Find best premium and Free Joomla templates at GetJoomlaTemplatesFree.com

Is Esophagostomy Necessary in Isolated Esophageal Atresia ? Report of Six Cases.

Aysenur Cerrah Celayir, Osman Zeki Pektas, Alp Gence, Cengiz Gül, Ceyhan Sahin
Department of Pediatric Surgery, Zeynep Kamil Maternal and Child Diseases Educational and Research Hospital, Istanbul, Turkiye

 

Correspondence 

Aysenur Cerrah Celayir, Associate Professor, Chief of The Pediatric Surgery and Director of the Hospital,
Zeynep Kamil Maternal and Child Diseases Educational and Research Hospital,
Zeynep Kamil Kadin ve Cocuk Hastaliklari Egitim ve Arastirma Hastanesi, Bashekim,
Arakiyeci Haci Mehmet Mah. Op.Dr.Burhanettin Ustunel Cad. No:10, 34668,Uskudar, Istanbul, Turkiye
Tel: + 90 216 343 20 73; Fax: + 90 216 343 92 51;
E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it. ; This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Background: Treatment of long gap esophageal atresia (EA) is still a major challenge. Treatment modalities for delayed repair with or without esophagostomy in isolated esophageal atresia still remains controversial. There is general agreement that the child's own esophagus is the best. The aim of this study is to discuss outcomes of delayed primary esophago-esophageal anastomosis after gastrostomy without esophagostomy in cases with isoleted esophageal atresia.

Material and Method: Results of the treatment of isolated esophageal atresia were analyzed over a 4 years period retrospectively. Gender, age at the time of the gastrostomy, associated anomaly, age of the primary repair of the esophagus, outcomes were analyzed from hospital records.

Results: Six neonates with isolated esophageal atresia were analyzed during that period. All cases were classified as long gap (equal or greater than 3 cm). All infants underwent gastrostomy without esophagostomy. Follow-up ranged from 3 months to 44 months. In all cases, esophago-esophageal anastomosis was performed. No ventilatory support was necessary in any patient after the delayed primary esophagus repair. Gastro-esophageal reflux disease occurred in 3 cases, of which, 1 required Thall funduplication. None of the patients had long term esophageal swallowing difficulties nor persistent dysphagia. Two children experienced food aversion. Mean hospital stay was 3 (1 to 6) months. There were 3 deaths: One was due to associated anomalies and two were secondary to late sepsis.

Conclusions: Delayed primary repair of the isolated esophageal atresia without esophagostomy provides successful repairing if upper eophageal pouch continuously and effectively aspirated. Gastroesophageal reflux represent the most frequent postoperative problem, but additional procedures required seem "acceptable" to maintain the patient's own esophagus and avoid replacement. Esophageal substitution in long gap esophageal atresia should be reserved only for cases in which a previous attempt of esophageal repairing failed.

Key words: esophageal atresia, delayed primary anastomosis, long gap, esophageal replacement

 

Introduction 

Treatment of long gap esophageal atresia (LGEA) or isolated esophageal atresia represents a surgical challenge. Despite the significant improvement of esophageal atresia patient outcome in general, LGEA patients still have a significant mortality and morbidity. In general, the consensus among pediatric surgeons is that a delayed repair seems appropriate using the native esophagus before considering the use of any esophageal replacement procedure. In many cases the substitute is clearly inferior to the native esophagus.

During the past four decades, several methods have been employed to overcome the technical difficulties encountered in bringing the two esophageal segments together [1,2]. Treatment modalities including delayed repair of with or without esophagostomy in isolated esophageal atresia still remains controversially. It has been shown that, if left undisturbed, the proximal pouch in pure esophageal atresia is capable of spontaneous growth, and that this is related to the normal swallowing reflex [2].

The purpose of this study was to review our long-term outcome in infants with isolated esophageal atresia (EA) treated by delayed primary anastomosis and to discuss outcomes of delayed primary esophago-esophageal anastomosis after gastrostomy without esophagostomy in cases with isolated esophageal atresia.

