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Apple peel deformity of the small bowel without atresia in a congenital mesenteric defect
Journal of Pediatric Surgery (2013) 48, E9–E11
www.elsevier.com/locate/jpedsurg
Apple peel deformity of the small bowel without atresia in a congenital mesenteric defect Nathaly Llore, Sandra Tomita ⁎ Division of Pediatric Surgery, Department of Surgery, New York University Langone Medical Center, New York NY 10016 Received 9 August 2012; revised 2 October 2012; accepted 3 October 2012
Key words: Apple peel; Mesenteric defect; Internal hernia; Intestinal obstruction
Abstract Congenital mesenteric defects are rare causes of bowel obstruction. Even rarer are mesenteric defects with an apple peel type of deformity, probably described definitively only once previously. We present a case of a 3 year old boy who presented with a septic-like picture of severe metabolic acidosis and lethargy from a bowel obstruction with bowel ischemia. At laparotomy he was found to have bowel infarction due to herniation through a congenital mesenteric defect with an apple peel type of deformity of the bowel without bowel atresia. © 2013 Elsevier Inc. All rights reserved.
Congenital mesenteric defects are rare but known cause of internal hernias and small bowel obstruction in children and adults. We present a case of an interesting form of congenital mesenteric defect in which an apple peel type of deformity of the bowel is present without small bowel atresia.
1. Case A 3 year old male with no past medical history presented to the emergency department with severe lethargy after his parents were unable to awaken him that morning from his sleep. The evening prior he complained of severe abdominal pain after but slept, having two or three episodes of emesis during the night. His parents reported no prior history of abdominal pain, diarrhea, or food intolerance. On initial presentation, he was pale, cyanotic, nonverbal, and minimally responsive to noxious stimuli. He was ⁎ Corresponding author. Tel.: +1 212 263 7391. E-mail address: [email protected] (S. Tomita). 0022-3468/$ – see front matter © 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2012.10.009
tachycardic and febrile. His abdomen was noted to be soft and mildly distended. Laboratory findings were notable for a pH of 7.04 and a lactate of 11.9. After an intraosseous catheter was placed and several crystalloid boluses were infused, he began to rouse and speak. Although he denied abdominal pain, there was now voluntary guarding on abdominal exam and he had two episodes of coffee-ground emesis. An abdominal radiograph showed prominent airfilled loops of bowel suggestive of a bowel obstruction. An ultrasound showed no intussusception. It did show aperistaltic loops of bowel with lack of appreciable Doppler signal within the bowel wall, concerning for ischemia. The patient was taken emergently to the operation room. At diagnostic laparoscopy, the patient was noted to have bloody serous ascites and necrotic, infarcted bowel. Laparotomy was performed and infarcted small bowel was seen herniating through a mesenteric defect in the distal ileum (Fig. 1). The defect was enlarged in order to reduce the hernia (Fig. 2). The herniated bowel was gangrenous and did not return to normal color once reduced. Resection of 240 cm of gangrenous bowel was done from the mid-small bowel to the terminal ileum, leaving the ileocecal valve intact. The
E10
N. Llore, S. Tomita
Fig. 1
Incarcerated transmesenteric hernia.
mesenteric defect was noted to be associated with a marginal vessel and an apple peel type deformity of the distal ileum normally seen with intestinal atresia (Fig. 3). Jejunoileostomy was performed, followed by closure of the mesenteric defect. Postoperatively, he required laparotomy for a mechanical obstruction and revision of the jejunoileostomy. He did well after the second operation and was discharged home 6 days later.
2. Discussion Internal hernias are rare causes of small bowel obstruction and may account of 0.6 to 5.8 of all cases of small bowel obstruction [1]. Most internal hernias occur
Fig. 2
Congenital mesenteric defect after reduction of hernia.
Fig. 3
Apple peel deformity of the small bowel.
