, she also had a severe congenital cardiac malformation. There w e r e no anastomotic leakages to free pleural cavity nor complications related to the myotomy. The ten survivors w e r e followed up for a mean period of 5.4 years (range 1.6 to 11.3 years). The subjective results at last follow-up according to the criteria of Desjardins w e r e excellent in eight and good in t w o patients. Esophagograms showed no strictures, but t w o patients had a m y o t o m y pouch. 9 19B7 by Grune & Stratton, Inc. INDEX W O R D S : Esophageal atresia; circular myotomy.

INCE ITS D E S C R I P T I O N ? circular myotomy of the upper segment has been used widely to achieve anastomosis in long gap esophageal atresia. 2-4 However, knowledge of the long-term outcome is still scarce and the reports of long-term results are conflicting) '6 The aim of this paper is to discuss both shortterm and long-term results of this procedure in light of our experience since 1973.

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MATERIALS A N D METHODS

During 1973 to 1983, 142 esophageal atresia patients were operated on at Helsinki Children's Hospital. Livaditis myotomy was performed on 12 patients (8 girls and 4 boys). Myotomy was used only when anastomosis otherwise was impossible because of a long gap. Ten patients had the usual malformation with a distal fistula, while two patients had an isolated atresia. The mean birth weight of the patient group was 2,436 g (range 1,380 to 3,210 g). Three patients had serious associated anomalies: one had an atrial septal defect and cleft lip and palate. One had Fallot's tetralogy with a vascular ring and partial aplasia of the vena cava inferior, cleft lip and palate and bilateral coloboma, and one patient had 18 trisomy, ventricular septal defect, mitral stenosis, malformation of aortic and pulmonary valves, hydronephrotic single kidney, and genital anomalies. In addition, one was suspected of fetal alcohol syndrome. Four patients belonged to Waterston's risk group A, four to group B, and four to group C. All patients were operated on transpleurally. The anastomosis was made with single-layer interrupted sutures. The ten patients with distal fistula were operated on immediately after their arrival at hospital, two on their day of birth, and the remaining eight at the age of one day. Gastrostomy was not performed on these patients, but a silicone nasogastric tube was introduced through the anastomosis and kept in suction for five days, after which feeding was started through the tube. The two patients with isolated atresia were Journal of Pediatric Surgery, Vol 22, No 2 (February), 1987: pp 109-112

primarily treated with feeding gastrostomy and upper pouch suction. Anastomosis was performed on both of them at the age of 3~/2 months. Routine postoperative dilatations were performed on all patients. Primary results were collected from hospital records. All surviving patients had regular postoperative checkups and they were followed up for a mean of 5.4 years (1.6 to 11.3 years). Routine postoperative esophagograms with colloidal barium sulphate under fluoroscopic control were performed on all patients. The mean age at the last fluoroscopic examination was 4.2 years (1.5 to 9.1 years). Information was obtained for the last follow-up in four cases by personal interview and in six by a questionnaire. Growth data were obtained from hospital records, children's welfare centers, and school health authorities. RESULTS

Primary Results A n a s t o m o s i s was p e r f o r m e d in 11 of the 12 cases. In one case a n a s t o m o s i s was i m p o s s i b l e even after m y o t o my, t h e r e f o r e seclusion of the fistula, g a s t r o s t o m y , a n d cervical e s o p h a g o s t o m y were p e r f o r m e d . T h i s b a b y w e i g h e d 1,380 g a n d also h a d t r i s o m y 18 s y n d r o m e , v e n t r i c u l a r septal defect, stenosis of the m i t r a l valve, m a l f o r m a t i o n of a o r t i c a n d p u l m o n a r y valves, h y d r o n e p h r o t i c single k i d n e y , a n d g e n i t a l a n o m a l i e s . S h e died of these associated m a l f o r m a t i o n s two d a y s after the operation. O n e p a t i e n t h a d refistula c a u s i n g p n e u m o n i a , which t o g e t h e r with F a l l o t ' s tetralogy, v a s c u l a r ring, a n d p a r t i a l a p l a s i a of the inferior v e n a c a v a c a u s e d the d e a t h of this f u l l - t e r m b a b y . T h r e e p a t i e n t s h a d p o s t o p e r a t i v e p n e u m o n i a , w h i c h resolved with a n t i b i o t i c t r e a t m e n t . T h e p a t i e n t with s u s p e c t e d fetal alcohol s y n d r o m e h a d a n u n e x p l a i n e d asystole at one h o u r after t h e o p e r a t i o n a n d was successfully resuscitated. T h e r e were n e i t h e r a n a s t o m o t i c l e a k a g e s to the free p l e u r a l c a v i t y n o r c o m p l i c a t i o n s r e l a t e d to the m y o t o m y . A total of t e n p a t i e n t s were d i s c h a r g e d f r o m the hospital with a successful a n a s t o m o s i s . Long-Term Results Late complications. N o late d e a t h s ing the follow-up period. T h r e e p a t i e n t s tions d u e to p a t h o l o g i c g a s t r o e s o p h a g e a l t h e m h a d tight a n a s t o m o t i c strictures,

