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CHAPTER 48

Oesophageal Atresia

Peter Beale
Kokila Lakhoo

Introduction The mechanism of development of tracheo-oesophageal fistula is
Little from Africa has been reported or written on the subject of oesoph- the failure of apposition of longitudinal ridges, whereas the mechanism
ageal atresia (OA). The frequency of diagnosis and especially survival of the development of oesophageal atresia is apposition too posteriorly.
is widely variable, depending on available resources and expertise. In OA/TOF is classified into six types (see Figure 48.1):
some areas, the incidence of diagnosis and survival is unlikely, whereas A. Isolated oesophageal atresia (8%)
in a developing country such as South Africa, where facilities are good B. Upper pouch fistula with oesophageal atresia (1%)
and a limited number of dedicated paediatric surgeons accumulate
a large amount of experience, results are comparable to those of the C. Oesophageal atresia with tracheo-oesophageal fistula (86%)
developed world. D. Upper and lower pouch fistula (0.5%)
Demographics E. H-type fistula (4%)
The incidence of oesophageal atresia in Africa is unknown but would F. Oesophagus with tracheal segment (0.5%)
appear to be no different from that in other populations. Amongst South Clinical Presentation
Africa’s multicultural population, the incidence in the white population Prenatally, the condition may be suspected from maternal polyhydram-
seems to be higher; however, this was very likely a spurious impression nios and absence of a fetal stomach bubble at the 20-week anomaly
due to missed diagnoses. scan. Prenatal scan diagnosis of OA/TOF is estimated to be less than
In 2008, Nandi and colleagues reported equivalent incidences of 42% sensitive with a positive predicted value of 56%. Additional
oesophageal atresia/tracheo-oesophageal fistula (OA/TOF; 2.1% of diagnostic clues are provided by associated anomalies, such as tri-
all neonatal admissions) at two linked surgical departments in Europe somy (13, 18, 21); VACTERAL (vertebral, anorectal, cardiac, tracheo-
and Africa: John Radcliffe, Oxford, United Kingdom; and Kilimanjaro oesophageal, renal, limbs) sequence; and CHARGE (coloboma, heart
Christian Medical Centre, Tanzania. Reports from Nigeria and Zimbabwe defects, atresia choanae, retarded development, genital hypoplasia,
show an incidence comparable to that at Great Ormond Street Children’s ear abnormality) association. These associated anomalies are present
Hospital in London. The incidence in Africa probably corresponds to the in more than 50% of cases and worsen the prognosis; thus, prenatal
1 per 3,000–4,500 reported across the world in the literature. karyotyping is essential. Duodenal atresia may coexist with OA/TOF.
Aetiology/Pathophysiology The risk of recurrence in subsequent pregnancies for isolated OA/TOF
The mechanisms of embryological development of OA/TOF occurs in is less than 1%. Delivery is advised to be at a specialised centre with
the embryo at 3 weeks postfertilisation during the demarcation of the neonatal surgical input.
proximal foregut as the oesophagus with a gastric bubble caudally and In the absence of prenatal diagnosis, the presentation may be respiratory
a ventral lung bud cranially. During the subsequent phase of elonga- distress, cyanotic spells, frothing around the mouth, and arrested passage
tion of the oesophagus and lung bud, there is a further division of the of a nasogastric tube. Recurrent pneumonia or failure to feed are noted in
tracheal primordium from the oesophagus. delayed presentations. Presentation with gastric rupture, especially in low
birth weight babies, is known to increase morbidity and mortality.

Figure 48.1: Types of oesophageal atresia and tracheo-oesophageal fistula.