Journal of Indian Association of Pediatric Surgeons
Journal of Indian Association of Pediatric Surgeons
                                                   Official journal of the Indian Association of Pediatric Surgeons                           
Year : 2012  |  Volume : 17  |  Issue : 1  |  Page : 26--27

Congenital diaphragmatic hernia in identical twins


Mustafa T Gurbaz, Ahmet Kazez, Unal Bakal, Tugay Tartar, Fikret Ersoz, Yunus Colakoglu 
 Department of Pediatric Surgery, Fırat University, Elazig, Turkey

Correspondence Address:
Ahmet Kazez
Department of Pediatric Surgery, Fırat University, Elazig
Turkey

Abstract

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



How to cite this article:
Gurbaz MT, Kazez A, Bakal U, Tartar T, Ersoz F, Colakoglu Y. Congenital diaphragmatic hernia in identical twins.J Indian Assoc Pediatr Surg 2012;17:26-27


How to cite this URL:
Gurbaz MT, Kazez A, Bakal U, Tartar T, Ersoz F, Colakoglu Y. Congenital diaphragmatic hernia in identical twins. J Indian Assoc Pediatr Surg [serial online] 2012 [cited 2019 Dec 7 ];17:26-27
Available from: http://www.jiaps.com/text.asp?2012/17/1/26/91083


Full Text

 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],[3],[4],[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 6 th and baby B was operated on the 7 th 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}{Figure 2}

 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],[3],[4],[5] The spleen was also located in the thorax in one pair. [2]{Table 1}

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 5 th pair, diagnosis was made on the 5 th day in one baby and in another it was diagnosed in the 6 th 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.

References

1Corbett HJ, Losty PD. Congenital diaphragmatic hernia. In: Rich DH, Crabbe DC, Auldist AW, Rothenberg SS, editors. Pediatric Thoracic Surgery. London: Springer-Verlag; 2009. p. 483-99.
2Hitch DC, Carson JA, Smith EI, Salare DC, Rennert OM. Familial diaphragmatic hernia is an autosomal recessive variant. J Pediatr Surg 1989;24:860-4.
3Mishalany H, Gordo J. Congenital diaphragmatic hernia in monozygotic twins. J Pediatr Surg 1986;21:372-4.
4Tazuke Y, Kawahara H, Soh H, Yoneda A, Yagi M, Imura K, et al. Congenital diaphragmatic hernia in identical twins. Pediatr Surg Int 2000;16:512-4.
5Watanatittan S. Congenital diaphragmatic hernia in identical twins. J Pediatr Surg 1983;18:628-9.