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Pediatr Surg lnt (1987) 2:216-222 Pediatric Surgery
Oesophageal atresia without fistula- anastomosis or replacement?
N. A. Myers, s . w . Beasley, A. W. Auldist, M. Kent, V. Wright*, and P. Chetcuti
Department of Surgery, Royal Children's Hospital, Flemington Road, Parkville, Victoria 3052, Australia
Abstract. At the Royal Children's Hospital, Mel- bourne, 553 babies with oesophageal atresia and / or tracheo-oesophageal fistula have been admit- ted during the past 39 years; 36 (6.5%) of these had oesophageal atresia without a tracheo-oeso- phageal fistula. Definitive surgery was performed in 27 patients: the primary definitive procedure was oesophageal anastomosis in 15 and oesopha- geal replacement in 12. Aspects of diagnosis and selection of the most appropriate treatment mo- dality are discussed, with the results of treatment presented. Our current policy is to perform an oesophageal anastomosis whenever possible, and this has been successful in 7 of the last t 1 patients.
Key words: Oesophageat atresia - Gasless abdo- men - Oesophageal replacement - Long gap
Introduction
Oesophageal atresia without an accompanying tracheo-oesophageal fistula is a relatively rare anomaly and varies between 5% and 10% in most reported series of patient~ with oesophageal atre- sia and tracheo-oesophageal fistula [1, 2, 5, 6, 10, l l , 14, 15, 17, 19, 20, 24, 26, 33, 35, 38]. Many centres therefore have a limited experience of the anomaly; at the Royal Children's Hospital in Melbourne there have been 36 patients who have had atresia without fistula. Thus, we have aver- aged tess than one patient a year during a period which has seen many advances in neonatal care.
*Present address: Queen Elizabeth Hospital for Children, Hackney Road, London E2 8PS, UK. Offprint requests to: N.A. Myers
Despite these advances and increasing experience controversy still exists as to the most appropriate management of the condition [3, 4, 7, 9, 12, 13, 25, 27, 28, 32]. This paper reports our experience and suggests a protocol which may assist in deter- mining whether primary anastomosis or replace- ment is appropriate.
Materials and methods
During the period 1948 - 1986, a total of 553 babies with oeso- phageal atresia and/or tracheo-oesophageal fistula were ad- mitted to the Royal Children's Hospital, Melbourne. Oe- sophageal atresia without fistula was seen in 36 (6.5%) of these (Table I).
Much of the data was collected prospectively by one of the authors (NAM) and supplemented by careful review of each patient's hospital records.
Oesophageal atresia with distal tracheo-oesophageal fistula
Oesophageal atresia with proximal tracheo-oesophageal fistula
Oesophageal atresia with proximal and distal tracheo-oesophageal fistula
Oesophageal atresia without fistula
Tracheo-oesophageal fistula ("H')
Total
475
11
1
36
30
553
Results
Polyhydramnios was present in 34 patients. Birth weight ranged from 1.6 to 3.6 kg (mean = 2.3 kg). One or more associated congenital anomalies were re¢ognised in 21 patients (Table 2). Of 3 t pa- tients in whom the gestation was recorded, t7 (55%) were tess than 37 weeks-
Table 2. Associated congenital anomalies in 21 patients
~3~ 1 I NO DEFINITIVE OESOPHAGO- OESOPHAGO ** PROSTHETIC SURGERY (TABLE 3} COLOPLAST¥ GASTROPLASTY OESOPHAGUS
9 5 6 1 (9) (4) (1 ) (1)
Fig. 1. Management of the 36 patients (9: no definitive sur- gery; 27: alimentary continuity established). Number of deaths indicated in parenthesis. *2 cases required oesophageal re- placement; **one case required later coloplasty
217
A definitive attempt to provide alimentary continuity was made in 27 patients: the remaining 9 either had no surgery or a gastrostomy and /o r oesophagostomy alone (Table 3).
The management of patients in whom a defin- itive attempt was made to establish continuity is summarised in Fig. 1. One colonic replacement followed failed gastric replacement, and one greater curvature gastric tube followed failure of a Rehbein procedure. The prosthetic oesophagus using polythene was inserted in 1953. Two pa- tients who had a primary oesophageal anastomo- sis subsequently required oesophageal replace- ment. In 1 patient excision of a complete oeso- phageal web was the only procedure required.
