- Email: [email protected]
Gastric antrum hypertrophy causing outlet obstruction in an infant with congenital diaphragmatic hernia
Journal of Pediatric Surgery (2011) 46, E11–E14
www.elsevier.com/locate/jpedsurg
Gastric antrum hypertrophy causing outlet obstruction in an infant with congenital diaphragmatic hernia Ahmad Saad a,⁎, Barton Kenney b , Robert Touloukian a a
Section of Pediatric Surgery, Yale University School of Medicine and Yale New Haven Children's Hospital, New Haven, CT 06520, USA b Department of Laboratory Medicine, Yale University School of Medicine, New Haven, CT 06520, USA Received 8 November 2010; revised 5 January 2011; accepted 14 February 2011
Key words: Congenital diaphragmatic hernia (CDH); Antrum hypertrophy; Gastric outlet obstruction
Abstract Congenital diaphragmatic hernia (CDH) is associated with multiple congenital anomalies affecting several organ systems, including the gastrointestinal system. Pyloric stenosis and bands are known and previously reported etiologies of gastric outlet obstruction in infants with CDH. We report the first case of gastric antrum hypertrophy causing gastric outlet obstruction in an infant with CDH. © 2011 Elsevier Inc. All rights reserved.
Major anomalies have been associated with congenital diaphragmatic hernia (CDH), in approximately 39% of liveborn cases [1], rising to approximately 95% in those with intrauterine death [2]. The most commonly encountered anomalies are cardiac, renal, central nervous system, and gastrointestinal [3]. Both major and minor gastrointestinal anomalies have been reported in association with 14% of cases of CDH. The most common gastrointestinal associations are intestinal fixation anomalies and gastroesophageal reflux. Several cases of hypertrophic pyloric stenosis have been reported [4,5], with an incidence of 1.2% in a recently published database of outcomes with CDH [6]. Feeding intolerance, secondary to ileus and gastroesophageal reflux, is often experienced postrepair; but more rarely encountered conditions may be overlooked. We report such a patient with delayed diagnosis of gastric antrum stenosis causing outlet obstruction. ⁎ Corresponding author. Tel.: +1 203 785 7890; fax: +1 203 737 5209. E-mail addresses: [email protected] (A. Saad), [email protected] (B. Kenney), [email protected] (R. Touloukian). 0022-3468/$ – see front matter © 2011 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2011.02.062
1. Case A 3.2-kg male infant was born after 39 weeks of gestation, by cesarean section, to a 33-year-old gravida 1 mother. Diagnosis of left CDH was known by prenatal ultrasound at 21 weeks of gestation with the stomach and small bowel seen in the left hemithorax. Gestation was complicated by diabetes and polyhydramnios. The infant was immediately intubated; an orogastric suction tube was placed for decompression. Chest x-ray at birth showed evidence of left diaphragmatic hernia and mediastinal shift to the right (Fig. 1). The infant maintained oxygen saturation of 95% on room air but gradually became tachypneic with a respiratory rate of 40 to 50 breaths per minute. Echocardiogram and renal ultrasound studies were normal. On the fourth day of life, the infant was taken to the operating room for open abdominal repair of CDH. The stomach, spleen, loops of small intestine, left kidney, and adrenal were found in the left hemithorax. The posterior leaf of the diaphragm was absent. The abdominal viscera were reduced back to the abdominal cavity, the diaphragmatic
E12
Fig. 1 Initial chest x-ray showing bowel gas pattern in the left hemithorax with mediastenal shift to the right consistent with CDH.
A. Saad et al. defect was closed using 4.0 silk sutures approximating the anterior portion of the diaphragm to the posterior ribs, and a left chest tube was placed. At this time, the bowel was noted to be air filled and distended, requiring ventral hernia repair. On postoperative day 4, the abdomen was primarily closed. During his hospital course, the infant was noted to have increased orogastric tube output. On the 18th day of life, radiographic studies showed absent gas and contrast in the bowel distal to the stomach (Fig. 2). A Ladd procedure was performed for obstruction with duodenal bands extending to the pylorus. At that time, air could be expressed from the pylorus into the small bowel. On the fifth week of life, the infant continued to have high orogastric tube output with intolerance of tube feeds. Upper gastrointestinal study continued to show contrast in the stomach without further progression into the small intestine. At reexploration, a proximal gastrostomy was performed, and a stenotic area was identified in the gastric antrum. The pylorus and small intestine were normal distal to the stenosis. A proximal antrum gastrectomy with gastrogastric anastomosis was performed. Postoperatively, the infant tolerated tube feeds. Upper gastrointestinal contrast study 4 weeks after partial gastrectomy showed a rapid emptying of contrast from stomach into intestine. The infant was discharged home at 2 1/2 months of age on full feeds.