Material and methods 

A retrospective review was undertaken of the medical records of all patients managed for isolated esophageal atresia during the 4 year period between January 2004 and January 2008. The total number of esophageal atresia patients which were operated on during this period were 46; there were 6 isolated esophageal atre- sia.

Data examined in cases with isolated esophageal atresia in- cluded: sex, maternal history of polyhydramnios, associated anomalies, gestational age, birth weight, Apgar scores, age when gastrostomy was performed, need for esophagostomies, gap length, and age at the time of the delayed primary anastomosis. In addition, it was noted whether circular myotomy to assist upper pouch elongation was performed. The duration of postoperative ventilatory support, immediate complications and management, late complications and management, how long gastrostomy feeds were needed after esophageal anastomosis, the length of the hospital stay and mortality were also noted.

Results

Fourtysix patients with esophageal atresia were treated between January 2004 and January 2008. Six neonates with isolated esophageal atresia were considered during the study period. The percentage of type A (no TEF) in the whole patients was 13%. There was positive maternal history of polyhydramnios in all six cases. Prenatal abnormalities were determined in four fetuses on antenatal ultrasounds. In three fetuses, gastric pouch were not seen in US; one of them had got hydrocephaly. In addition, megacystis were determined in one patients, referred us due to this abnormality . All patients were born by cesarian section, three of them were referred to our department from another hospitals. Median Apgar scores were six at first minute and nine at fifth minute. Maternal ages varied from 20 years old to 30 years old, mean age of the mothers was 26 years old. Three mothers had an abortus, three mothers had a baby death. Mean gestational age of 6 cases with isolated esophageal atresia was 35 weeks (ranges from 28 weeks to 38 weeks). Maternal and prenatal features of cases were summarised in Table 1.

Table I: Maternal and prenatal features of cases 

No

Sex

Prenatal US

Maternal age

Gravida (G)

Abortus (A) Exitus (E)

Gestational age

1

F

Megacystis

20 y

2G

1A

35 w

2

M

No gastric pouch

30 y

1G

36 w

3

M

No gastric pouch

28 y

5G

1A,3E

36 w

4

F

-

23 y

3G

1A,1E

28 w

5

F

Hydrocephaly

No gastric pouch

26 y

4G

1E,1N,1hipophisis

Deficiency

37 w

6

M

-

30 y

3G

38 w

 

Mean birth weight of 6 cases with isolated esophageal atresia was 1925 gram ( ranges from 1040 gram to 2900 gram). In all cases with isolated esophageal atresia, associated anomalies were determined after birth. One had cardiac murmur, megacystitis and suspected urogenital sinus; second baby had microcephaly without cardiac murmur, two babies had DOWN syndrome signs and cardiac anomalies, one had hidrocephaly and hemivertebra and cardiac anomaly; one patient had got TAR syndrome and cardiac anomalies (TAR Syndrome: Thrombocytopenia with Absent Radius is a rare genetic disorder which is characterized by the absence of the radius bone in the forearm). Cardiac anomalies were very severe in three babies, central cyanosis progressed in first days.

A gastrostomy without esophagostomy was performed on day 1 to 8 in all patients, when they arrived in our department. All cases were estimated as long gap initially because of the isolated esophageal atresia and level of the uper pouch on X-Ray. It was not easy to find the actual gap length before the operation. We performed rigid and flexible esophagoscopy. Forced opaque graphy from the gastrostomy was not successful to show distal esophagus length in generally ( fig. 1). The distance was assessed succesfully in three cases only by radiological testing between two opaque bars (Nasogastric tube and Hegar’s or uretral dilator) un- der x-ray screen before the time of the thoracotomy. The lenghts of the gap were seen and assessed at thoracotomy in all patients. Characteristics of the patients were summarised in Table 2.

