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A Uniquely Abnormal Stool Specimen: A Case Report
The Journal of Emergency Medicine, Vol. -, No. -, pp. e1–e3, 2018 Published by Elsevier Inc. 0736-4679/$ - see front matter
https://doi.org/10.1016/j.jemermed.2018.07.032
Clinical Communications: Pediatric A UNIQUELY ABNORMAL STOOL SPECIMEN: A CASE REPORT Riley Hoyer, MD, Christopher J. Amann, MD, and Andrea L. Austin, MD Emergency Department, Naval Medical Center San Diego, San Diego, California Reprint Address: Riley Hoyer, MD, Naval Medical Center San Diego, 34800 Bob Wilson Dr., San Diego, CA 92134.
, Abstract—Background: Autoamputation of the appendix is a condition associated with the Ladd’s procedure, a pediatric surgical technique for correction of intestinal malrotation. A 4-year-old male patient with a history of a Ladd’s procedure performed as a newborn was brought in by his mother for ‘‘passing intestine’’ just prior to arrival. She reported that for several weeks her son had intermittent, crampy abdominal pain that resolved after the unusual-appearing bowel movement. After reviewing an image of the bowel movement, and in consultation with pediatric surgery, it was concluded that the patient had passed a devascularized appendix in his stool immediately prior to arrival. Case Report: A 4-year-old boy with a past medical history of heterotaxy syndrome (inversion of the thoracoabdominal organs), a double outlet right ventricle, and Ladd’s procedure presented to the Emergency Department (ED) after ‘‘passing intestine’’ in his stool. Close examination of the photo demonstrated a tubular structure with taenia, consistent with an appendix. Why Should an Emergency Physician Be Aware of This?: Autoamputation of the appendix is an uncommon presentation in the ED. Passing a devascularized appendix is a benign condition and can present with weeks of intermittent abdominal pain that resolves with passage of appendix in the stool. Remnants of the appendix can remain within the intestinal lumen years after the Ladd’s procedure. Emergency physicians with a general
awareness of this rare phenomenon can confidently make the diagnosis and reassure worried parents. Published by Elsevier Inc. , Keywords—appendix; Ladd’s procedure; inversion appendectomy; intussusception; pediatrics
INTRODUCTION Delayed passage of an autoamputated appendix in the pediatric population is a rare presenting chief complaint for the emergency department (ED). Its appearance is associated with intermittent abdominal pain and anorexia over several weeks that can clinically mimic other important medical emergencies, including intussusception and bowel obstruction. This phenomenon is a benign etiology of abdominal pain, and reassurance must be provided to family members by astute emergency physicians (EPs) familiar with this rare occurrence. CASE REPORT A 4-year-old boy with a past medical history of heterotaxy syndrome (inversion of the thoraco-abdominal organs), a double outlet right ventricle, and Ladd’s procedure presented to the ED after ‘‘passing intestine.’’ According to the patient’s mother, the child had been complaining of intermittent crampy abdominal pain for several weeks. This was associated with some anorexia, but no fever, chills, vomiting, blood in stool, or diarrhea. The mother reported an episode of pain on
This work was a Poster Presentation at the Society for Academic Emergency Medicine Annual Meeting, 2016. The views expressed in this article are those of the authors and do not reflect the official policy or position of the Department of the Navy, Department of Defense, or the United States Government.