2. Pathology Fig. 2 Upper gastrointestinal study at 21 days of life shows no progression of the contrast beyond the stomach.
Histologic examination of the resection specimen revealed prominent hypertrophic changes within the gastric
Fig. 3 A, Muscular hypertrophy, with abundant, haphazardly arranged muscle fibers (hematoxylin and eosin [H&E], original magnification ×10). B, Red histochemical staining with trichrome, confirming muscle differentiation and revealing no significant component of fibrosis (Klatskin trichrome, original magnification ×10). C, Positive immunostaining for smooth muscle actin, again confirming muscle differentiation (smooth muscle actin immunostain, original magnification ×10).
Gastric antrum hypertrophy causing outlet obstruction
E13
Fig. 4 A, Nerve hypertrophy (H&E, original magnification ×4; arrows indicate irregular and hypertrophic nerve bundles within the myenteric plexus). B, S100 immunostain confirming nerve hypertrophy (S100 immunostain, original magnification ×4; brown areas highlight hypertrophic nerve). C, PGP9.5 immunostain, again confirming nerve hypertrophy(PGP9.5 immunostain, original magnification ×4; brown areas highlight hypertrophic nerve.
Fig. 5 A, Prepyloric gastric muscular hypertrophy (H&E, original magnification ×20). B, Gastric antral mucosa with ischemic injury and superficial necrosis (H&E, original magnification ×100). C, Transition between ischemic gastric mucosa (left) and duodenal-type epithelium (right) (H&E, original magnification ×50). D, Duodenal metaplasia; note villous architecture and presence of goblet cells (H&E, original magnification ×200).
E14
A. Saad et al.
muscularis propria, characterized by nodular muscular proliferation and disorganized intersection of smooth muscle fascicles (Fig. 3). The associated myenteric plexus also appeared hypertrophic, with a notable increase in autonomic nerve bundles scattered variously throughout the muscle layers (Fig. 4). Interestingly, in addition to ischemic injury to the gastric mucosa, there was evidence of enteric metaplasia, with formation of rudimentary villi and epithelial transition toward a goblet cell–rich columnar phenotype (Fig. 5).
The incidence of feeding intolerance after repair of CDH often necessitates contrast studies to evaluate the differential diagnosis of prepyloric and postpyloric gastric outlet obstruction. We are reporting the first known case of antral stenosis secondary to mucosal, muscular, and neurogenic hypertrophy. The possibility must be considered in the future to avoid delayed diagnosis and misdiagnosis when pyloric stenosis and bands are often considered first.
3. Discussion
References
The etiology of prepyloric stenosis, with mucosal metaplasia, in a patient with CDH is unknown but shows the importance of the milieu and the conditions in which organogenesis occurs in early fetal life. In vitro and in vivo studies have been done and described in the literature, in which human pluripotent stem cells developed into different tissue subtypes depending on the local milieu and microenvironment [7]. The intrauterine location of the stomach in the fetal chest, and the atypical physiologic conditions for upper gastrointestinal development, might have created a new milieu where tissue hyperplasia occurred more proximal than the usual (ie, pylorus) along with the transition point from gastric to duodenal mucosa. This anomalous location of foregut structures in the chest might have disrupted the normal development of the stomach and proximal duodenum from the foregut, a process that usually starts as early as 4 weeks of gestation.
[1] Skari H, Bjornland K, Haugen G, et al. Congenital diaphragmatic hernia: a meta-analysis of mortality factors. J Pediatr Surg 2000;35: 1187-97. [2] Puri P, Gorman F. Lethal nonpulmonary anomalies associated with congenital diaphragmatic hernia: implications for early intrauterine surgery. J Pediatr Surg 1984;19:29-32. [3] Skarsgard ED, Harrison MR. Congenital diaphragmatic hernia: the surgeon's perspective. Pediatr Rev 1999;20:71-8. [4] Al-Salem AH, Grant C, Khwaja S. Infantile hypertrophic pyloric stenosis and congenital diaphragmatic hernia. J Pediatr Surg 1990;25: 607-8. [5] Redman M, Ross DA. Hypertrophic pyloric stenosis following repair of congenital diaphragmatic hernia. J Pediatr Surg 1979;14:607. [6] Abdullah F, Zhang Y, Sciortino C, et al. Congenital diaphragmatic hernia: outcome review of 2,173 surgical repairs in US infants. Pediatr Surg Int 2009;25:1059-64. [7] Moosmann S, Hutter J, Moser C, et al. Milieu-adopted in vitro and in vivo differentiation of mesenchymal tissues derived from different adult human CD34-negative progenitor cell clones. Cells Tissues Organs 2005;179:91-101.