occurred durhad complicareflux. T w o of w h i c h did n o t

From the Helsinki University Central Hospital, Children's Hospital, Helsinki, Finland. Address reprint requests to Harry Lindahl, MD, Department of Pediatrics, Division of Surgery, Turku University Central Hospital, SF 20520 Turku 52, Finland. 9 1987 by Grune & Stratton, Inc. 0022-3468/87/2202~9002503.00/0 109

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respond to dilatations and one had distal hemorrhagic esophagitis. All three underwent Nissen's fundoplication. After fundoplication both anastomotic strictures responded to dilatation, and the distal esophagitis healed. However, the patient with distal esophagitis had recurrent hiatal hernia and refundoplication was performed at the age of 21/2 years. She has been well subsequently. Three patients had an episode of a swallowed foreign body requiring endoscopic removal. All these incidents occurred at the age of 9 to 13 months. Three patients had recurrent respiratory infections that subsided without treatment after the age of 3 to 4 years. Last esophagograms. No strictures were detected. All patients showed pathologic esophageal motility. In two patients gastroesophageal reflux could be demonstrated to the level of the clavicle; neither of these patients had, however, any symptoms of it. Two patients had asymptomatic bulging at the site of the myotomy. In one further patient bulging was observed on an earlier esophagogram at the age of 6 months. This had disappeared at the age of 11/2years. Figure 1 shows the largest myotomy pouch that was detected. Last follow-up. Altogether ten patients were left for follow-up. The subjective results at the end of the follow-up period were classified according to Desjar-

dins et al. 5 An excellent result was one free of any esophageal or respiratory symptoms. The result was considered good if the patient had only occasional dysphagia. A fair result was one with frequent swallowing difficulties or respiratory symptoms. Eight patients had an excellent result. Two patients had occasional slight dysphagia and the result was classified as good. The patient who had postoperative asystole and required resuscitation showed an excellent result in view of the condition of his esophagus, but had mild diplegia for which he is receiving physiotherapy. Growth. Figure 2 shows the last height and weight measurements. As can be seen, the patients have grown normally. DISCUSSION

Long-gap esophageal atresia presents a technical difficulty for which several methods of treatment have been devised. Steele 6 was the first to face this problem by trying to push the two segments of the atretic

9 BOYS

9 GIRLS

HEIGHT

L

0

Fig 1.

Esophagogram of the largest myotomy pouch.

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10 AGE ,YEARS

Fig 2. Growth: The last measurements of height and weight. The shaded area represents _+ 2 SD of normal Finnish children.

LIVADITIS M Y O T O M Y

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esophagus together with two bougies. Anastomosis has been achieved by means of gastric mobilization, 7,s but complications associated with an intrathoracic stomach have made this technique less attractive, s-l~ Bouginage of esophageal segments, 11-13 or mere waiting for the segments to grow 14'15have been used successfully in long-gap atresia. Circular myotomy ~ permits anastomosis in most cases, even those with a very long gap. 4 Primary anastomosis can be almost always achieved in patients with a distal fistula. The only exception in this material was a very small premature infant. Combined with bouginage of the segments or mere waiting, circular myotomy seems to enable anastomosis even in most cases of isolated atresia. Instead of esophageal substitution we now try to save the patient's own esophagus. Some authors 2'3'16 have used multiple myotomies to elongate the upper segment. Only a single myotomy was used in this material. However, after the present series, there have been two further patients with isolated atresia both undergoing successful anastomosis after waiting. In the first, a single myotomy was used, but in the other, a second myotomy was necessary, which was made through a separate neck incision. This myotomy yielded about 4 to 5 m m length. The problem with multiple myotomies is the shortness of the upper segment, which usually makes a second myotomy impossible in cases where it is most needed. The absence of anastomotic leakages is noteworthy, especially as all these anastomoses were made under considerable tension. In comparison, the frequency of anastomotic leakage in our unselected material is a b o u t 12%. 17 Perforations at the myotomy site have been reported; 3'ls in our material there were none. There was one refistula leading to pneumonia, which together with other serious anomalies resulted in the patient's death. The other death in this series was caused by associated malformations incompatible with life. Long-term follow-up studies of Livaditis myotomy patients have been few and conflicting. In Janik and coworkers '3 series of six patients, the long-term (1 to 4