RECEIVED: 10 July 2018; ACCEPTED: 22 July 2018 e1
e2
the morning of presentation, which was relieved by a bowel movement, at which point she observed an abnormal material in the toilet bowl (Figure 1). The mother captured a digital image of the specimen prior to discarding the specimen at home. No sample could be obtained for pathologic review. This child had undergone a Ladd’s procedure for malrotation within the first week of life. He recovered well and had been asymptomatic until a few weeks prior to presentation. Physical examination revealed a well-appearing child in no apparent distress with a soft, nontender abdominal examination without acute peritoneal signs. The sole abnormal finding from the examination was hypoxia, with a peripheral oxygen saturation at 85%, but this was at the child’s baseline, related to his congenital cardiac disease. Given the child’s history of prior abdominal surgery and intermittent crampy pain, an upright abdominal plain film was obtained to evaluate for obstruction, intussusception, or other abnormality. This was normal. Close examination of the photo demonstrated a tubular structure with taenia, consistent with an appendix. The child had undergone a Ladd’s procedure for malrotation more than 4 years prior to his presentation. Due to the child’s surgical history, reported intermittent episodes of abdominal pain, and the unusual-appearing stool photo, the case was discussed with the child’s pediatric surgeon. After further evaluation in the ED by Pediatric Surgery and review of the image, it was concluded that the child had passed a devascularized inverted appendix as a result of inverting the appendix during a Ladd’s procedure as an infant. The family of the patient was reassured that this commonly occurs in this specific patient population
Figure 1. Devascularized appendix; photo provided by patient’s mother.
R. Hoyer et al.
who have undergone the procedure, and the patient was discharged to home with follow-up by Pediatric Surgery. DISCUSSION In a child with heterotaxy, the internal thoraco-abdominal organs are abnormally arranged over the left-right axis of the body (1). These patients often have a variety of complex cardiac and extracardiac abnormalities, including intestinal malrotation. Malrotation, with or without midgut volvulus, is commonly seen among these patients, with reported incidence as high as 70% (2). Surgical correction of intestinal malrotation, at any age, is performed surgically by the Ladd’s procedure (3). The incidence of intestinal malrotation is about 1 in 500 live births and may even be as high as 1% of the total population, according to autopsy studies (4). This operation consists of four steps. First, there is surgical division of Ladd’s bands, which are fibrous stalks of peritoneal tissue that attach the cecum to the abdominal wall. Prior to surgical division, Ladd’s bands can create an obstruction of the duodenum. The following steps involve widening of the small intestine mesentery, performing an appendectomy, and correctional placement of the cecum and colon (5). In this specific case, an inversion appendectomy was performed. Inversion appendectomy, also known as simple inversion of the appendix, was first introduced by George Edebohls in 1895. Edebohls advocated that the procedure be performed during any open abdominal surgery (6,7). The procedure was further advocated by John Lilly in 1968, as inversion appendectomy does not involve opening the lumen of intestinal tract, as opposed to other prophylactic surgical techniques performed, including incidental appendectomies. Bacterial contamination can occur with transection of the appendix in incidental appendectomies, thus increasing the risk of postoperative infection (7). Incidental appendectomies may increase the risk of wound infection and intraperitoneal sepsis (8). By performing inversion appendectomy during sterile open abdominal procedures, postoperative infection rates can be further reduced and the risk of future appendicitis is removed. The lifelong risk of appendicitis requiring appendectomy is roughly 1 in 6 in childhood and 1 in 10 during the teenage years (9). There are reports of late complications of inversion appendectomy. These include intussusception, with inverted appendix acting as a lead point, as well as hemorrhage due to an incompletely devascularized appendix (10,11). Few case reports of intussusception after inversion appendectomy have been reported in the literature (12). However, in a review of long-term outcomes of simple inversion of the appendix in a cohort
A Uniquely Abnormal Stool Specimen
of patients, neither intussusception nor hemorrhage were observed as postoperative complications (13). During this procedure, the appendix is skeletonized from its mesentery and inverted via a blunt probe into the cecal lumen. Then, the remaining part of tissue is ligated and inverted with a purse-string stitch (14). The entire appendix will subsequently autoamputate into the cecum and be passed with a bowel movement. After inversion of the appendix, the appendix will be expelled within the stool in, on average, 9–10 days (15). Retained fragments of a devascularized, inverted appendix have been reported in the medical literature up to 15 years after the original surgery (16). In this specific case, the patient had retained the appendix for 3 years after the Ladd’s procedure. The variability in length of time an inverted appendix remains in the intestinal lumen continues to be unclear, but likely involves multiple factors, including adequacy of devascularization, progression of tissue atrophy, and fibrotic adherence to the intestinal wall. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS? After extensive review of emergency medicine and pediatric surgery literature, this case represents a unique example of an ED visit involving the passage of a devascularized appendix in one’s stool. Key points that are important for EPs include: 1) Inversion appendectomy results in a retained, devascularized appendix within the lumen of the cecum that usually passes with 10 days of the procedure. 2) Patients with history of inversion appendectomy are at increased risk for intussusception. 3) Hemorrhage due to an incompletely devascularized appendix is also a rare complication of the procedure. 4) Remnants of the appendix can remain with intestinal lumen years after the initial procedure. 5) Passing a devascularized appendix is a benign condition and typically presents with weeks of intermittent abdominal pain that resolves with passage of the appendix in the stool.