Oesophageal anastomosis was achieved in 2 patients by mobilisation of the lower oesophagus through an abdominal approach at the same time as thoracotomy. The lower oesophagus was freed
Recent diagnosis of oesophagitis. 15 Recent rectoplasty
Occasional regurgitation and heartburn. 14 Food sticks occasionally. Drinks with meals
No recent information 10
Regurgitation a problem and food sticks "about once a week"
within the hiatus and allowed to ride up into the chest, facilitating direct anastomosis of the oe- sophageal ends through the thoracotomy incision.
There were 6 deaths in the 12 patients with "primary" oesophageal replacement. The further 2 patients in whom primary anastomosis was un- successful, necessitating subsequent oesophageal replacement, are alive, bringing to 8 the total number of survivors with oesophageal replace- ment.
There were 3 deaths in the 15 patients having an oesophageal anastomosis: 2 died of complica- tions of the procedure and 1 of congenital sub- glottic stenosis. Ten are alive with a functioning oesophagus and 2 required replacement. The age at anastomosis varied from 4 weeks to 16 weeks (Table 4). The baby with a complete oesophageal web was repaired during the 1st week of life
Follow-up has ranged from 1 to 27 years: de- tails are summarised in Tables 5 and 6.
D i s c u s s i o n
Until the 1960s, oesophageal anastomosis in oe- sophageal atresia without fistula was considered impossible and the standard management in- volved establishment of a salivary fistula in the neck as a cervical oesophagostomy. In 1965 Koop and Hamilton [16, 22] introduced the concept of "staged" management for oesophageal atresia with distal fistula, advocating early gastrostomy
219
followed by a period of waiting before oesopha- geal anastomosis. This concept was extended to the baby with atresia without fistula. Preservation of the upper pouch produced new problems: the baby had to remain in hospital; there was risk of aspiration from accumulation of saliva in the upper pouch; and the child could not "sham feed". Aspiration was reduced by instituting an intensive programme of nursing care, attention to the posture of the baby, and continuous or fre-
Fig. 2. Contrast study of upper oesophageal pouch in baby with airless abdomen, Upper pouch fistula excluded
Fig. 3. Metal bougie in upper pouch - baby had airless abdomen
Fig. 4. X-ray taken at time of gastrostomy; sounds in both oesophageal segments
Fig. 5. Sounds introduced into both oesophageal segments; antero-posterior view in baby aged 3 weeks (same patient as Fig. 4)
220
quent suction of the upper pouch. If further eval- uation showed that oesophageal anastomosis was not feasible, a cervical oesophagostomy was per- formed. In 1963 upper pouch bougienage was in- troduced. In retrospect, we believe that the main advantage of bougienage was to encourage upper pouch preservation in babies who would other- wise have had a cervical oesophagostomy.
The use of upper pouch bougienage as first de- scribed by Howard and Myers [21] led to the de- velopment of other techniques such as Rehbein's Olive technique [29, 30], electro-magnetic bougie- nage [18], and circular myotomy [23, 31, 34, 36, 37, 39], each of which had its proponents. We no longer perform bougienage, but do encourage large bolus gastrostomy feeds with the object of promoting gastro-oesophageal reflux in the belief that spontaneous growth of the lower oesophageal segment may be increased by reflux.
Once a child with oesophageal atresia has been shown to have a gasless abdomen two ques- tions need answering: (1) is there a proximal tracheo-oesophageal fistula present? This is de- monstrated by a careful upper pouch contrast study and in our experience about one in four will have an upper-pouch fistula [8]; and (2) what is the length of the gap between the oesophageal ends and is it such that a primary anastomosis can be performed?
The gap between the oesphageal segments in isolated oesophageal atresia tends to be much greater than when a tracheo-oesophageal fistula is present. However, we know from our own experi- ence that direct end-to-end oesophageal anasto- mosis is possible in many of these patients and has been successfully performed in 7 of our last 11 patients.
