Concurrent Left Congenital Diaphragmatic Hernia and Esophageal Atresia: Case Report and Review of the Literature By Ahmed
H. Al-Salem,
Syed Qaisruddin, and Koyikal Qatif, SaudiArabia
0 Coexisting congenital diaphragmatic hernia and esophageal atresia is an extremely rare phenomenon. Details of one infant with such a combination is presented, and the literature on the subject is reviewed. Copyright o 1997 by W.B. Saunders Company INDEX WORDS: tracheoesophageal
Diaphragmatic fistula.
hernia,
esophageal
atresia,
E
SOPHAGEAL ATRESIA with or without tracheoesophageal fistula is a relatively common neonatal condition with an incidence of about 1 in 4,500 live births.’ The combination of esophageal atresia, with or without tracheoesophageal fistula, with congenital diaphragmatic hernia is exceedingly rare.’ We found only six cases reported in detail in the literature, and we describe an infant with pure esophageal atresia and concurrent left congenital diaphragmatic hemia.2-6 CASE
Varma
DISCUSSION
The incidence of anomalies associated with esophageal atresia is 48% and that of congenital diaphragmatic hernia is 21%.7.s The coexistence of both conditions in the same newborn, however, is extremely rare.’ The first detailed description of this association was reported by Ahmed in 1970.3 Table 1 gives the available data for the six reported cases described in detail in the literature along with those of our own. The presence of tracheoesophageal fistula makes the diagnosis of associated diaphragmatic hernia easier. The air escaping through the fistula will distend the stomach and intestine, which will appear to herniate into the chest. In four of the seven cases there was an associated tracheoesophageal fistula. In our case, as well as that reported by Takehara et al6 the diagnosis of associated
REPORT
A 1,350-g, 33-week-gestation boy was born to a para 2 healthy mother who had polyhydramnios. Apgar score was 5 and 6 at 1 minute and 6 minutes, respectively. The baby was noticed to have abundant frothy saliva after birth, and his abdomen was scaphoid. He was intubated immediately and ventilated. Pneumothorax developed on his right side and a thoracostomy tube was inserted (Fig 1). The nasogastric tube did not pass beyond the C5 level, and the abdomen was gasless on x-ray, confirming the diagnosis of esophageal atresia. The nasogastric tube was put on continuous low suction. The left hemithorax was opaque in its lower part with a small amount of aeration at the apex. The mediastinum was central. A concomitant left congenital diaphragmatic hernia was suspected. Three days later, after the infant was stabilized on ventilator, the abdomen was explored. There was a large left diaphragmatic hernia with small bowel, large bowel, stomach, and left lobe of liver in the left hemithorax. The contents were reduced and the diaphragmatic defect temporarily repaired by suturing the peritoneal flaps raised from the edges of the defect. Gastrostomy and drainage of the left thoracic cavity was performed. Contrast study through the gastrostomy failed to show any length of the lower esophagus (Fig 2). Ten days later he underwent re-operation because of recurrence of his left diaphragmatic hernia. This was closed with a Gore-Tex patch. Postoperatively, the baby improved gradually, but he continued to be ventilated, and 17 days following his second operation methicillinresistant staphylococcus aureus (MRSA) septicemia developed, and he died.
From the Division of Pediatric Surgery, Department of Surgery, Qatif Central Hospital, Saudi Arabia. Address reprint requests to Ahmed H. Al-Salem, FRCSI, FIGS, FACS, PO Box 18432, Qatif 31911, Saudi Arabia. Copyright o 1997 by WB. Saunders Company 0022-3468/97/3205-0032$03.00/O 772
Karthikeya
’
‘,‘,
“, :
Fig 1. A radiograph showing men and opaque left hemithorax.
Journal
ofPediatrIc
Surgery,
right
Vol32,
pneumothorax
No 5 (May),
gasless
abdo-
1997: pp 772-774
CONCURRENT
LEFT CDH AND
ESOPHAGEAL
ATRESIA
773
Fig 2. Postoperative chest x-ray with contrast study demonstrating the nasogastric tube in the upper pouch. No reflex of contrast from the gastrostomy into the lower esophageal pouch was seen.
congenital diaphragmatic hernia was delayed. In Takehara’@ case there was a perforation of the lower esophagus, which they attributed to prolonged kinking of the esophagocardiac junction after herniation of the stomach Table 1. Summary Case NO.