postoperatively after incomplete closure of a surgically created mesenteric defect. Internal hernias caused by congenital defects in the mesentery are very rare and represented by a number of case reports and small case series in the literature [2–14]. Most of these reports point to Rokitansky as the first to describe the first congenital transmesenteric hernia in 1836, at a postmortem examination, in which the cecum herniated through a defect in the mesentery near the ileocecal valve. Most of these case reports and series also report the transmesenteric defects located in the distal ileal mesentery as was the case in our patient. Other types of congenital internal hernias which appear distinct from these hernias include paraduodenal hernias which are associated with intestinal rotational disorders, mesocolic hernias, and Treves’ fold hernias which are associated with a pouch [15–18]. The apple peel deformity seen in our case of a congenital mesenteric defect appears to have been clearly reported only once before in the literature. [2] This case report by Hull has no photographs but clearly describes the absent mesentery from the superior mesenteric artery to the ileocolic vessels with a blood supply from a marginal vessel with an “apple peel” or “Christmas tree” deformity normal associated with an atresia but no atresia present. In addition, reports by Tassinari and Sato (a post-mortem exam) appear to show this same anomaly in their reports’ images, although the term apple peel or Christmas tree deformity is not mentioned or described [11,14]. The etiology of congenital mesenteric defects is not known. However, it has been theorized that an in-utero vascular accident involving the superior mesenteric artery can produce a defect in the mesentery. Our case like Hull's with a
Congenital mesenteric defect with apple peel deformity
E11
mesenteric defect and an apple peel deformity would seem to point to a theory that small bowel atresias and congenital transmesenteric hernias may be variants of the same entity. As Hull suggests, the mesenteric defect if associated with a marginal artery, may cause the coiling defect of the apple peel as the bowel outgrows it blood supply. Although most case reports of congenital mesenteric defects appear in toddler age children as was our case, there is a report in a neonates as well as several reports in adults, suggesting that these transmesenteric hernias can present at any time [8–10,12,13]. Most present acutely with obstructive symptoms but can present more insidiously with intermittent abdominal pain [6,14]. Incarceration of herniated loops may progress to strangulation and shock. Patients may have abdominal tenderness and distension. Laboratory tests may show leukocytosis and metabolic acidosis and plain abdominal radiographs may show evidence of a bowel obstruction. Ultrasound may be helpful in diagnosing ischemia. Treatment of these hernias involves reduction of the hernia with closure of the defect and bowel resection in cases of strangulation. The rarity of this entity often contributes to its delay in diagnosis and may lead to death [7,11]. Exploratory laparoscopy/laparotomy is the only definitive means of diagnosis.
[3] Garignon C, Paparel R, Liloku R, et al. Mesenteric hernia: a rare cause of intestinal obstruction in children. J Pediatr Surg 2002;37:1493-4. [4] Cascio S, Sze Loong Tien A, Agarwal P, et al. Dorsal mesenteric agenesis without small bowel atresia: a rare cause of midgut volvulus in children. J Pediatr Surg 2006;41:E5-7. [5] Ming Y-C, Chau JC, Luo C-C. Congenital mesenteric hernia causing intestinal obstruction in children. Eur J Pediatr 2007;166:1045-7. [6] Page M, Ricca R, Resnick A, et al. Newborn and toddler intestinal obstruction owing to congenital mesenteric defects. J Pediatr Surg 2008;43:755-8. [7] Byard R, Wick R. Congenital mesenteric defects and unexpected death —a rare finding at autopsy. Ped Dev Path 2008;11:245-8. [8] Capito C, Podevin J, Lascarrou JB, et al. Large congenital transmesenteric hernia: a missed small-bowel atresia? Hernia 2009;13:209-2011. [9] Gyedu A, Damah M, Baidoo, et al. Congenital transmesenteric defect causing bowel strangulation in an adult. Hernia 2010;14:643-5. [10] Gomes R, Rodrigues J. Spontaneous adult transmesenteric hernia with bowel gangrene. Hernia 2011;15:343-5. [11] Sato T, Abe S, Tsuboi K, et al. Sudden death of a child because of an intestinal obstruction caused by a large congenital mesenteric defect. Leg Med 2012;14:157-9. [12] Hashimoto D, Hirota M, Sakata K, et al. Adult transmesenteric hernia: report of two cases. Surg Today 2012;42:489-92. [13] Malit M, Burjonrappa S. Congenital mesenteric defect: description of a rare cause of distal intestinal obstruction in a neonate. Int J Surg Case Rep 2012;3:121-3. [14] Tassinari D, Santoro S, Bernardi F, et al. A mesenteric hernia complicated with a triple necrotic volvulus. BMJ Case Rep 2012, http://dx.doi.org/10.1136/bcr-2012-006448. [15] Teng B, Yamout SZ. Left paraduodenal hernia causing small bowel obstruction in an adolescent patient. J Pediatr Surg 2009;44:2417-9. [16] Villalona GA, Diefenbach KA, Touloukian RJ. Congenital and acquired mesocolic hernias presented with small bowel obstruction in childhood and adolescence. J Pediatr Surg 2010;45:438-42. [17] Lough JO, Estrada RL, Wiglesworth FW. Internal hernia into Treves’ field pouch report of two cases and review of literature. J Pediatr Surg 1969;4:198-207. [18] Vaos G, Skondras C. Treves’ field congenital hernias in children: an unsuspected rare cause of acute small bowel obstruction. Pediatr Surg Int 2007;23:337-42.
