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Esophago-Pericardial Fistula in Childhood - Case Report and Literature Review

José L. Salinas Ruiz¹, Blanca J. R. Núñez², Miguel P. Piñeiro³
¹Departament of pediatric Surgery, Hospital Pediátrico Provincial Docente de Sancti-Spíritus “José Martíy Pérez”, Cuba
²Radiology Department, Sancti-Spiritus Provincial Teaching Pediatric Hospital, Cuba
³Cardiology Department, Sancti-Spiritus Provincial Teaching Pediatric Hospital, Cuba

 

Abstract

A case of esophago-pericardial fistula presenting as cardiac tamponade in a 2 years old girl with corrosive esophageal stenosis under esophageal dilatation treatment is reported. The patient survived the episode and six months later an esophageal substitution was successfully performed. A review of the literature reveals the rarity of this situation and several interesting aspects related to it are discussed.

Keywords: esophago-pericardial fistula, cardiac tamponade, child

 

Correspondence

José Luis Salinas Ruiz

Department Pediatric Surgery

Sancti-Spiritus Provincial Teaching Pediatric Hospital

Quinta del Oeste #52 Reparto Colon, Sancti-Spiritus, CP 60100, CUBA

E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Accidental caustic ingestion in childhood is a highly dramatic event for the potential severity of the damage and the fact of converting healthy children in chronic patients. In this paper we report a case of esophago-pericardial fistula, which demonstrate that severe and unusual complications are possible, demanding a wide knowledge spectrum for physicians treating these children. A world literature review reveals the rarity of this episode [1]. Our patient survived and is very well 18 months after definitive surgical treatment.

Case Report

A previously healthy two-years-old white girl accidentally swallowed sodium hydroxide at home in a rural community. The initial endoscopy showed a severe circumferential lesion (III degree) (transmural) of the whole esophagus. The treatment was in accordance with a protocol that forbids steroid administration and esophageal instrumentation in the first three weeks after the ingestion. TPN was started after the endoscopy. During the second week, normal swallowing wasn’t still reestablished and a temporal gastrostomy was performed.

Three weeks after the caustic ingestion an esophagogram showed a narrow passage all along the esophagus and weekly guided esophageal dilatation under general anesthesia was started. After the second session of dilatation, the patient had fever 39oC and general malaise during 15 days but without any other associated signs or symptoms. Blood, urine and cerebrospinal fluid cultures were all negative. The WBC showed leucocytosis (12 per 109 per liter) and neutrophilia (76%) and erythrocyte sedimentation rate was accelerated (40 millimeter per hour). After a week, symptoms relieved, WBC and erythrocyte sedimentation rate turned to normal and the patient was discharged.

Five days later she arrived to our hospital in emergency, badly ill with paleness, cyanosis, diaphoresis, cold limbs, weak peripheral pulses, neck veins turgescence and lethargy. Heartbeats were practically inaudible. A posteroanterior chest X ray in vertical position showed the presence of a hydro-pneumo-pericardium and cardiac tamponade was diagnosed. At the pediatric intensive care unit a sinus tachycardia of 240 per minute was diagnosed.

A pericardial puncture by the Marfan’s route with a Cavafix catheter (Gauge 17) allowed to evacuated 50 ml of slightly turbid yellow liquid and air. The patient improved immediately and a second radiography showed complete resolution of the hydro-pneumopericardium. Meglumine diatrizoate (Gastrografin) given per oral allowed esophageal and pericardial drawing, demonstrating the esophago-pericardial fistula (Fig. 1).

Figure 1: Contrast swallowed is depicting the burned esophagus and the pericardium.

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Broad-spectrum antimicrobial therapy was started and a terminal cervical esophagostomy performed. The pericardial catheter was attached to a collector bag and cyclically suctioned, and removed in the fifth day. Esophageal replacement with left colon was successfully performed 6 months later and the patient is in good health 18 months after last surgery.

Discussion

The first report of an esophago-pericardial fistula was published in 1838 [1] and in a recent review by Hamid et al. [2] less than 100 cases have been reported to date, most of them in adult patients. Children are clearly a minority in this group and the authors were able to find only 5 previous reported pediatric cases with fistula from the pericardium to the native esophagus [3, 4, 5, 6, 7] (Table 1).

Table 1. Abridgment of all pediatric cases

Author

Year

Age

Aetiology

Treatment

Survival

 

Peeler (3)

1957

11 months

Foreign body

Medical

 No

Dons (4)

1964

6 years

Esophagitis, esophageal ulcer

Spontaneous closure

Yes

(first survivor)

Priozhenko (cited by (5) )

1968

?

          ?

 

Spontaneous closure

 Yes

Pulatov (cited by (6) )

1992

?

Foreign body

   ?