3.3 2009 -6 -1

Fig. 1. Distal esophagus wasn’t assessed with opaque graphy from the gastrostomy before operation of the primary repair in second case

Table 2: characteristics of cases with isolated esophageal atresia 

No

Sex

Birth weight

Associated anomaly

Waterstone classification

Upper pouch level

Day of gastrostomy

1

F

1690g

Cardiac anomaly, urogenital sinus megacystis

C2

3rd intercostal

3rd

2

M

1780g

Microcepgaly

C1

4th intercostal

2nd

3

M

1990g

TAR, cardiac anomaly

C2

2nd intercostal

5th

4

F

1040g

Down, cardiac anomaly

C2

4th intercostal

8th

5

F

2150g

Hydrocephaly. Hemivertebra, cardiac anomaly

B2

2nd intercostal

3rd

6

M

2900g

Down, cardiac anomaly

A2

3rd intercostal

2nd

 

Delayed primary esophageal anastomosis was performed successfully in three patients (50%). Single layer end-to-end anastomosis was performed in three patients witout any circular myotomy. No ventilatory support was necessary after the delayed primary esophagus repair in any patient. There were no anastomotic leakage nor strictures encountered in these three patients. Mean age at delayed primary anastomosis in cases with isolated esophageal atresia was 4,3 month old (range 2 month old to 7 month old) and the mean hospital stay was 3 months (range 1 months to 6 months). The patients were followed for a period of 1.5 years to 5 years.

Contrast studies were done after the primary repair. Esophagus was seen without leakage in all patients ( fig. 2 A,B,C). Gastrostomy feeds were stopped within one month of final repair in all patients. Gastro-esophageal reflux occurred in all 3 cas- es, of which one required Thall funduplication after three months because of severe reactive airway and recurrent bronchial asthma crisis ( fig 2). None of the patients had difficulty swallowing nor persistent dysphagia. Two children experienced food aversion for approximately three months. Three babies died (50%) prior to primary esophageal anastomosis. These patients had also severe cardiac anomalies. Two deaths were caused by severe associated anomalies and one was caused by late sepsis. A lateral cervical esophagostomy was fashioned in one patient with TAR syndrome, when he was one month old before discharging hom. He died one day after discharge. Autopsy was not performed to confirm the cause of death in these three patients because of lack of parental consent.

3.3 2009 -6 -2a 3.3 2009 -6 -2b 3.3 2009 -6 -2c
Fig. 2A. Esophagography after primary repair of the esophagus in first case Fig. 2B. Esophagography after primary repair of the esophagus in second case Fig. 2C. Esophagography after primary repair of the esophagus in fourth case

Discussion

Esophageal atresia with or without tracheoesophageal fistula (TEF) is a rare congenital anomaly, and a number of anatomical variants are described that relate to the length of the gap between the upper and lower esophagus and to the type of fistula which may be present between the esophagus and trachea [2]. When there is a TEF, the ideal surgical management consists of divi- sion of the fistula and primary end-to-end anastomosis between the upper and lower esophageal segments. The management of a long gap esophageal atresia (LGEA) with or without a TEF remains a major challenge among pediatric surgeons. Despite advances in the management and significant improvement in the outcome of esophageal atresia patients for the last few decades [3,4,5], the management of LGEA or isolated EA remains controversial. Most surgeons agree that the presence of a LGEA may preclude immediate primary repair, but the diagnostic criteria for LGEA differ among different surgeons. There are no uniformly accepted criteria that define LGEA. Some authors divided the anomaly into short and long gap with 2 cm as the cutoff point [6], whereas others classified it into short, intermediate, and long with 1 cm and 2.5 to 3cm as the cutoff points [7] and [8]. Although these classifications have been proposed, their clinical significance has not been substantiated because of a lack of uniformity of methods used to measure gap length. Most reports did not indicate whether the gap was measured before or after dis- section and if the gap was measured under tension.

