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Improved Outcome in Management of Oesophageal Atresia with or without Tracheo-Oesophagial Fistula

Gamal Al-Saied¹ and Ali Alzhrani²

¹Department of Pediatric Surgery - Al-Azhar University, Cairo, Egypt

²Department of Neonatal Intensive Care Unit (NICU)

¹,²King Abdul Aziz Specialist Hospital, Taif, Saudi Arabia.

 

Correspondence

Gamal Al-Saied,
King Abdul Aziz Specialist Hospital, Taif, Saudi Arabia, POB: 10127,
Email: This email address is being protected from spambots. You need JavaScript enabled to view it. ,
Tel.: + 96627310998,
Mobile: + 966508566499

 

Abstract

Background: Oesophageal Atresia (OA) with or without Tracheo-Oesophageal Fistula (TOF) is a rare congenital anomaly. The survival rate of infants with OA with or without TOF has improved over the past few decades. The aim of this study was to critically review our surgical experience of the last 5 years to evaluate the outcome in management of OA with or without TOF in our region in comparison with the international results.

Patients and Methods: This work was carried out at King Abdul Aziz Specialist Hospital, Taif, Saudi Arabia in the period from December 2001 through December 2006. The period of follow up was 50 months on average. Thirty two neonates (21 males and 11 females) diagnosed as OA with or without TOF. After stabilization, 27 cases were treated by oesophago-oesophageal anastomosis via posterolateral thoracotomy, extrapleural approach, 5 cases were treated by staged procedure (ligation of fistula, cervical oesophagostomy and feeding gastrostomy) due to long gap oesophageal atresia and marked prematurity. The other 4 extremely low birth weight babies passed away 4-6h after birth due to cardiopulmonary failure.

Results: 11 (34.38%) cases developed gastro-oesophageal reflux, 2 cases (6.25%) developed minimal leakage postoperatively which healed spontaneously by conservative treatment,6(18.75%) cases developed pneumonia, 3 cases (9.38%) developed stricture resolved by regular balloon dilatation. Death has occurred in 2(6.25%) cases postoperatively and 4 (12.50%) cases before definitive surgery due to severe sepsis and cardiopulmonary failure. There was no single case of fistula recurrence or redo. Our survival rate in comparison with the published international survival rates using Waterston, Spitz and Bremen prognostic classification are summarized in tables (3,4 and5) respectively.

Conclusion: On the basis of this critical retrospective review of our surgical experience over the last 5 years, our results are comparable with the published international survival rate. We believe that the advances in neonatal intensive care, pediatric anesthesia, and refinements in surgical techniques have contributed to improve outcomes of oesophageal atresia.

Key words: Oesophageal Atresia , Tracheo-Oesophageal Fistula


Introduction:

The survival rates of oesophageal atresia have continued to improve since the first successful repairs in the 1940s, transforming this entity from an anomaly with virtually 100% mortality rate to one with expected survival rates exceeding 90%.[2-5] This improvement is due to the advances in neonatal intensive care, ventilator and nutritional support, pediatric anesthesia and refinement of surgical techniques in management of esophageal atresia and its associated anomalies.(8) Several prognostic classification systems have been described to assess the severity and predict the outcomes for neonates with esophageal atresia.[4-7] The aim of this study was to critically review our surgical experience of the last 5 years to evaluate the outcome in management of OA with or without TOF in our region in comparison with the international results.

Patients and Methods

This retrospective study was carried out at King Abdul Aziz Specialist Hospital, Taif, Saudi Arabia, in the period from Dec. 2001 through Dec. 2006. The period of follow up was 50 months on average. The medical records of thirty two neonates diagnosed as OA with or without TOF were reviewed regarding the type of OA, gestational age, birth weight, and age at diagnosis, gender, associated anomalies, and pulmonary status, type of surgical approach, morbidity and mortality. The study was reviewed and approved by the review board and written consent of patient’s parents was taken in accordance with the human rights committee guidelines of the hospital.

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Figure 1A. Right Postero-lateral thoracotomy, extrapleural approach through the 4th intercostal space. Ligation and division of azygos vein to facilitate access for TOF (arrow).


Table 1. Preoperative data and operative approaches in our study.