www.elsevier.com/locate/jpedsurg
Gastric antrum hypertrophy causing outlet obstruction in an infant with congenital diaphragmatic hernia Ahmad Saad a,⁎, Barton Kenney b , Robert Touloukian a a
Section of Pediatric Surgery, Yale University School of Medicine and Yale New Haven Children's Hospital, New Haven, CT 06520, USA b Department of Laboratory Medicine, Yale University School of Medicine, New Haven, CT 06520, USA Received 8 November 2010; revised 5 January 2011; accepted 14 February 2011
Key words: Congenital diaphragmatic hernia (CDH); Antrum hypertrophy; Gastric outlet obstruction
Abstract Congenital diaphragmatic hernia (CDH) is associated with multiple congenital anomalies affecting several organ systems, including the gastrointestinal system. Pyloric stenosis and bands are known and previously reported etiologies of gastric outlet obstruction in infants with CDH. We report the first case of gastric antrum hypertrophy causing gastric outlet obstruction in an infant with CDH. © 2011 Elsevier Inc. All rights reserved.
Major anomalies have been associated with congenital diaphragmatic hernia (CDH), in approximately 39% of liveborn cases [1], rising to approximately 95% in those with intrauterine death [2]. The most commonly encountered anomalies are cardiac, renal, central nervous system, and gastrointestinal [3]. Both major and minor gastrointestinal anomalies have been reported in association with 14% of cases of CDH. The most common gastrointestinal associations are intestinal fixation anomalies and gastroesophageal reflux. Several cases of hypertrophic pyloric stenosis have been reported [4,5], with an incidence of 1.2% in a recently published database of outcomes with CDH [6]. Feeding intolerance, secondary to ileus and gastroesophageal reflux, is often experienced postrepair; but more rarely encountered conditions may be overlooked. We report such a patient with delayed diagnosis of gastric antrum stenosis causing outlet obstruction. ⁎ Corresponding author. Tel.: +1 203 785 7890; fax: +1 203 737 5209. E-mail addresses: [email protected] (A. Saad), [email protected] (B. Kenney), [email protected] (R. Touloukian). 0022-3468/$ – see front matter © 2011 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2011.02.062
1. Case A 3.2-kg male infant was born after 39 weeks of gestation, by cesarean section, to a 33-year-old gravida 1 mother. Diagnosis of left CDH was known by prenatal ultrasound at 21 weeks of gestation with the stomach and small bowel seen in the left hemithorax. Gestation was complicated by diabetes and polyhydramnios. The infant was immediately intubated; an orogastric suction tube was placed for decompression. Chest x-ray at birth showed evidence of left diaphragmatic hernia and mediastinal shift to the right (Fig. 1). The infant maintained oxygen saturation of 95% on room air but gradually became tachypneic with a respiratory rate of 40 to 50 breaths per minute. Echocardiogram and renal ultrasound studies were normal. On the fourth day of life, the infant was taken to the operating room for open abdominal repair of CDH. The stomach, spleen, loops of small intestine, left kidney, and adrenal were found in the left hemithorax. The posterior leaf of the diaphragm was absent. The abdominal viscera were reduced back to the abdominal cavity, the diaphragmatic
E12
Fig. 1 Initial chest x-ray showing bowel gas pattern in the left hemithorax with mediastenal shift to the right consistent with CDH.
A. Saad et al. defect was closed using 4.0 silk sutures approximating the anterior portion of the diaphragm to the posterior ribs, and a left chest tube was placed. At this time, the bowel was noted to be air filled and distended, requiring ventral hernia repair. On postoperative day 4, the abdomen was primarily closed. During his hospital course, the infant was noted to have increased orogastric tube output. On the 18th day of life, radiographic studies showed absent gas and contrast in the bowel distal to the stomach (Fig. 2). A Ladd procedure was performed for obstruction with duodenal bands extending to the pylorus. At that time, air could be expressed from the pylorus into the small bowel. On the fifth week of life, the infant continued to have high orogastric tube output with intolerance of tube feeds. Upper gastrointestinal study continued to show contrast in the stomach without further progression into the small intestine. At reexploration, a proximal gastrostomy was performed, and a stenotic area was identified in the gastric antrum. The pylorus and small intestine were normal distal to the stenosis. A proximal antrum gastrectomy with gastrogastric anastomosis was performed. Postoperatively, the infant tolerated tube feeds. Upper gastrointestinal contrast study 4 weeks after partial gastrectomy showed a rapid emptying of contrast from stomach into intestine. The infant was discharged home at 2 1/2 months of age on full feeds.