The inability to perform a primary anastomosis between the two esophageal ends because of the long gap as an indicator of the diagnosis was considered [8]. Retention of the esophagus was felt to be ideal by most of the surgeons, and different elongation procedures were developed to achieve this. The delayed primary anastomosis had the best long-term outcome compared to all other previously reported techniques [9]. Though esophageal continuity is achieved by all the various techniques, mobilizing the stomach into the thorax results in displacement of the gastroesophageal junction into the chest, leading to a persistent gastroesophageal reflux and strictures in virtually all cases [9,10,11]. Other longterm complications also are described with these techniques [12,13]. The best management in children with LGEA is still undetermined, but children whose esophagus is preserved seem to have a better long-term outcome with respect to swallowing and gastroesophageal reflux related symptoms [10]. At present, most pediatric surgeons agree that delayed esophageal anastomosis with an end-to-end anastomosis is the procedure of choice in the management of isolated esophageal atresia as it offers the most physiological reconstruction possible [11,12,13,14].

Table 3: Operative findings in primary repair, morbidity and outcomes 

No

Sex

Classification

Birth weight

Assoc. anomaly

Gastrostomy

Primary repair

Gap length

Total hospitalization

Outcome

1

F

C2

1690g

Cardiac anomaly, urogenital sinus, megacystis

3rd day

7th month

4 cm Esop. Dup. Cyst 1x2x3

62 days

Good

2

M

C1

1780g

microcephaly

2nd day

4th month

4 cm

102 days

Good

3

M

C2

1990g

VATER, TARR, cardiac anomaly

5th day

-

No thoracotomy

42 days

Exitus

2 months

4

F

C2

1040g

Down, cardiac anomaly

8th day

2nd month

3 cm

63 days

Good

5

F

B2

2150g

Hydrocephaly, hemivertebra, cardiac anomaly

3rd day

-

No thoracotomy

11 days

Exitus

(menengitis sepsis) 1 month

6

M

A2

2900g

Down, cardiac anomaly

2nd day

2nd month thoracotomy, traction sutures

4 cm

82 days

Exitus

3 months

(sepsis)

 

Because of the high incidence of complications and long-term side effects associated with esophageal replacement procedures [15,16,17], much research has been focused on developing surgical strategies to preserve the infant’s native esophagus. It has been shown that, if left undisturbed, the proximal pouch in type A esophageal atresia is capable of spontaneous growth, and that this is related to the normal swallowing reflex [1]. However, this conservative management may result in prolonged hospital stay and increased risk of complications due to aspiration of saliva from the upper pouch [13]. Proximal and distal myotomies [19,20], mechanical bougienage either manual or electromagnetic [21,22,23,24,25] distal pouch mobilisation [26], multistage ex- trathoracic esophageal elongation [27], upper pouch flap [28], or combination of them [29] was used for that purpose of elongation of the upper pouch. Even when the primary anastomosis was not achieved in the first attempt, traction sutures can be used to produce significant esophageal elongation within days [8]. Although these methods of esophageal elongation remain in current practice, various complications have been reported. Stricture, leak, recurrent TEF, and esophageal mucosal outpouching have been reported [30,31]. Daily “stretching” tecniques (i.e. bougenage) of the proximal and distal pouches (using the gastrostomy opening and a 4-5 mm metal dilator) is well tolerated in most infants and can significantly reduce the length of time required before primary anastomosis can be accomplished [21,22,23,24,25].

Esophagostomy may be undertaken when a significant delay is anticipated before repair. The advantages are that it helps simplify patient care for the family, it may significantly reduce hospitalization time while waiting for the repair and it allows the introduction of sham feeding. However, apart from esophagostomy used in the multistaged extrathoracic esophageal elongation [26], it seems that esophagostomy has fallen out of favor over the years. 