Total No. of cases 32 neonates
Type of oesophageal anomaly 28 (87.5%) OA with distal TOF &
4 (12.5%) isolated atresia
Gender 21 (65.62%) boys &
11 (34.38%) girls (♂ to ♂ ratio is 1.90 to 1.00)
Associated anomalies 14 ( 43.75%) Cardiac, 9(28.12%)GIT, 3(9.38%) Skeletal deformity,
www.jpedss.com
Genitourinary 5(15.62%) & 1(3.13%) Down’s Syndrome.
Gestational age 36.8 wk (34–38)
Birth weight 2.1 Kg (range: 0.6 kg to 2.5 kg)

 Esophageal gap ≤ 3 cm (27 cases 84.37%) &
> 5 cm (5 cases 15.63%)
 Oesophago-oesophageal anastomosis Early in 27 (84.38%) 
 Gastrostomy with or without cervical
oesophagostomy
5 cases (15.63%) 

Table 2. Postoperative complications

Gastro-oesophageal reflux 11/32 cases (34.38%)
Leakage 2/32 cases (6.25%)
healed spontaneously by conservative treatment (figure 3A, 3B)
Pneumonia 6/32 cases (18.75%)
Stricture 3/32 cases (9.38%)
treated by regular balloon dilatation
Fistula recurrence none
Redo none
Mortality 2 cases (6.25%) due to severe sepsis and cardiopulmonary failure.
4 (12.50%) cases (before definitive surgery).

Table 3. Comparison of our survival with Waterston Prognostic Classification

Waterston Prognostic Classification (n = 113)

Published SR

Our study (n = 32)

No

Death

SR

 

A-Birth wt >2.500 Kg and well       (n = 38)

(36/38)

95%

17

0

100%¹

B-Birth wt 1.800 to 2.500Kg and well or Birth

wt >2.500 Kg, but moderate pneumonia

and other congenital anomaly (n = 43)

(29/43)

68%

5

1

80%²

C-Birth wt <1.800Kg or Birth wt >1.800Kg with severe

pneumonia, and severe congenital anomaly (n = 32)

(02/ 32)

6%

10

5

50%³

1: z=0.14    p= 0.89     non-significant (NS)
2: z=0.04    p=0.97       (NS)
3: z=2.79     p=0.005     (significant)

 

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Figure 1B. A tape is placed around lower oesophageal segment to control the TOF before excision and closure of the fistula (arrow).

All cases were diagnosed initially by prenatal ultrasound (U/S) and confirmed postnatally by clinical examination and Chest x-ray, abdominal U/S and Echocardiography (ECHO) and lab study. After proper pre-operative preparation and stabilization, 27 cases were treated by oesophago-oesophageal anastomosis using single layer of interrupted 5/0 polyglactin suture (Vicryl) via posterolateral thoracotomy, 4th intercostal space, extrapleural approach. All patients were managed postoperatively in the neonatal intensive care unit (NICU). Intravenous fluids were administered and prophylactic broad spectrum antibiotic were continued until paranastomotic intercostal tube drain was removed. Regular chest physiotherapy with gentle naso-osophageal suction was done as required. Oesophageal anastomosis was performed under tension in 3 patients. So, they postoperatively kept electively paralyzed and mechanically ventilated for 5 days in order to minimize disruptive forces (hiccup and crying) to the site of anastomosis.

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Figure 1C. End to end oesophago-oesophgeal anastomosis with interrupted simple sutures of an absorbable suture (vicryl 4/0) nasogastric tube (arrow). Figure 1D. 7th day Post Operative Oesophagogram showing smooth passage of contrast without leakage or stricture.

 

Table 4. Comparison of our survival rate (SR) with Spitz Prognostic Classification

Spitz Prognostic Classification (n = 372)

Published SR

Our study (n = 32)

No

Death

SR

 

I- Birth wt >1.500 Kg, without major cardiac disease (n = 293)

(283/293) 97%

22

1

95.46 %¹

II- Birth wt <1.500 Kg, or major cardiac disease (n = 70)

(41/70) 59%

7

3

57.14 %²

III-Birth wt <1.500 Kg and major cardiac disease (n = 09)

(2/9) 22%

3

2

33.33 %³

1: z=0.24      p=0.81    (NS)
2: z= 0.31        p=0.76   (NS)
3: z=  0.38       p=0.71   (NS)

The transanastomotic tube was removed and contrast study done on 7th postoperative day to rule out anastomotic leak before starting feeding (Fig. 1A, 1B, 1C&1D). Five cases were treated by staged procedure (feeding gastrostomy with or without cervical oesophagostomy) due to long gap oesophageal atresia (Fig 2A&2B). All patients were kept on regular follow up in outpatient clinic.