2. Pathology Fig. 2 Upper gastrointestinal study at 21 days of life shows no progression of the contrast beyond the stomach.
Histologic examination of the resection specimen revealed prominent hypertrophic changes within the gastric
Fig. 3 A, Muscular hypertrophy, with abundant, haphazardly arranged muscle fibers (hematoxylin and eosin [H&E], original magnification ×10). B, Red histochemical staining with trichrome, confirming muscle differentiation and revealing no significant component of fibrosis (Klatskin trichrome, original magnification ×10). C, Positive immunostaining for smooth muscle actin, again confirming muscle differentiation (smooth muscle actin immunostain, original magnification ×10).
Gastric antrum hypertrophy causing outlet obstruction
E13
Fig. 4 A, Nerve hypertrophy (H&E, original magnification ×4; arrows indicate irregular and hypertrophic nerve bundles within the myenteric plexus). B, S100 immunostain confirming nerve hypertrophy (S100 immunostain, original magnification ×4; brown areas highlight hypertrophic nerve). C, PGP9.5 immunostain, again confirming nerve hypertrophy(PGP9.5 immunostain, original magnification ×4; brown areas highlight hypertrophic nerve.
Fig. 5 A, Prepyloric gastric muscular hypertrophy (H&E, original magnification ×20). B, Gastric antral mucosa with ischemic injury and superficial necrosis (H&E, original magnification ×100). C, Transition between ischemic gastric mucosa (left) and duodenal-type epithelium (right) (H&E, original magnification ×50). D, Duodenal metaplasia; note villous architecture and presence of goblet cells (H&E, original magnification ×200).
E14
A. Saad et al.
muscularis propria, characterized by nodular muscular proliferation and disorganized intersection of smooth muscle fascicles (Fig. 3). The associated myenteric plexus also appeared hypertrophic, with a notable increase in autonomic nerve bundles scattered variously throughout the muscle layers (Fig. 4). Interestingly, in addition to ischemic injury to the gastric mucosa, there was evidence of enteric metaplasia, with formation of rudimentary villi and epithelial transition toward a goblet cell–rich columnar phenotype (Fig. 5).
The incidence of feeding intolerance after repair of CDH often necessitates contrast studies to evaluate the differential diagnosis of prepyloric and postpyloric gastric outlet obstruction. We are reporting the first known case of antral stenosis secondary to mucosal, muscular, and neurogenic hypertrophy. The possibility must be considered in the future to avoid delayed diagnosis and misdiagnosis when pyloric stenosis and bands are often considered first.
3. Discussion
References
The etiology of prepyloric stenosis, with mucosal metaplasia, in a patient with CDH is unknown but shows the importance of the milieu and the conditions in which organogenesis occurs in early fetal life. In vitro and in vivo studies have been done and described in the literature, in which human pluripotent stem cells developed into different tissue subtypes depending on the local milieu and microenvironment [7]. The intrauterine location of the stomach in the fetal chest, and the atypical physiologic conditions for upper gastrointestinal development, might have created a new milieu where tissue hyperplasia occurred more proximal than the usual (ie, pylorus) along with the transition point from gastric to duodenal mucosa. This anomalous location of foregut structures in the chest might have disrupted the normal development of the stomach and proximal duodenum from the foregut, a process that usually starts as early as 4 weeks of gestation.
[1] Skari H, Bjornland K, Haugen G, et al. Congenital diaphragmatic hernia: a meta-analysis of mortality factors. J Pediatr Surg 2000;35: 1187-97. [2] Puri P, Gorman F. Lethal nonpulmonary anomalies associated with congenital diaphragmatic hernia: implications for early intrauterine surgery. J Pediatr Surg 1984;19:29-32. [3] Skarsgard ED, Harrison MR. Congenital diaphragmatic hernia: the surgeon's perspective. Pediatr Rev 1999;20:71-8. [4] Al-Salem AH, Grant C, Khwaja S. Infantile hypertrophic pyloric stenosis and congenital diaphragmatic hernia. J Pediatr Surg 1990;25: 607-8. [5] Redman M, Ross DA. Hypertrophic pyloric stenosis following repair of congenital diaphragmatic hernia. J Pediatr Surg 1979;14:607. [6] Abdullah F, Zhang Y, Sciortino C, et al. Congenital diaphragmatic hernia: outcome review of 2,173 surgical repairs in US infants. Pediatr Surg Int 2009;25:1059-64. [7] Moosmann S, Hutter J, Moser C, et al. Milieu-adopted in vitro and in vivo differentiation of mesenchymal tissues derived from different adult human CD34-negative progenitor cell clones. Cells Tissues Organs 2005;179:91-101.