e3
EPs must consider this rare phenomenon when providing care for patients with a history of Ladd’s procedure for surgical correction of intestinal malrotation. Acknowledgments—We would like to thank the mother of the patient for providing images of the stool specimen with the understanding that the image would be used for publication.
REFERENCES 1. Jacobs JP, Pasquali SK, Morales DL, et al. Heterotaxy: lessons learned about patterns of practice and outcomes from the congenital heart surgery database of the society of thoracic surgeons. World J Pediatr Congenit Heart Surg 2011;2:278–86. 2. Mahalik SK, Khanna S, Menon P. Malrotation and volvulus associated with heterotaxy syndrome. J Indian Assoc Pediatr Surg 2012; 17:138–40. 3. Frasier LL, Leverson G, Gosain A, Greenberg J. Laparoscopic versus open Ladd’s procedure for intestinal malrotation in adults. Surg Endosc 2014;29:1598–604. 4. Durkin ET, Lund DP, Shaaban AF, Schurr MJ, Weber SM. Agerelated differences in diagnosis and morbidity of intestinal malrotation. J Am Coll Surg 2008;206:658–63. 5. Ingoe R, Lange P. The Ladd’s procedure for correction of intestinal malrotation with volvulus in children. AORN J 2007;85:300–8. quiz 309–12. 6. Edebohls GM. Inversion of the vermiform appendix. In: Transactions of the Medical Society of the State of New York. Philadelphia: Medical Society of the State of New York; 1895. 7. Lilly JR, Randolph JG. Total inversion of the appendix: experience with incidental appendectomy in children. J Pediatr Surg 1968;3:357–63. 8. Mulvihill S, Goldthorn J, Woolley MM. Incidental appendectomy in infants and children. Risk v rationale. Arch Surg 1983;118:714–6. 9. Hewitt D, Milner J, Le Riche WH. Incidental appendectomy: a statistical appraisal. Can Med Assoc J 1969;100:1075–81. 10. Arora A, Caniano DA, Hammond S, Besner GE. Inversion appendectomy acting as a lead point for intussusception. Pediatr Surg Int 2008;24:1261–4. 11. Reding R, Clapuyt P, Lengele B, et al. Intestinal hemorrhage three years after incidental appendectomy by total inversion. Eur J Pediatr Surg 1993;3:59–60. 12. Hoehner JC, Kimura K, Soper RT. Intussusception as a complication of inversion appendectomy. Pediatr Sure Int 1995;10:51–3. 13. Almasad JK, Daoud FS. Long-term outcome of simple inversion of the appendix as an alternative to incidental appendectomy. Ann Saudi Med 2008;28:179–82. 14. Voeller GR, Fabian TC. Inversion-ligation appendectomy for incidental appendectomy. Am J Surg 1991;161:483–4. 15. Hallatt JG. Inversion of the entire appendix as an incidental procedure. Am J Obstet Gynecol 1958;75:1043–7. 16. Ja¨rvensivu P, Lehtola J, Karvonen AL, Rinne A, Suramo I. Colonoscopic appearance of the remnant of the appendix after total inversion. Endoscopy 1982;14:66–8.