References [1] Newsom B, Kukora J. Congenital and acquired internal hernias: unusual causes of small bowel obstruction. Am J Surg 1986;152: 279-85. [2] Hull J, Kiesel J, Proudfoot W, et al. Agenesis of the dorsal mesentery without jejunoileal atresia (“apple peel small bowel”). J Pediatr Surg 1975;10:277-9.
www.elsevier.com/locate/jpedsurg
Apple peel deformity of the small bowel without atresia in a congenital mesenteric defect Nathaly Llore, Sandra Tomita ⁎ Division of Pediatric Surgery, Department of Surgery, New York University Langone Medical Center, New York NY 10016 Received 9 August 2012; revised 2 October 2012; accepted 3 October 2012
Key words: Apple peel; Mesenteric defect; Internal hernia; Intestinal obstruction
Abstract Congenital mesenteric defects are rare causes of bowel obstruction. Even rarer are mesenteric defects with an apple peel type of deformity, probably described definitively only once previously. We present a case of a 3 year old boy who presented with a septic-like picture of severe metabolic acidosis and lethargy from a bowel obstruction with bowel ischemia. At laparotomy he was found to have bowel infarction due to herniation through a congenital mesenteric defect with an apple peel type of deformity of the bowel without bowel atresia. © 2013 Elsevier Inc. All rights reserved.
Congenital mesenteric defects are rare but known cause of internal hernias and small bowel obstruction in children and adults. We present a case of an interesting form of congenital mesenteric defect in which an apple peel type of deformity of the bowel is present without small bowel atresia.
1. Case A 3 year old male with no past medical history presented to the emergency department with severe lethargy after his parents were unable to awaken him that morning from his sleep. The evening prior he complained of severe abdominal pain after but slept, having two or three episodes of emesis during the night. His parents reported no prior history of abdominal pain, diarrhea, or food intolerance. On initial presentation, he was pale, cyanotic, nonverbal, and minimally responsive to noxious stimuli. He was ⁎ Corresponding author. Tel.: +1 212 263 7391. E-mail address: [email protected] (S. Tomita). 0022-3468/$ – see front matter © 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2012.10.009
tachycardic and febrile. His abdomen was noted to be soft and mildly distended. Laboratory findings were notable for a pH of 7.04 and a lactate of 11.9. After an intraosseous catheter was placed and several crystalloid boluses were infused, he began to rouse and speak. Although he denied abdominal pain, there was now voluntary guarding on abdominal exam and he had two episodes of coffee-ground emesis. An abdominal radiograph showed prominent airfilled loops of bowel suggestive of a bowel obstruction. An ultrasound showed no intussusception. It did show aperistaltic loops of bowel with lack of appreciable Doppler signal within the bowel wall, concerning for ischemia. The patient was taken emergently to the operation room. At diagnostic laparoscopy, the patient was noted to have bloody serous ascites and necrotic, infarcted bowel. Laparotomy was performed and infarcted small bowel was seen herniating through a mesenteric defect in the distal ileum (Fig. 1). The defect was enlarged in order to reduce the hernia (Fig. 2). The herniated bowel was gangrenous and did not return to normal color once reduced. Resection of 240 cm of gangrenous bowel was done from the mid-small bowel to the terminal ileum, leaving the ileocecal valve intact. The
E10
N. Llore, S. Tomita
Fig. 1
Incarcerated transmesenteric hernia.
mesenteric defect was noted to be associated with a marginal vessel and an apple peel type deformity of the distal ileum normally seen with intestinal atresia (Fig. 3). Jejunoileostomy was performed, followed by closure of the mesenteric defect. Postoperatively, he required laparotomy for a mechanical obstruction and revision of the jejunoileostomy. He did well after the second operation and was discharged home 6 days later.
2. Discussion Internal hernias are rare causes of small bowel obstruction and may account of 0.6 to 5.8 of all cases of small bowel obstruction [1]. Most internal hernias occur
Fig. 2
Congenital mesenteric defect after reduction of hernia.
Fig. 3
Apple peel deformity of the small bowel.