years) outlook was good and corresponded to the outlook of esophageal atresia patients whose anastomoses have been achieved without a myotomy. In contrast, Otte and coworkers 19 reported severe symptomatic ballooning of the myotomy site in two of their six patients. In one, the pseudodiverticula caused severe dysphagia by the age of 16 months and in the other life-threatening respiratory distress by the age of 3 months. In both cases the esophagus finally had to be excluded and continuity was restored by means of esophageal substitution. Our results agree with those of Janik and coworkers. Most of our patients are thriving and their outlook does not differ from that of patients with anastomosis without a myotomy. 2~ Gastroesophageal reflux seems to be frequent in patients with myotomy. This is, however, not a complication of the procedure, but rather of the primary condition. In these cases the anastomosis is usually made under considerable tension and this probably pulls up the cardioesophageal junction, thus shortening the intra-abdominal esophagus and opening the angle of His, with resultant pathologic gastroesophageal reflux. Three of our patients suffered from this problem. All of them are well after surgical correction, although one patient had to be fundoplicated twice. We feel that these patients have to be watched for pathologic gastroesophageal reflux even more carefully than children with normal esophageal atresia. Strictures were absent at last esophagograms and contrary to Otte and coworkers '19 series there have been no problems with myotomy site bulging. Two of our patients had slight bulging without symptoms and in one further case the bulging disappeared spontaneously. The growth of myotomy patients does not differ from that of patients in whom anastomosis has been achieved without a myotomy. 2~ In conclusion, Livaditis myotomy is a safe and effective method of achieving anastomosis in long gap esophageal atresia. The long-term outlook of patients treated with Livaditis myotomy and anastomosis does not differ from the outlook of those with anastomosis achieved without myotomy.

REFERENCES

1. Livaditis A: Esophageal atresia: A method of overbridging large segmentalgaps. Z Kinderchir 13:298-306, 1973 2. de Lorimer AA, Harrison MR: Long gap esophageal atresia. J Thorac CardiovascSurg 79:138-141, 1980 3. Janik JS, Filler RM, Ein SH, et al: Long-term follow-up of circular myotomy for esophageal atresia. J Pediatr Surg 15:835841, 1980 4. Ricketts RR, Luck SR, RaffenspergerJG: Circular esophagomyotomy for primary repair of long-gap esophageal atresia. J Pediatr Surg 16:365-369, 1981 5. Desjardins JG, Stephens CA, Moes CAF: Results of surgical

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gastric tube in infants and children. J Pediatr Surg 3:246-252, 1968 10. Cohen DH, Middleton AW, Fletcher J: Gastric tube esophagoplasty. J Pediatr Surg 9:451-460, 1974 11. Howard R, Myers NA: Esophageal atresia: A technique for elongating the upper pouch. Surgery 58:725-727, 1965 12. Johnston PW: Elongation of the upper segment in esophageal atresia: Report of a case. Surgery 58:741-744, 1965 13. Hendren WH, Hale JR: Esophageal atresia treated by electromagnetic bouginage and subsequent repair. J Pediatr Surg 11:713-722, 1976 14. Hays DM, Woolley MM, Snyder WH: Esophageal atresia and tracheoesophageal fistula: Management of the uncommon types. J Pediatr Surg 1:240-252, 1966 15. Purl P, Blake N, O'Donnell B, et al: Delayed primary

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anastomosis following spontaneous growth of esophageal segments in esophageal atresia. J Pediatr Surg 16:180-183, 1981 16. Takada Y, Kent G, Filler RM: Circular myotomy and esophageal length and safe esophageal anastomosis: An experimental study. J Pediatr Surg 16:343-348, 1981 17. Lindahl H, Louhimo I: Anastomotic disruption and its management in oesophageal atresia patients with a special reference to re-suturation. Z Kinderchir Kongressberichte 1982 116-120, 1983 (suppl) 18. Vizas D, Ein SH, Simpson JS: The value of circular myotomy for esophageal atresia. J Pediatr Surg 13:357-359, I978 19. Otte JB, Gianello P, Wese FX, et al: Diverticulum formation after circular myotomy for esophageal atresia. J Pediatr Surg 19:68-71, 1984 20. Lindahl H: Long-term prognosis of successfully operated oesophageal atresia--With aspects on physical and psychological development. Z Kinderchir 39:6-10, 1984