https://doi.org/10.1016/j.jemermed.2018.07.032
Clinical Communications: Pediatric A UNIQUELY ABNORMAL STOOL SPECIMEN: A CASE REPORT Riley Hoyer, MD, Christopher J. Amann, MD, and Andrea L. Austin, MD Emergency Department, Naval Medical Center San Diego, San Diego, California Reprint Address: Riley Hoyer, MD, Naval Medical Center San Diego, 34800 Bob Wilson Dr., San Diego, CA 92134.
, Abstract—Background: Autoamputation of the appendix is a condition associated with the Ladd’s procedure, a pediatric surgical technique for correction of intestinal malrotation. A 4-year-old male patient with a history of a Ladd’s procedure performed as a newborn was brought in by his mother for ‘‘passing intestine’’ just prior to arrival. She reported that for several weeks her son had intermittent, crampy abdominal pain that resolved after the unusual-appearing bowel movement. After reviewing an image of the bowel movement, and in consultation with pediatric surgery, it was concluded that the patient had passed a devascularized appendix in his stool immediately prior to arrival. Case Report: A 4-year-old boy with a past medical history of heterotaxy syndrome (inversion of the thoracoabdominal organs), a double outlet right ventricle, and Ladd’s procedure presented to the Emergency Department (ED) after ‘‘passing intestine’’ in his stool. Close examination of the photo demonstrated a tubular structure with taenia, consistent with an appendix. Why Should an Emergency Physician Be Aware of This?: Autoamputation of the appendix is an uncommon presentation in the ED. Passing a devascularized appendix is a benign condition and can present with weeks of intermittent abdominal pain that resolves with passage of appendix in the stool. Remnants of the appendix can remain within the intestinal lumen years after the Ladd’s procedure. Emergency physicians with a general
awareness of this rare phenomenon can confidently make the diagnosis and reassure worried parents. Published by Elsevier Inc. , Keywords—appendix; Ladd’s procedure; inversion appendectomy; intussusception; pediatrics
INTRODUCTION Delayed passage of an autoamputated appendix in the pediatric population is a rare presenting chief complaint for the emergency department (ED). Its appearance is associated with intermittent abdominal pain and anorexia over several weeks that can clinically mimic other important medical emergencies, including intussusception and bowel obstruction. This phenomenon is a benign etiology of abdominal pain, and reassurance must be provided to family members by astute emergency physicians (EPs) familiar with this rare occurrence. CASE REPORT A 4-year-old boy with a past medical history of heterotaxy syndrome (inversion of the thoraco-abdominal organs), a double outlet right ventricle, and Ladd’s procedure presented to the ED after ‘‘passing intestine.’’ According to the patient’s mother, the child had been complaining of intermittent crampy abdominal pain for several weeks. This was associated with some anorexia, but no fever, chills, vomiting, blood in stool, or diarrhea. The mother reported an episode of pain on
This work was a Poster Presentation at the Society for Academic Emergency Medicine Annual Meeting, 2016. The views expressed in this article are those of the authors and do not reflect the official policy or position of the Department of the Navy, Department of Defense, or the United States Government.
