Association of hiatus hernia with asplenia syndrome

Wang, Jou‐Kou; Chang, Mei–Hwei

doi:10.1016/0002-9149(95)80055-w

Cited by 4 publications

(7 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Due to lateralization failure in the embryo stage of heterotaxy syndrome, the ligaments that fix the GI tracts are usually defective, and this leads to malrotation, or even volvulus of the gut. It is noteworthy that only rarely has hiatal hernia been reported in right isomerism 1, 9, 10. In fact, in the normal population, the incidence of this is reported to be only 0.1%, a stark contrast to the 14.3% in the present study 18.…”

Section: Discussioncontrasting

confidence: 84%

“…It has previously been estimated that 40% of patients with heterotaxy syndrome have digestive tract disorders 9. In an earlier study, we reported four patients with right isomerism who had hiatal hernia 10. Hiatal hernia in patients with situs solitus may cause a disruption in the gastro‐oesophageal sphincter and subsequent gastro‐oesophageal reflux.…”

Section: Introductionmentioning

confidence: 85%

“…GI tract anomalies associated with heterotaxy syndrome, including malrotation, malposition and malfixation of the abdominal organs, have been reported in the literature 1–39, 10,16, 17. Due to lateralization failure in the embryo stage of heterotaxy syndrome, the ligaments that fix the GI tracts are usually defective, and this leads to malrotation, or even volvulus of the gut.…”

Section: Discussionmentioning

confidence: 99%

“…Hiatal hernia in patients with situs solitus may cause a disruption in the gastro‐oesophageal sphincter and subsequent gastro‐oesophageal reflux. Though the findings mentioned above have been widely accepted, the clinical impact of hital hernia on the long‐term outcome of patients with right isomerism has largely remained unclear 9, 10. Now that improved survival from cardiac intervention is possible, it is important that the clinical implication of hiatal hernia in patients with right isomerism be more clearly defined and explained.…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Clinical implication of hiatal hernia in patients with right isomerism

et al. 2005

Self Cite

View full text Add to dashboard Cite

Objective: Despite a reported association between hiatal hernia in patients with heterotaxy syndrome, the clinical significance has remained unclear. Based on large patient cohorts, this study specifically aimed at defining the implication of hiatal hernia in patients with right isomerism. Methods: From 1994 to 2002, 143 patients were identified as having right isomerism. Among them, 119 received ultrafast computed tomography (CT) to determine the presence of hiatal hernia as well as any cardiovascular anomalies. Results: Hiatal hernia was found in 17 patients (seven females and 10 males, 14.3%). The upper gastrointestinal (GI) series in six patients confirmed the diagnosis of hiatal hernia in all and revealed severe gastro‐oesophageal reflux in four. The most common symptom of hiatal hernia was vomiting (47%), followed by recurrent bronchiolitis or pneumonia (41.2%) and upper gastrointestinal bleeding (11.8%). Three patients with hiatal hernia underwent fundoplication for medically refractory vomiting. Seven patients were found to have midgut malrotation (5.9%), and four of these had both hiatal hernia and malrotation. Pulmonary atresia was closely associated with the presence of hiatal hernia (p=0.02). One patient with hiatal hernia died suddenly at 6 mo. However, overall mortality was similar between those patients with hiatal hernia and those without. Conclusions: The incidence of hiatal hernia was considerably high in patients with right isomerism, especially in those with pulmonary atresia, and it is this that may have led to vomiting and recurrent airway infections. While an association between sudden death and hiatal hernia may well exist, this does require further clarification.

show abstract

Section: Discussioncontrasting

confidence: 84%

Section: Introductionmentioning

confidence: 85%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Clinical implication of hiatal hernia in patients with right isomerism

et al. 2005

Self Cite

View full text Add to dashboard Cite

show abstract

“…Asplenia usually occurs as an isolated abnormality (Lindor et al, 1995) or less commonly as recognized syndrome -a rare congenital complex of splenic agenesis, cardiac malformation, malposition of abdominal viscera and various gastrointestinal abnormalities including biliary atresia, duodenal stenosis, imperforate anus, hischurungs disease, hiatus hernia, etc. (Berman, 1982;De Mingo et al, 1992;Hausdorf, 1983;Hutchins et al, 1982;Shirotani et al, 1983;Tunauglu et al, 1991;Wang, 1991;and Wang, 1993).…”

Section: Discussionmentioning

confidence: 99%

Congenital Asplenia In A Surgical Patient

Okpala¹,

Petit²

2008

Jnl Sci Tech

View full text Add to dashboard Cite

Congenital asplenia is rare and could occur sporadically or less commonly as part of a syndrome. Sporadic or isolated congenital asphenia is not usually associated with significant morbidity. Congenical isolated asphenia can be present as septicaemia, commonly pneumococcal, hence the importance of screening the peripheral blood for Howell-Jolly bodies. Abdominal imaging becomes necessary if screening is positive. An isolated case of congenital asphenia is discovered by chance in a 69 year-old man, after falling off a ladder from a height of about 15 feet. Standard surgical procedure was followed in managing the patient. Here, abdominal ultrasound commented on the absence of the spleen followed trauma. Fracture of the spleen was suspected hence exploratory laparotomy was performed. To the best of our knowledge, this report is probably an isolated abnormality and most likely a sporadic case.

show abstract

Surgical Management of Hiatal Hernia in Children with Asplenia Syndrome

et al. 2016

View full text Add to dashboard Cite

Patients with asplenia syndrome (AS) are likely to have upper gastrointestinal tract malformations such as hiatal hernia. This report discusses the treatment of such conditions. Seventy-five patients with AS underwent initial palliation in our institution between 1997 and 2013. Of these, 10 patients had hiatal hernia. Of the patients with hiatal hernia, 6 had brachyesophagus and 7 had microgastria. Of the 10 patients with hiatal hernia, 9 underwent surgery in infancy (7 before Glenn operation, 2 after Glenn operation). Two underwent typical Toupet fundoplication, and the other 7 underwent atypical repair including reduction of the stomach. Two patients with atypical repair showed recurrence of hernia and required reoperation. Three patients required reoperation due to duodenal obstruction. Duodenal obstruction occurred due to preduodenal portal vein or abnormal vessels compressing the duodenum. Obstructive symptoms were not seen in any cases preoperatively. In patients with hiatal hernia, typical fundoplication is often difficult because most have concomitant brachyesophagus, microgastria, and hypoplasia of the esophageal hiatus. However, we should at least reduce the stomach to the abdominal cavity as early as possible to increase thoracic cavity volume and allow good feeding. Increasing the volume of the thoracic cavity thus makes Glenn and Fontan circulations more stable. Duodenal obstruction secondary to vascular anomalies is also common, so the anatomy in the area near the duodenum should be evaluated pre- and intraoperatively.

show abstract

Association of hiatus hernia with asplenia syndrome

Cited by 4 publications

References 18 publications

Clinical implication of hiatal hernia in patients with right isomerism

Clinical implication of hiatal hernia in patients with right isomerism

Congenital Asplenia In A Surgical Patient

Surgical Management of Hiatal Hernia in Children with Asplenia Syndrome

Contact Info

Product

Resources

About