Statistical analysis was done using the Z test for proportions. The results were considered significant when p<=0.05

Results: The preoperative data and operative approaches in our study are shown in table 1, postoperative complications are shown in table 2. Tables 3,4 and 5 summarize our survival rate (SR) in comparison with the published international survival rates using Waterston, Spitz and Bremen prognostic classification respectively. Postoperative complications are also shown in table 2.

Table 5. Comparison of our survival rate (SR) with Bremen Prognostic Classification.

Bremen Prognostic Classification         (n = 113)

 

Published SR

Our study (n=32)

A-Without complications               (n = 100)

No

Death

SR (%)

I-Birth wt >1.500 Kg, without major cardiac disease (n=80)

(80/80) 100%

20

0

100%

II-Birth wt <1.500 Kg, or major cardiac disease (n = 18)

(9/18) 50%

4

2

50%¹

III-Birth wt <1.500 Kg and   major cardiac disease (n = 02)

(00/02) 0%

2

1

50%²

B-With complications (ie, Preop RDS/ pneumonia) (n = 13)

SR

No

Death

SR

I-Birth wt >1.500 Kg, without major cardiac disease (n = 06)

(02/06) 33.3%

2

1

50%³

II-Birth wt <1.500 Kg, or major cardiac disease (n = 05)

(2/5) 40%

3

1

67%

III-Birth wt <1.500Kg and major cardiac disease (n = 02)

(0/2) 0%

1

1

0%

1: z=0.55 p= 0.58 (NS)

2: z=0.0 p= 1.00 (NS)

3: z=0.42 p= 0.67 (NS)

4: z=0.01 p=0.99 (NS)

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Figure 2A. Cervical oesophagostomy Figure 2B. Feeding gastrostomy

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Figure 3A. Oesophagogram done on 5th day post-op showing leakage of contrast (arrow)

Figure 3B. Oesophagogram of the same patient showing smooth passage of the oral water soluble contrast (no leakage of the contrast or stricture)

Discussion

Oesophageal atresia affects approximately 1 in 4,000 to 4,500 neonates [1]. Brown et al stated that the incidence of first-degree relatives having 1 or more components of the VACTERL association was 5.5% in the esophageal atresia [2]. Many prognostic classification systems have been described to assess the severity and predict the outcomes for neonates with esophageal atresia [4-7].

The Waterston prognostic classification for survival of neonates with OA was proposed in 1962 and still widely used in many pediatric surgery centers [4,9]. The original survival rate (SR) in his 3 categories were 95%, 68%, and 6%, whereas the SR in our study in same 3 categories were 100%,80% and 50% respectively. Our survival rate is better in class C as p=0.005 which is significant, table 3. This significant difference could be attributed to the improvements in surgical techniques, anesthesia, and pre-and postoperative management of very low birth weight newborn and proper management of preoperative pneumonia in neonatal intensive care unit (NICU).

In 1994 Spitz [6] described prognostic classification and the original survival in his categories were 97%, 59% and 22% whereas in this study the SR in same 3 categories were 95.46%, 57.14% and 33.14% respectively, table 4. There was no significant difference between our SR and published SR in Spitz series in all classes.

In 2000 Yagyu et al described a new prognostic classification (Bremen classification) [7]. They classified their patients according to the absence or presence of preoperative pulmonary complications (pneumonia or respiratory distress syndrome RDS) in addition to the birth weight and associated cardiac anomalies. The published SR in their first category without pulmonary complications were 100% 50% and 00% in class I, II and III respectively. In this study, the SR in same category were 100%, 50% and 50%; table 5. In their 2nd category, the SR were 33%,40% and 00% in classes I, II&III respectively. Whereas our SR in same category were 50%, 67% and 00% in same classes, Table 5.

