Congenital diaphragmatic hernia in identical twins.

Congenital diaphragmatic hernia (CDH, Bochdalek type) is rarely seen in both members of identical twins. Herein, we report a 37 weeks' twins with CDH along with a brief review of the literature. Both the neonates survived.

INTRODUCTION

Congenital diaphragmatic hernia (CDH) is a life-threatening anomaly, with an incidence of one in 2500 newborns.[1] Although it is accepted as a developmental anomaly, genetic factors cannot be excluded due to the probability of existence in siblings.[2] To our knowledge, only five instances of surviving twins with CDH could be found in the English literature.[2-5] We herein report an occurrence of CDH in twins.

CASE REPORT

Two girls were delivered by caesarean section at 37 weeks of gestation from the third pregnancy of a 29-year-old woman. Baby A and B, whose placenta was defined as singleton weighed, 2850 g and 2000 g. Both babies received mechanical ventilation due to respiratory distress. The chest radiographs of both revealed left diaphragmatic hernia (Bochdalek type) [Figure 1]. Baby A was operated on the 6th and baby B was operated on the 7th day after delivery. Primary diaphragmatic repair was performed. Small intestine, spleen and a big proportion of the colon were located in the thorax in both the babies, and the stomach was in the abdomen. The postoperative recovery was uneventful. Both children were thriving at 15 months follow-up [Figure 2].

Figure 1

Preoperative plain chest radiographs of the babies

Figure 2

Babies are 15-months old

DISCUSSION

CDH is a very rare anomaly in twins. Till date, only five pairs of surviving twins with CDH have been reported, except stillborns. One of them was reported by Locatelli in Italian language.[5] The details of Locatelli's case could not be obtained. The data of the other four cases are outlined in Table 1. Prenatal diagnosis was reported to be made in cases of Tazuke et al.[4] The diagnosis could only be made postnatally in other cases, including ours. CDH was described in another sibling only, in one patient of the twins.[2] This was a case of dizygotic twins. Four cases were monozygotic twins. In our case, the intestines (but not stomach) and spleen had herniated into the thorax. Similarly, small intestine and colon have been reported to be herniated into the thorax in other pairs. None of them had stomach herniation.[2-5] The spleen was also located in the thorax in one pair.[2]

Table 1

General features of twins with congenital diaphragmatic hernia

Three pairs were girls, one pair was boys and one pair of twins consisted of a girl and a boy (the dizygotic twin). While the diagnosis could be made in the first hours of postnatal period in three of four pairs diagnosed postnatally, in the 5th pair, diagnosis was made on the 5th day in one baby and in another it was diagnosed in the 6th month. In the latter case, not taking a chest radiograph despite asphyxia and respiratory difficulty has drawn attention as an interesting detail.[5] As a major complication, ileostomy was performed because of intestinal perforation and peritonitis and closed thereafter in one of the twins in one case.[4] In another pair, inguinal hernia repair was performed.[3]

In conclusion, the likelihood of the presence of CDH in both twin pairs is quite low. However, when the case that was diagnosed late is taken into consideration, a meticulous examination is recommended if CDH is detected in one of the twin pairs.