Moreover, none of the survey responders will consider esophagostomy for the management of LGEA. This might be because of fears that it could jeopardize the use of the native esophagus while waiting for the delayed repair. Use of end-esophagostomy perhaps should be limited to complicated cases to avoid recurrent aspiration and sepsis and only after all attempts of preserving the native esophagus have failed. In these cases, lateral esophagostomy may be helpful in the initial management of long gap esophageal atresia without fistula. This technique represents a safe way of managing newborns with long gap esophageal atresia without fistula, allowing them to be discharged home on G-tube feedings while waiting for spontaneous growth of the proximal pouch to occur. This may increase the possibility of accomplishing a definitive correction with a delayed primary anastomosis using the infant’s own esophagus [32]. The importance of sham feeding using a diverting esophagostomy has been advocated for the avoidance of food aversion in these patients. Proponents feel that it may prevent a delay in the development of normal swallowing. Sham feeding, has been shown to have a significant positive impact on the progress of these patients after repair [33]. The feeding difficulties can persist for many months during which gastrostomy feding is required. In the long term, the majority of patients can eat and swallow normally [33].

In conclusion, delayed primary repair of the isolated long gap esophageal atresia without end or lateral esophagostomy can be successful if upper proximal esophageal pouch is aspirated continuously and effectively. Gastroesophageal reflux represent the most frequent postoperative problem, but additional procedures required seem “acceptable” to maintain the patient’s own esopha- gus and avoid replacement. Esophageal substitution in long gap esophageal atresia should be reserved only for cases in which a previous attempt of esophageal repair has failed. This report has some limitations due to the retrospective nature and of small number of cases which could impact the validity of the datas. Our long-term follow-up suggests that the delayed primary anastomosis without esophagostomy provides excellent functional results in patients born with isolated esophageal atresia or LGEA.

 