   ?

Han (7) 

2008

1 year

Esophageal ulcer

Surgical            closure

 Yes

(youngest survivor)

At least three-fourths of cases are caused by benign diseases of the esophagus such as foreign bodies (pin, fish bone, etc.) [3, 4, 11], ulcer, reflux esophagitis, Boerhaave syndrome [12], tuberculosis, esophageal diverticulum, Barrett’s esophagus and achalasia. Esophageal interventions (esophagoscopy, esophageal dilatation, endoscopic sclerotherapy) can produce this eventual phenomenon too [13,14,15] and have been described even as a late complication, several months after these procedures [6]. Also malignant tumors have been found presenting with esophago-pericardial fistula in adults [13].

Only 3 iatrogenic and 2 caustic injuries are registered in a serie collected by Cirlak et al. in 1983 corresponding to the 6% and the 4% respectively of all non-malignant cases (76%) [16]. Again in 1991 a world literature review by Miller et al. just two reported cases of esophago-pericardial fistula caused by caustic ingestion were found [13]. In our patient both situations coexisted. Esophageal foreign bodies have been reported as an important etiology in childhood [3, 13, 14]. Recently, ablation of the atrial fibrillation focus has been reported as new iatrogenic cause of esophago-pericardial fistula [17, 18].

The anatomic relations between the esophagus and the pericardium at the level of T3 – T11 explain the occurrence of these fistulas. In some patients it presents as cardiac tamponade [1, 15] and others develop purulent pericarditis [3, 19, 20, 21]. Nevertheless atypical cases, simulating acute myocardial infarction [22], cardiac failure [13] or acute respiratory distress [23, 24] have been reported.

Several authors had described a noise similar to the splashing of a waterwheel that is pathognomonic of the hydro-pneumo-pericardium and can be heard over the precordium during the systole. Gurgling sensation in the chest has been adverted by a few patients [1, 13].

Mortality rate was very high in the past (83% as reported by Miller) and we think this is in accordance with diagnostic delay, development of purulent pericarditis, severe sepsis and previous general health deterioration. Significantly the first reported survivor was a 6 years old girl by Dons et al. in 1964 [4, 8, 9,10] and survival has been typically greater in children.

Fistula formation in our patient should consist of two stages: the first was perforation and adherence of the esophagus to the pericardium and secondly a real communication was established and cardiac tamponade took place. Hivet described a similar mechanism. This author believes that causal factor producing the fistula in his patient was the corrosive damage of the esophagus not concurrent esophageal instrumentation [25, 26]. In our patient both factors most have participated in the process.

It have been suggested that tension hydropneumo- pericardium take place when the fistula has a valve mechanism and is patent just intermittently [1, 23].

It is possible that in this case the gauge of the fistula would be very small and it could help in the spontaneous closure. In 1987 Naggar et al. compiled four patients with spontaneous closure of the fistula and two children who survived surgical closure [5].

Diagnostic difficulties and severity of the clinical problem in these kinds of patients are factors obligating to keep a great suspicion. The postero-anterior chest X-ray in vertical position, sonography and esophagogram with water-soluble contrast medium are the main support for diagnosis and should be accomplished immediately. So if cardiac tamponade is present, pericardial drainage should be the first step.

Shah et al. remarked the usefulness of esophagoscopy mainly guiding the nature and extension of the surgery [25] but this was no possible in our patient due to severe esophageal injury and stenosis.

In 2004 Solorzano et al. reported a case of esophago-pericardial fistula in a woman with her native esophagus partially excluded by closure at the cervical level and a retrosternal reversed gastric tube, but in that case the previous condition was different (hiatal hernia and gastroesophageal reflux) [27].

Treatment in our case consisted of pericardial drainage and cervical esophagostomy as a lifesaving procedure followed by reconstructive surgery because of the severe damage of the esophagus. Surgical closure of the fistula can be accomplished cautiously but mediastinitis, subsequent to the failure of esophageal suture, may be fatal therefor the use of a pleural or pericardial patch have been recommended [28, 29, 30].

In some cases, esophagectomy or terminal cervical esophagostomy plus temporal gastrostomy and esophageal substitution on a second step could be safer. The successful use of esophageal stents and combined methods to closure the fistula have been reported [17, 29, 31] and recently an over-the-scope clip was reported by Gubler and Bauerfeind in a very well-illustrated issue [32].

Several factors can influence the choice of the surgical technique: size of the fistula, coexistence or not of purulent pericarditis, primary disease, esophageal impairment and technological resources availability in the setting. General support and broad-spectrum antimicrobial therapy complete the tripod upon which patient’s life is kept, but early diagnosis and treatment is still the key in obtaining survival. Thus a greater clinical awareness is needed.

 

 

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