An unusual case: a giant paraesophageal hiatal hernia with intrathoracic spleen, preduodenal portal vein, malrotation, and left inferior vena cava

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Journal of Pediatric Surgery (2007) 42, E23–E25

An unusual case: a giant paraesophageal hiatal hernia with intrathoracic spleen, preduodenal portal vein, malrotation, and left inferior vena cava A. Can Başaklar, Kaan Sönmez, Ramazan Karabulut ⁎, Zafer Türkyılmaz, Serdar Moralıoğlu Department of Pediatric Surgery, Faculty of Medicine, Gazi University, Gazi University, 06500 Ankara, Turkey Received 28 June 2007; revised 13 August 2007; accepted 20 August 2007

Index words: Paraesophageal hernia; Nonrotating; Preduodenal portal vein; Intrathoracic spleen; Left inferior vena cava

Abstract A giant paraesophageal hiatal hernia with preduodenal portal vein, nonrotating gut, intrathoracic spleen, and left inferior vena cava has not been reported to date. This set of complex anomalies can have significant clinical implications. Awareness of these anomalies is essential to avoid further complications. © 2007 Elsevier Inc. All rights reserved.

Primary paraesophageal hiatal hernias (PHH) are rare in children. Paraesophageal hiatal hernia may present with different clinical problems including gastric volvulus, hematemesis, vomiting, failure to thrive, and respiratory distress. Because of the high incidence of complications, surgical repair of PHH is generally indicated [1-3]. This is the first report to describe an unusual complex of abdominal anomalies including a giant PHH with intrathoracic spleen, preduodenal portal vein, nonrotating gut, and left inferior vena cava (IVC) demonstrated at laparotomy for repair of PHH.

1. Case report A 5-year-old boy was referred to our hospital for evaluation of fever and cough. The physical examination showed no obvious abnormalities. The plain chest radio⁎ Corresponding author. Tel.: +90 312 2026210; fax: +90 312 2230528. E-mail address: [email protected] (R. Karabulut). 0022-3468/$ – see front matter © 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2007.08.055

graph demonstrated a lucent area over the cardiac shadow. In addition, a butterfly vertebra in the third thoracic vertebrae, fusion between the left second and third ribs posteriorly, and scoliosis of left upper thorax were observed. Computed tomography (CT) showed intrathoracic position of a large part of the stomach and the spleen, and air densities were observed in the stomach. The appearance of circular structures on both sides of the spleen was interpreted as stomach folds. A linear density extending from the hilum of the spleen to the right and anterior was interpreted as mesenteric fat tissues (Fig. 1). Barium contrast swallow showed gastric herniation into the chest (Fig. 2). At the time of a midline supraumbilical laparotomy, all the colon segments were observed on the left; on the cecum, in the lower left quadrant; and on the small intestines, on the left. In the upper left quadrant, neither the stomach nor the spleen was observed. A large (8 × 3 cm) PHH with intrathoracic spleen without any signs of torsion but with normal ligamentous attachment was observed. The esophagus and gastroesophageal junction (GEJ) were in a normal position.


Fig. 1 Computed tomography shows intrathoracic position of a large part of the stomach and the spleen. The circular structures observed on the spleen on CT are stomach folds and do not exist in the lower sections. In addition, a large paraesophageal hiatal defect is seen, and the vena cava enters the thorax in its normal position. The linear density on the spleen was interpreted as mesenteric fat tissue (MFT).

The greater curvature of the stomach was in an anterior and higher position and had undergone normal organo-axial rotation.. However, because of a large hiatal defect and the superiorly located stomach and spleen, the esophagus appeared to have an upper left position. In addition, a left IVC, a long right renal vein, nonrotation of the bowel, and preduodenal portal vein (PDPV) were detected. The duodenum was unobstructed. This large (PDPV) vein passed ventrally to the duodenum and pancreas. The left IVC ended at the right renal vein; the left renal vein crossed anterior to the aorta to form a normal right-sided prerenal IVC. Reduction of the stomach and spleen and excision of the hernia sac were followed by narrowing the widely open hiatus by suture approximation of the crura. The peritoneal folds causing an adhesion between the portal area and duodenum were separated and an appendectomy was performed because of nonrotation of the bowel. The postoperative course was uneventful and the child was discharged on the sixth postoperative day. The patient was well at the time of follow-up examination 2 months later. Routine follow-up visits were scheduled and the patient's family was informed of the possible postoperative complications.