RECEIVED: 10 July 2018; ACCEPTED: 22 July 2018 e1
e2
the morning of presentation, which was relieved by a bowel movement, at which point she observed an abnormal material in the toilet bowl (Figure 1). The mother captured a digital image of the specimen prior to discarding the specimen at home. No sample could be obtained for pathologic review. This child had undergone a Ladd’s procedure for malrotation within the first week of life. He recovered well and had been asymptomatic until a few weeks prior to presentation. Physical examination revealed a well-appearing child in no apparent distress with a soft, nontender abdominal examination without acute peritoneal signs. The sole abnormal finding from the examination was hypoxia, with a peripheral oxygen saturation at 85%, but this was at the child’s baseline, related to his congenital cardiac disease. Given the child’s history of prior abdominal surgery and intermittent crampy pain, an upright abdominal plain film was obtained to evaluate for obstruction, intussusception, or other abnormality. This was normal. Close examination of the photo demonstrated a tubular structure with taenia, consistent with an appendix. The child had undergone a Ladd’s procedure for malrotation more than 4 years prior to his presentation. Due to the child’s surgical history, reported intermittent episodes of abdominal pain, and the unusual-appearing stool photo, the case was discussed with the child’s pediatric surgeon. After further evaluation in the ED by Pediatric Surgery and review of the image, it was concluded that the child had passed a devascularized inverted appendix as a result of inverting the appendix during a Ladd’s procedure as an infant. The family of the patient was reassured that this commonly occurs in this specific patient population
Figure 1. Devascularized appendix; photo provided by patient’s mother.
R. Hoyer et al.
who have undergone the procedure, and the patient was discharged to home with follow-up by Pediatric Surgery. DISCUSSION In a child with heterotaxy, the internal thoraco-abdominal organs are abnormally arranged over the left-right axis of the body (1). These patients often have a variety of complex cardiac and extracardiac abnormalities, including intestinal malrotation. Malrotation, with or without midgut volvulus, is commonly seen among these patients, with reported incidence as high as 70% (2). Surgical correction of intestinal malrotation, at any age, is performed surgically by the Ladd’s procedure (3). The incidence of intestinal malrotation is about 1 in 500 live births and may even be as high as 1% of the total population, according to autopsy studies (4). This operation consists of four steps. First, there is surgical division of Ladd’s bands, which are fibrous stalks of peritoneal tissue that attach the cecum to the abdominal wall. Prior to surgical division, Ladd’s bands can create an obstruction of the duodenum. The following steps involve widening of the small intestine mesentery, performing an appendectomy, and correctional placement of the cecum and colon (5). In this specific case, an inversion appendectomy was performed. Inversion appendectomy, also known as simple inversion of the appendix, was first introduced by George Edebohls in 1895. Edebohls advocated that the procedure be performed during any open abdominal surgery (6,7). The procedure was further advocated by John Lilly in 1968, as inversion appendectomy does not involve opening the lumen of intestinal tract, as opposed to other prophylactic surgical techniques performed, including incidental appendectomies. Bacterial contamination can occur with transection of the appendix in incidental appendectomies, thus increasing the risk of postoperative infection (7). Incidental appendectomies may increase the risk of wound infection and intraperitoneal sepsis (8). By performing inversion appendectomy during sterile open abdominal procedures, postoperative infection rates can be further reduced and the risk of future appendicitis is removed. The lifelong risk of appendicitis requiring appendectomy is roughly 1 in 6 in childhood and 1 in 10 during the teenage years (9). There are reports of late complications of inversion appendectomy. These include intussusception, with inverted appendix acting as a lead point, as well as hemorrhage due to an incompletely devascularized appendix (10,11). Few case reports of intussusception after inversion appendectomy have been reported in the literature (12). However, in a review of long-term outcomes of simple inversion of the appendix in a cohort
A Uniquely Abnormal Stool Specimen
of patients, neither intussusception nor hemorrhage were observed as postoperative complications (13). During this procedure, the appendix is skeletonized from its mesentery and inverted via a blunt probe into the cecal lumen. Then, the remaining part of tissue is ligated and inverted with a purse-string stitch (14). The entire appendix will subsequently autoamputate into the cecum and be passed with a bowel movement. After inversion of the appendix, the appendix will be expelled within the stool in, on average, 9–10 days (15). Retained fragments of a devascularized, inverted appendix have been reported in the medical literature up to 15 years after the original surgery (16). In this specific case, the patient had retained the appendix for 3 years after the Ladd’s procedure. The variability in length of time an inverted appendix remains in the intestinal lumen continues to be unclear, but likely involves multiple factors, including adequacy of devascularization, progression of tissue atrophy, and fibrotic adherence to the intestinal wall. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS? After extensive review of emergency medicine and pediatric surgery literature, this case represents a unique example of an ED visit involving the passage of a devascularized appendix in one’s stool. Key points that are important for EPs include: 1) Inversion appendectomy results in a retained, devascularized appendix within the lumen of the cecum that usually passes with 10 days of the procedure. 2) Patients with history of inversion appendectomy are at increased risk for intussusception. 3) Hemorrhage due to an incompletely devascularized appendix is also a rare complication of the procedure. 4) Remnants of the appendix can remain with intestinal lumen years after the initial procedure. 5) Passing a devascularized appendix is a benign condition and typically presents with weeks of intermittent abdominal pain that resolves with passage of the appendix in the stool.
