Answer for BIR CoW 06 Nov 2022
Congenital hiatal hernia
IMAGING FINDINGS:
The ultrasound examination was performed and on the transverse section of the thorax, the heart was normally located, without any apparent mediastinal shift. The lungs showed normal echogenicity and volume. A round cystic structure was noted behind the heart in the posterior mediastinum. In sagittal section, there was absent normal stomach bubble in the abdomen in addition to the cystic structure in posterior fetal thorax. During the examination period, there was no change in size and shape of the cystic structure.
DIAGNOSIS:
The presence of a cystic mass in the fetal thorax made us to consider the differential diagnosis of congenital diaphragmatic hernia, cystic pulmonary adenomatoid malformation of the lung, or rarer conditions such as esophageal duplication cyst, bronchogenic cyst, neuroenteric cyst, teratoma or lymphangioma. Hiatal hernia is usually not commonly considered in the differential diagnosis. But the presence of cystic lesion located adjacent to posterior to heart should raise the suspicion.
FOLLOW UP:
The parents were counselled and on follow up ultrasound at 37 weeks, the cystic structure was more prominent and dilated. There was polyhydramnios also; suggestive of gastric outlet obstruction. She underwent elective caesarean section; with expert team of obstetrician, neonatologist and pediatric surgeon, ready for safe delivery and further management. She delivered a low-birth-weight baby without any respiratory distress; saturation was well maintained in the postnatal period. The abdominal radiograph on day 1 of neonatal life revealed a gasless abdomen, with air filled radiolucent structure in retrocardiac region and coiling of nasogastric feeding tube in chest itself. Computed tomography of the baby’s chest and abdomen was also performed; revealing the same. Elective laparotomy was performed and found to have hiatal hernia type 2; hernial reduction and posterior fundoplication was performed. Post procedure, the baby recovered well with post operative abdominal radiograph on day 7 showing normal bowel gas pattern.
Discussion
Hiatal hernia is defined as the herniation of the abdominal organs into the chest, through the esophageal diaphragmatic hiatus. It usually occurs due to the physiologically lax esophagus but rarely associated with primary diaphragmatic defect.There are four types of Hiatal hernia: • Type I - sliding hiatus hernia; • Type II - “rolling” or “pure” paraesophageal hernia, usually associated with primary diaphragmatic defect; • Type III - combination of sliding and paraesophageal hernia with herniation of gastroesophageal junction; • Type IV, essentially a large type III hernia, which may contain the entire stomach, omentum, or other abdominal viscera. Congenital hiatal hernia is a very rare entity with very few reported cases in literature. The antenatal diagnosis is usually made in the third trimester. The earlier the diagnosis poorer will be the prognosis; mainly due to pulmonary hypoplasia and pulmonary hypertension.
REFERENCES:
1. Bahado-Singh R. O., Romero R., Vecchio M., Hobbins J. C. Prenatal diagnosis of congenital hiatal hernia. Journal of Ultrasound in Medicine. 1992;11(6):297–300 2. Khanna A., Finch G. Paraoesophageal herniation: a review. Surgeon. 2011;9(2):104–111. doi: 10.1016/j.surge.2010.10.010. 3. Di Francesco S, Lanna MM, Napolitano M, et al. A case of ultrasound diagnosis of fetal hiatal hernia in late third trimester of pregnancy. Case Rep Obstet Gynecol. 2015;2015:194090. doi:10.1155/2015/194090
IMAGING FINDINGS:
The ultrasound examination was performed and on the transverse section of the thorax, the heart was normally located, without any apparent mediastinal shift. The lungs showed normal echogenicity and volume. A round cystic structure was noted behind the heart in the posterior mediastinum. In sagittal section, there was absent normal stomach bubble in the abdomen in addition to the cystic structure in posterior fetal thorax. During the examination period, there was no change in size and shape of the cystic structure.
DIAGNOSIS:
The presence of a cystic mass in the fetal thorax made us to consider the differential diagnosis of congenital diaphragmatic hernia, cystic pulmonary adenomatoid malformation of the lung, or rarer conditions such as esophageal duplication cyst, bronchogenic cyst, neuroenteric cyst, teratoma or lymphangioma. Hiatal hernia is usually not commonly considered in the differential diagnosis. But the presence of cystic lesion located adjacent to posterior to heart should raise the suspicion.
FOLLOW UP:
The parents were counselled and on follow up ultrasound at 37 weeks, the cystic structure was more prominent and dilated. There was polyhydramnios also; suggestive of gastric outlet obstruction. She underwent elective caesarean section; with expert team of obstetrician, neonatologist and pediatric surgeon, ready for safe delivery and further management. She delivered a low-birth-weight baby without any respiratory distress; saturation was well maintained in the postnatal period. The abdominal radiograph on day 1 of neonatal life revealed a gasless abdomen, with air filled radiolucent structure in retrocardiac region and coiling of nasogastric feeding tube in chest itself. Computed tomography of the baby’s chest and abdomen was also performed; revealing the same. Elective laparotomy was performed and found to have hiatal hernia type 2; hernial reduction and posterior fundoplication was performed. Post procedure, the baby recovered well with post operative abdominal radiograph on day 7 showing normal bowel gas pattern.
Discussion
Hiatal hernia is defined as the herniation of the abdominal organs into the chest, through the esophageal diaphragmatic hiatus. It usually occurs due to the physiologically lax esophagus but rarely associated with primary diaphragmatic defect.There are four types of Hiatal hernia: • Type I - sliding hiatus hernia; • Type II - “rolling” or “pure” paraesophageal hernia, usually associated with primary diaphragmatic defect; • Type III - combination of sliding and paraesophageal hernia with herniation of gastroesophageal junction; • Type IV, essentially a large type III hernia, which may contain the entire stomach, omentum, or other abdominal viscera. Congenital hiatal hernia is a very rare entity with very few reported cases in literature. The antenatal diagnosis is usually made in the third trimester. The earlier the diagnosis poorer will be the prognosis; mainly due to pulmonary hypoplasia and pulmonary hypertension.
REFERENCES:
1. Bahado-Singh R. O., Romero R., Vecchio M., Hobbins J. C. Prenatal diagnosis of congenital hiatal hernia. Journal of Ultrasound in Medicine. 1992;11(6):297–300 2. Khanna A., Finch G. Paraoesophageal herniation: a review. Surgeon. 2011;9(2):104–111. doi: 10.1016/j.surge.2010.10.010. 3. Di Francesco S, Lanna MM, Napolitano M, et al. A case of ultrasound diagnosis of fetal hiatal hernia in late third trimester of pregnancy. Case Rep Obstet Gynecol. 2015;2015:194090. doi:10.1155/2015/194090
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!