References 

  1. Puri P, Blake N, O’Donnell B, Guiney EJ. Delayed primary anastomosis following spontaneous growth of esophageal segments in esophageal atresia. J Pediatr Surg 1981; 16: 180–3
  2. Sir Paran T, Decaluwe D, Corbally M, Puri P. Long-term results of delayed primary anastomosis for pure oesophageal atresia: a 27-year follow up. Pediatr Surg Int 2007; 23: 647-51
  3. Spitz L. Gastric transposition for esophageal substitution in children. J Pediatr Surg 1992; 27: 252–7
  4. Louhimo I, Lindahl H. Esophageal atresia: primary results of 500 consecutively treated patients. J Pediatr Surg 1983; 18: 217–29
  5. Ein SH, Shandling B. Pure esophageal atresia: a 50-year review. J Pediatr Surg 1994; 29: 1208–11
  6. Hands LJ, Dudley NE. A comparison between gap length and Waterson classifications as guides to mortality and morbidity after surgery for esophageal atresia. J Pediatr Surg 1986; 21: 404–6
  7. Brown AK and Tam PKH. Measurement of gap length in esophageal atresia: a simple predictor of outcome. J Am Coll Surg 1996; 182: 41–5
  8. Foker JE, Linden BC, Boyle EM et al. Development of a true primary repair for the full spectrum of esophageal atresia. Ann Surg 1997; 226: 533–43
  9. Spitz L. Esophageal atresia: Past, present, and future. J Pediatr Surg 1996; 31: 19–25
  10. Rescorla FR, West KW, Scherer III LR et al. The complex nature of type A (long-gap) esophageal atresia. Surgery 1994; 116: 658–64
  11. Lindahl H, Louhimo I, Virkola K. Colon interposition or gastric tube? Follow up study of colon esophagus and gastric tube-esophagus patients. J Pediatr Surg 1983; 18: 58–63
  12. Erdogan E, Emir H, Eroglu E, Danismend N, Yeker D. Esophageal replacement using the colon: a 15-year review. Pediatr Surg Int 2000; 16: 546–9
  13. Davenport M, Hosie GP, Tasker RC et al. Long-term effects of gastric transposition in children: a physiological study. J Pediatr Surg 1996; 31: 588–93
  14. Gupta DK, Kataria R, Bajpai M. Gastric transposition for esophageal replacement in children: An Indian experience. Eur J Pediatr Surg 1997; 7: 143–6
  15. Ahmad SA, Sylvester KG, Hebra A, et al (1996) Esophageal replacement using the colon: Is it a good choice? J Pediatr Surg 1996; 31:1026-30
  16. Stone MM, Fonkalsrud EW, Mahour GH, et al. Esophageal replacement with colon interposition in children. Ann Surg 1986; 203: 346-51
  17. Valente A, Brereton RJ, Mackersie A. Esophageal replacement with whole stomach in infants and children. J Pediatr Surg 1987; 22: 913-7
  18. Healey PJ, Sawin RS, Hall DG, et al. Delayed primary repair of esophageal atresia with tracheoesophageal fistula: is it worth the wait? Arch Surg 1987; 133:552-6
  19. Lai JY, Sheu JC, Chang PY et al. Experience with distal circular myotomy for long-gap esophageal atresia. J Pediatr Surg 1996; 31: 1503–8
  20. Ricketts RR, Luck SR, Raffensperger JG. Circular esophagomyotomy for primary repair of long-gap esophageal atresia. J Pediatr Surg 1981; 16: 365–9
  21. Hendren WH, Hale JR: Esophageal atresia treated by electromagnetic bougienage and subsequent repair. J Pediatr Surg 1976; 11:713-22
  22. Woolley MM (1980) Esophageal atresia and tracheoesophageal fistula. Am J Surg 1980; 139: 771–4
  23. Hikida S, Tanaka Y, Tsuru T et al. The elongation of distal esophageal pouch by mechanical bougienage for a year resulted in a tension-free anastomosis in a patient with long gap esophageal atresia: A case report. Kurume Med J 2003; 50: 139–42
  24. Puri P, Ninan GK, Blake NS et al. Delayed primary anastomosis for esophageal atresia: 18 months’ to 11 years’ follow up. J Pediatr Surg 1992; 27: 1127–1130
  25. Quan L, Smith DW. The VATER association. Vertebral defects, Anal atresia, T-E fistula with esophageal atresia, Radial and Renal dysplasia: a spectrum of associated defects. J Pediatr 1973; 82:104-7
  26. Lessin MS, Wesselhoeft CW, Luks FI et al. Primary repair of long-gap esophageal atresia by mobilization of the distal esophagus. Eur J Pediatr Surg 1999; 9: 369–72
  27. Kimura K, Nishijima E, Tsugawa C et al. Multistaged extrathoracic esophageal elongation procedure for long gap esophageal atresia: Experience with 12 patients. J Pediatr Surg 2001; 36: 1725-7
  28. Brown AK, Gough MH, Nicholls G et al. Anterior flap repair of esophageal atresia: a 16-year evaluation. Pediatr Surg Int 1995; 10: 525–8
  29. Lopes MF, Reis A, Coutinho S et al. Very long gap esophageal atresia successfully treated by esophageal lengthening using external traction sutures. J Pediatr Surg 2004; 39: 1286–7
  30. Janik JS, Filler RM and Ein SH et al. Long-term follow up of circular myotomy for esophageal atresia. J Pediatr Surg 1980; 15: 835–41
  31. Otte JB, Gianello P, Wese FX et al. Diverticulum formation after circular myotomy for esophageal atresia. J Pediatr Surg 1984; 19: 68–71
  32. Aloisi AS, de Freitas S Jr, Colombo AC, Amalfi R, Sbragia-Neto L, Bustorff-Silva JM (2000) Lateral Esophagostomy: An Alternative in the Initial Management of Long Gap Esophageal Atresia Without Fistula. J Pediatr Surg 35:1827-9
  33. Masked DJ, Blane CE, Drongowski RA et al (1997) Complications after gastric transposition in children. Can Assoc Radiol J 48: 259–64
Joomla templates by a4joomla
More Free Joomla Templates at GetJoomlaTemplatesFree.com