A.C. BasSaklar et al. develop on either side of the midline in the mediastinum. The recess on the left side is transitory. Persistence of the recess on the right creates a flattened and elongated mesothelial lined space within the esophageal hiatus. The space is approximately 1 cm long at birth and may become obliterated during adulthood [4-6]. Alternatively, an aberration of embryonic development of the lumbar component of the diaphragm, which originates from mesodermal cells around the aorta, may be responsible. The hernias have been diagnosed prenatally and shortly after birth and in siblings, further supporting the theory that these patients have an abnormal anatomical predisposition [2]. Paraesophageal hiatal hernia can surprisingly be asymptomatic (8%). With time, symptoms of postprandial discomfort, breathlessness, nausea, vomiting, and pain may develop. Obstruction caused by gastric torsion can occur at the GEJ, mid body of the stomach, or duodenum. Symptoms and signs will be determined by these factors and are caused primarily by displacement of the stomach with entrapment of gas and food in the intrathoracic segment. Dysphagia is uncommon. In true anatomical PHH, the GEJ remains in its normal position, and the sphincter mechanism therefore remains intact. However, with cephalad displacement, the sphincter may become dysfunctional and pathologic gastroesophageal reflux (GER) may result. Other structures, such as the colon, small bowel, duodenum, and

2. Discussion The incidence of PHH varies from 3.5% to 5% of all operated hiatus hernias [2]. Paraesophageal hiatal hernia in childhood is thought to be caused by a congenital abnormality that can be explained on an embryologic basis. During the development of the human diaphragm, 2 small coelomic spaces, called pneumoenteric recesses,

Fig. 2 Upper gastrointestinal contrast study showing gastric (STMC) herniation into the chest. End of the inferior esophagus has moved to the left owing to the hernia and the spleen.

PHH with intrathoracic spleen, preduodenal portal vein, malrotation and left IVC even spleen, may accompany the stomach's migration into the chest and may complicate the situation further [1]. The diagnosis is usually first suspected because of an abnormal chest radiograph. Opaque study of the upper gastrointestinal tract is the diagnostic study of choice. A barium swallow provides the diagnosis in virtually every case. The pathognomonic findings are an “upside down” stomach within the chest in association with a normally located GEJ. The GEJ remains fixed in its normal infradiaphragmatic location. In the literature, PHH is of clinical significance because of its very high incidence of GER (68.4%). In patients with massive hiatal hernia and GER, acquired shortening is the result of inflammatory changes in the thoracic esophagus, secondary to acute and chronic reflux esophagitis [1,3]. The length of the esophagus is a criterion for determining the type of antireflux procedure. The recurrence rate is high when the esophagus is short and no attempt is made to lengthen it during the antireflux surgery [3]. No acceptable medical treatment regimen exists for patients with PHH. Complications are more prevalent in PHH than in sliding hiatus hernias, and elective repair is advocated on diagnosis, even in asymptomatic cases. An abdominal surgical approach is usually preferred. The principles of repair consist of reduction of the hernia, partial or complete excision of the accompanying sac to prevent recurrence and cyst formation, and crural approximation [1-3]. If the operation is delayed and repair is done on an emergency basis, there is a 19% operative mortality, compared with less than 1% mortality noted for elective repair [7]. In our patient, no previous associated symptoms of GER or a developmental disorder was noted. Thus, fundoplication was not performed. Preduodenal portal vein is a rare anomaly described in only 82 patients in the literature and usually as a part of “polysplenia syndrome,” or as an associated anomaly in situs inversus [8]. The embryogenesis of this anomaly described by Gray and Skandalakis [9] consisted of anomalous regression of the cranial and middle anastomotic veins, which connect the 2 vitelline veins that run on either side of the duodenum in the 5-mm embryo. The caudal anastomotic vein that runs ventral to the duodenum remains a part of the portal vein and results in the anomalous position of the portal vein [8,9]. It presents clinically in 50% of the patients as duodenal obstruction, but only in a few of cases is the PDPV apparently the direct cause of obstruction; the rest were caused by associated anomalies such as duodenal membrane, annular pancreas, and intestinal malrotation. In the remaining 50%, PDPV was asymptomatic and diagnosed incidentally during surgery. Associated cardiac and gastrointestinal anomalies are frequent. It is described in polysplenia syndrome and in patients with situs inversus [8,10,11]. However, to date, no reports of a paraesophageal hernia and associated complex anomalies have been made. A left IVC results from regression of the right supracardinal vein with persistence of the left supracardinal vein.


The prevalence is 0.2% to 0.5%. Typically, the left IVC joins the left renal vein, which crosses anterior to the aorta in the normal fashion, uniting with the right renal vein to form a normal right-sided prerenal IVC. Spontaneous rupture of an abdominal aortic aneurysm into a left IVC has been reported [12]. Wandering spleen is a rare condition that is difficult to diagnose owing to its nonspecific symptoms. A frequent complaint is recurrent abdominal pain caused by intermittent torsion or vascular kinking. Left-sided diaphragmatic defects can be associated with abnormally positioned spleen and can be a cause of acute abdomen in children. Wandering spleen in association with gastric volvulus has been reported only 4 times in the literature [13,14]. Complex malformations, such as the ones described in this report, may be encountered during operations. In such cases, primary treatment may be directed toward the symptomatic component of the malformation and the patient should be closely followed to avoid further complications associated with the other malformation.

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