To establish the suitability for anastomosis it is necessary to evaluate the lengths of both oesopha- geal segments. Initially, the length of the upper segment is shown by contrast study (Fig. 2) and subsequently by the use of a metal bougie (Fig. 3). The length of the lower segment is evaluated at the time of gastrostomy, by the introduction of a bougie or sound, and this can be combined with the simultaneous introduction of a bougie into the upper segment by the anaesthetist (Fig. 4). Further evaluation is performed at monthly intervals by simultaneous insertion of metal sounds into both oesophageal segments until a decision is made re- garding the definitive operative management (Figs. 5-7). After 3 months further decrease in the gap is unlikely. The vertebral column is used as the reference point for estimating the gap between the segments.
Fig. 6. Sounds introduced into both oesophageal segments; lateral view in baby aged 4 weeks (same patient as Fig. 4)
Fig. 7. Lateral view with sounds in both segments in baby aged 4 weeks. Ruler to facilitate assessment of progress
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I GAP LESS THAN TWO VERTEBRAE
PROCEED TO ANASTOMOSIS
OESOPHAGEAL ATRESIA GASLESSARDOMEN
DYE STUDY OF UPPER POUCH TO EXCLUDE PROXIMAL FISTULA
GASTROSTOM¥
I EVALUATE LENGTH OF OESOPHAGEAL
SEGMENTS
t GAP BETWEEN TWO AND FOUR VERTEBRAE
RE-EVALUATE AT MONTHLY INTERVALS UP TO THREE MONTHS
1 PROCEED TO ANASTOMOSIS
I GAP GREATER THAN FOUR VERTEBRAE
I RE-EVALUATE AT MONTHLY INTERVALS UP TO THREE MONTHS
I PROCEED TO ANASTOMOSIS +/-- INTRA ABDOMINAL MOBILISATION OF LOWER OESOPHAGUS
I IF FAILS
CERVICAL OESOPHAGOSTOMY AND SUBSEQUENT OESOPHAGEAL REPLACEMENT
Fig. 8. Oesophageal atresia with airless abdomen: scheme of management
In our experience a gap of less than 2 verte- bral bodies justifies oesophageal anastomosis (Fig. 8). A gap between 2 and 4 vertebrae merits monthly re-evaluation in the expectation that anastomosis will be possible at a later stage even if it necessitates full mobilisation of the lower seg- ment. We have performed abdominal mobilisa- tion of the lower end of the oesophagus to allow successful intrathoracic oesophageal anastomosis on two occasions. Both patients have subsequent- ly required an abdominal Nissen fundoplication for severe gastro-oesophageal reflux but are now well. Circular myotomy was not used in this se- ries, although other authors [23, 31] have suggest- ed it may facilitate anastomosis when the gap is large.
When the radiological or operative evidence indicates that anastomosis is unlikely to succeed, or in the event of early and major anastomotic disruption, cervical oesophagostomy should be performed with oesophageal replacement when the baby reaches 10 kg. Our current preference is for replacement by means of a greater curvature tube of stomach.
C o n c l u s i o n s
The diagnosis of oesophageal atresia without fis- tula should only be accepted after a careful upper
pouch study to exclude a fistula. Initial manage- ment should involve a gastrostomy with evalua- tion of both oesophageal segments at the time. If the gap between the ends is small (less than two vertebral bodies) anastomosis can proceed. If the gap is larger' re-evaluation at 4-weekly intervals is indicated, as the gap tends to decrease up till about 12 weeks of age. Successful anastomosis is precluded in most cases where the gap is more than 4 vertebral bodies in length.
Where an anastomosis is performed it is to be anticipatedthat gastro-oesophageal reflux will oc- cur and may necessitate anti-reflux surgery.
The long-term follow-up suggests that oesoph- ageal anastomosis is preferable to replacement, and in our current practice is possible in most in- stances.
Acknowledgements. The authors are grateful to Miss Vicki Holt who typed the manuscript, Miss Jocelyn Brady, Research As- sistant, and to the Department of Radiology and Education Resource Centre, Royal Children's Hospital. The study was supported by a grant from the Royal Children's Hospital Re- search Foundation and Oesophageal Atresia Foundation.
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