Year
Study
of the Clinical Site of
Type
CDH
of EA
1
1970
SaeedAhmed3
R
C
2
1983
Bowen
3
1984
Rawlings
L R
c C
et aI4 et aI5
and Operative
Findings
of Seven
Operative
FindIngs and Procedures
Via a (Rt) thoracoabdominal Meckel’s diverticulum
1987
Udassrn
et al*
L
C
1987
Udasscn
6
1993
Takehara
7
1995
Present
et aI2 et aI6 case
L
B
L
B
L
B
Abbreviatrons: CDH, congenital diaphragmatrc with distal TEF; Type (6) EA. esophageal atresia
approach. gastrostomy
With
EA With
or Without
and divisron
Malrotation of bowel + ligation and divisron
+
Died 20 days
hernra; EA, esophageal without fistula.
atresra;
postoperatively
surgery postoperatively
of TEF. No esophageal
of Meckel’s diverticulum and both ends as rleostomres and gastrostomy.
and esophagogastrostomy. + gastrostomy and closure patch
Died 30 hours
Died before of
of ileum
of large CDH with
TEF, tracheoesophageal
Died shortly
after surgery
ex-
Gastrostomy + closure of large CDH with SILASTIC@ sheet through the abdomen. Reparr of (Lt) CDH and later partial resection of the lower esophagus Laprotomy Gore-Tex
TEF and CDH Outcome
Via an abdominal incision. Reduction of CDH contents, legation and division of TEF. Repair of CDH usrng a SILASTIC@ sheet. Resectron tenor&d
5
Patients
of TEF. Direct reparr of CDH. No esophageal anastomosis. Trisomy 18 + multiple anomalres. No operatrons. Via a transverse abdominal incisron. Gastrostomy + repair CDH. (Rt) thoracotomy anastomosrs.
4
into the left thoracic cavity. In cases of pure esophageal atresia associated with nonaeration of the lungs, the possibility of associated congenital diaphragmatic hernia must be considered. With the recent advances in prenatal ultrasonography, the associated diaphragmatic hernia can be diagnosed prenatally, which makes the diagnosis of the combination easier. as in the second case of Udassin et al.’ The operative approach to this combination should be individualized based on the site of the diaphragmatic defect as well as the presence or absence of tracheoesophageal fistula. For those with right-sided diaphragmatic hernia, both defects can be dealt with via a right thoracotomy. Those with left diaphragmatic hernia and tracheoesophageal fistula will require a right thoracotomy with ligation and division of the fistula to prevent gastrointestinal distension from air escaping via the fistula, which may further compromise their ventilation, and a laparotomy to repair the diaphragmatic defect. For temporary decompression of the gastrointestinal tract in these patients, Rawlings et al5 advocated passing a small tube through the endotracheal tube, which is advanced via the fistula into the stomach. Udassin et al2 successfully ligated a tracheoesophageal fistula in one of their patients via the transabdominal approach. In those with congenital diaphragmatic hernia and esophageal atresia without fistula, the diaphragmatic hernia is repaired transabdominally, and a gastrostomy is fashioned. The esophageal atresia is dealt with later. The diaphragmatic defect in these infants tends to be very large, necessitating patching with synthetic material. All infants with this combination described so far have died, indicating associated severe pulmonary hypoplasia, although sepsis was a contributing factor in some of them.
Died 24 hours
postoperatively
Died at 161 days a
Died 30 days
fistula;
of age
postoperatrvely
Type (C) EA, esophageal
atresia
774
AL-SALEM,
QAISRUDDIN,
AND
VARMA
REFERENCES 1. Myers NA: Oesophageal atresia: The epitome of modern surgery. Ann R Co11 Surg Engl54:277-287, 1974 2. Udassm R, Zamir 0. Peleg 0. et al: Co-existmg left diaphragmatic hernia and esophageal atresia. Pediatr Surg Int 2:301-303, 1987 3. Ahmed S: Right-sided bochdalek hernia associated with esophageal atresia and trachea-esophageal fistula. J Pediatr Surg 5:256. 1970 4. Bowen A: The ventilatory dilemma of co-existing diaphragmatic hernia, esophageal atresia and tracheoesophageal fistula. Crit Care Med 11:390-391, 1983 5. Rawlings JS, Shetler PL, Fill WL, et al: Concurrent right dia-
phragmatic hernia and type C tracheoesophageal fistula. Clin Pediatr 235 18-520, 1984 6. Takehara H, Komi N, Okada A, et al: Left diaphragmatic hernia associated with lower esophageal atresia. 3 Pediatr Int 8:339-340, 1993 7. Holder TM, Cloud DT, Lewis JE. et al: Esophageal atresia and tracheoesophageal fistula: A survey of its members by the surgical section of the american academy of pediatrics. Pediatrics 34:542-549, 1964 8. Butler N, Claireoux AE: Congenital diaphragmatic hernia as a cause of permatal mortality. Lancet 1:659-663. 1962