There was no significant difference between our SR and published SR in Yagyu et al series in all classes using Z test for proportions, because p value were more than 0.05 in all classes of Bremen prognostic classification, table 5. Pediatric anesthesia care has also improved in the last period with accurate management of oxygen for low-birth-weight patient, better capacity of monitoring, and improved equipment to prevent hypothermia[20,21]. Accumulated surgical experience, the improvements in suture materials, use of the finest instruments and the use of magnification tools have contributed to the improved outcomes of OA repair[22]. The thoracoscopic approach for OA repair might bring further improvement in the outcomes for these patients[23].

In 2006 Spitz have stated that the overall incidence of oesophageal leak is 10% of early anastomoses, most were minor, which sealed spontaneously and strictures at the anastomotic site were recognized in 37% of cases. Most were minor and responded to 1 or 2 dilatations [3,10,11]. In our study, 2(6.25%) cases developed minimal leakage postoperatively which healed spontaneously by conservative treatment and 3(9.38%) cases developed stricture that were treated by regular balloon dilatation.

In same report of Spitz published in 2006, he mentioned that significant reflux occurs in 40% of infants after repair of esophageal atresia; half of them required antireflux surgery after failure of intensive antireflux therapy[3,12]. In our study, 11(34.38%) cases developed gastro-oesophageal reflux, 9 cases of them treated conservatively and the other 2 cases required floppy Nissen fundoplication at the age of 2 year.

Although early repair is the favored method of treatment for most infants, a staged approach is still recommended for infants with homodynamic instability, marked prematurity and long gap OA. The staged approach consisted of a feeding gastrostomy and cervical oesophagostomy in the neonatal period. However, terminal oesophagostomy almost invariably leads to the need for an esophageal replacement procedure using the colon or stomach. Alternative methods to preserve the native esophagus, such as lengthening procedures [13,14] or lateral oesophagostomy in the proximal pouch [15] have been described. However, delayed repair in several months remains the preferred procedure for many surgeons where definitive surgery is delayed until the gap narrows enough to permit oesophago-oesophgeal anastomosis[16] In another study, there were some cases in which the repair was delayed because of prematurity or other complicating factors [1].

In this study, early oesophago-oesophageal anastomosis was performed in 27(84.38%) cases and staged repair (feeding gastrostomy with or without cervical oesophagostomy) was done for 5(15.63%) cases because of long gap oesophageal atresia and marked prematurity

There is a group of infants with OA with lethal congenital anomalies for whom surgery would be of no benefit. Those include major cerebral anomalies, grade IV intraventricular haemorrhage, pulmonary atresia, trisomy 18, and complex CHARGE (coloboma, heart anomaly, atresia, retardation, genital anomaly, and ear anomaly) syndrome[6,18[. These infants should not be included in survival statistics[22].

In our study, we excluded 4 extremely low birth weight babies passed away 4-6h after birth due to severe respiratory distress and the associated lethal cardiac anomalies, in spite of neonatal intensive care management.

The improved survival was the result of the advances in the management of the low-birth-weight neonates in NICU, including the management of respiratory distress syndrome, nutritional support and improved neonatal transport systems[17,18,19]  Pediatric anesthesia care has also improved in the last period with accurate management of oxygen for low-birth-weight patient, better capacity of monitoring, and improved equipment to prevent hypothermia[20,21]. Accumulated surgical experience, the improvements in suture materials, use of the finest instruments and the use of magnification tools have contributed to the improved outcomes of OA repair[22]. The thoracoscopic approach for OA repair might bring further improvement in the outcomes for these patients[23].

Conclusion

On the basis of this critical retrospective review of our surgical experience over the last 5 years, our results are comparable with the international figures. We believe that the advances in neonatal intensive care especially ventilator and nutritional support that facilitate proper pre-operative preparation and treatment of associated anomalies as well as the advances in pediatric anesthesia, and refinements in surgical techniques have contributed to improved outcomes of oesophageal atresia.

 

Acknowledgments

The authors thank all colleagues of pediatric surgical unit who assisted in this work and all the staff of neonatal intensive care unit, King Abdul Aziz Specialist Hospital Taif, Saudi Arabia, for their effort in pre-and postoperative management of neonatal cases of oesophageal atresia.

 

 

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