postoperatively after incomplete closure of a surgically created mesenteric defect. Internal hernias caused by congenital defects in the mesentery are very rare and represented by a number of case reports and small case series in the literature [2–14]. Most of these reports point to Rokitansky as the first to describe the first congenital transmesenteric hernia in 1836, at a postmortem examination, in which the cecum herniated through a defect in the mesentery near the ileocecal valve. Most of these case reports and series also report the transmesenteric defects located in the distal ileal mesentery as was the case in our patient. Other types of congenital internal hernias which appear distinct from these hernias include paraduodenal hernias which are associated with intestinal rotational disorders, mesocolic hernias, and Treves’ fold hernias which are associated with a pouch [15–18]. The apple peel deformity seen in our case of a congenital mesenteric defect appears to have been clearly reported only once before in the literature. [2] This case report by Hull has no photographs but clearly describes the absent mesentery from the superior mesenteric artery to the ileocolic vessels with a blood supply from a marginal vessel with an “apple peel” or “Christmas tree” deformity normal associated with an atresia but no atresia present. In addition, reports by Tassinari and Sato (a post-mortem exam) appear to show this same anomaly in their reports’ images, although the term apple peel or Christmas tree deformity is not mentioned or described [11,14]. The etiology of congenital mesenteric defects is not known. However, it has been theorized that an in-utero vascular accident involving the superior mesenteric artery can produce a defect in the mesentery. Our case like Hull's with a
Congenital mesenteric defect with apple peel deformity
E11
mesenteric defect and an apple peel deformity would seem to point to a theory that small bowel atresias and congenital transmesenteric hernias may be variants of the same entity. As Hull suggests, the mesenteric defect if associated with a marginal artery, may cause the coiling defect of the apple peel as the bowel outgrows it blood supply. Although most case reports of congenital mesenteric defects appear in toddler age children as was our case, there is a report in a neonates as well as several reports in adults, suggesting that these transmesenteric hernias can present at any time [8–10,12,13]. Most present acutely with obstructive symptoms but can present more insidiously with intermittent abdominal pain [6,14]. Incarceration of herniated loops may progress to strangulation and shock. Patients may have abdominal tenderness and distension. Laboratory tests may show leukocytosis and metabolic acidosis and plain abdominal radiographs may show evidence of a bowel obstruction. Ultrasound may be helpful in diagnosing ischemia. Treatment of these hernias involves reduction of the hernia with closure of the defect and bowel resection in cases of strangulation. The rarity of this entity often contributes to its delay in diagnosis and may lead to death [7,11]. Exploratory laparoscopy/laparotomy is the only definitive means of diagnosis.
[3] Garignon C, Paparel R, Liloku R, et al. Mesenteric hernia: a rare cause of intestinal obstruction in children. J Pediatr Surg 2002;37:1493-4. [4] Cascio S, Sze Loong Tien A, Agarwal P, et al. Dorsal mesenteric agenesis without small bowel atresia: a rare cause of midgut volvulus in children. J Pediatr Surg 2006;41:E5-7. [5] Ming Y-C, Chau JC, Luo C-C. Congenital mesenteric hernia causing intestinal obstruction in children. Eur J Pediatr 2007;166:1045-7. [6] Page M, Ricca R, Resnick A, et al. Newborn and toddler intestinal obstruction owing to congenital mesenteric defects. J Pediatr Surg 2008;43:755-8. [7] Byard R, Wick R. Congenital mesenteric defects and unexpected death —a rare finding at autopsy. Ped Dev Path 2008;11:245-8. [8] Capito C, Podevin J, Lascarrou JB, et al. Large congenital transmesenteric hernia: a missed small-bowel atresia? Hernia 2009;13:209-2011. [9] Gyedu A, Damah M, Baidoo, et al. Congenital transmesenteric defect causing bowel strangulation in an adult. Hernia 2010;14:643-5. [10] Gomes R, Rodrigues J. Spontaneous adult transmesenteric hernia with bowel gangrene. Hernia 2011;15:343-5. [11] Sato T, Abe S, Tsuboi K, et al. Sudden death of a child because of an intestinal obstruction caused by a large congenital mesenteric defect. Leg Med 2012;14:157-9. [12] Hashimoto D, Hirota M, Sakata K, et al. Adult transmesenteric hernia: report of two cases. Surg Today 2012;42:489-92. [13] Malit M, Burjonrappa S. Congenital mesenteric defect: description of a rare cause of distal intestinal obstruction in a neonate. Int J Surg Case Rep 2012;3:121-3. [14] Tassinari D, Santoro S, Bernardi F, et al. A mesenteric hernia complicated with a triple necrotic volvulus. BMJ Case Rep 2012, http://dx.doi.org/10.1136/bcr-2012-006448. [15] Teng B, Yamout SZ. Left paraduodenal hernia causing small bowel obstruction in an adolescent patient. J Pediatr Surg 2009;44:2417-9. [16] Villalona GA, Diefenbach KA, Touloukian RJ. Congenital and acquired mesocolic hernias presented with small bowel obstruction in childhood and adolescence. J Pediatr Surg 2010;45:438-42. [17] Lough JO, Estrada RL, Wiglesworth FW. Internal hernia into Treves’ field pouch report of two cases and review of literature. J Pediatr Surg 1969;4:198-207. [18] Vaos G, Skondras C. Treves’ field congenital hernias in children: an unsuspected rare cause of acute small bowel obstruction. Pediatr Surg Int 2007;23:337-42.
References [1] Newsom B, Kukora J. Congenital and acquired internal hernias: unusual causes of small bowel obstruction. Am J Surg 1986;152: 279-85. [2] Hull J, Kiesel J, Proudfoot W, et al. Agenesis of the dorsal mesentery without jejunoileal atresia (“apple peel small bowel”). J Pediatr Surg 1975;10:277-9.