e3
EPs must consider this rare phenomenon when providing care for patients with a history of Ladd’s procedure for surgical correction of intestinal malrotation. Acknowledgments—We would like to thank the mother of the patient for providing images of the stool specimen with the understanding that the image would be used for publication.
REFERENCES 1. Jacobs JP, Pasquali SK, Morales DL, et al. Heterotaxy: lessons learned about patterns of practice and outcomes from the congenital heart surgery database of the society of thoracic surgeons. World J Pediatr Congenit Heart Surg 2011;2:278–86. 2. Mahalik SK, Khanna S, Menon P. Malrotation and volvulus associated with heterotaxy syndrome. J Indian Assoc Pediatr Surg 2012; 17:138–40. 3. Frasier LL, Leverson G, Gosain A, Greenberg J. Laparoscopic versus open Ladd’s procedure for intestinal malrotation in adults. Surg Endosc 2014;29:1598–604. 4. Durkin ET, Lund DP, Shaaban AF, Schurr MJ, Weber SM. Agerelated differences in diagnosis and morbidity of intestinal malrotation. J Am Coll Surg 2008;206:658–63. 5. Ingoe R, Lange P. The Ladd’s procedure for correction of intestinal malrotation with volvulus in children. AORN J 2007;85:300–8. quiz 309–12. 6. Edebohls GM. Inversion of the vermiform appendix. In: Transactions of the Medical Society of the State of New York. Philadelphia: Medical Society of the State of New York; 1895. 7. Lilly JR, Randolph JG. Total inversion of the appendix: experience with incidental appendectomy in children. J Pediatr Surg 1968;3:357–63. 8. Mulvihill S, Goldthorn J, Woolley MM. Incidental appendectomy in infants and children. Risk v rationale. Arch Surg 1983;118:714–6. 9. Hewitt D, Milner J, Le Riche WH. Incidental appendectomy: a statistical appraisal. Can Med Assoc J 1969;100:1075–81. 10. Arora A, Caniano DA, Hammond S, Besner GE. Inversion appendectomy acting as a lead point for intussusception. Pediatr Surg Int 2008;24:1261–4. 11. Reding R, Clapuyt P, Lengele B, et al. Intestinal hemorrhage three years after incidental appendectomy by total inversion. Eur J Pediatr Surg 1993;3:59–60. 12. Hoehner JC, Kimura K, Soper RT. Intussusception as a complication of inversion appendectomy. Pediatr Sure Int 1995;10:51–3. 13. Almasad JK, Daoud FS. Long-term outcome of simple inversion of the appendix as an alternative to incidental appendectomy. Ann Saudi Med 2008;28:179–82. 14. Voeller GR, Fabian TC. Inversion-ligation appendectomy for incidental appendectomy. Am J Surg 1991;161:483–4. 15. Hallatt JG. Inversion of the entire appendix as an incidental procedure. Am J Obstet Gynecol 1958;75:1043–7. 16. Ja¨rvensivu P, Lehtola J, Karvonen AL, Rinne A, Suramo I. Colonoscopic appearance of the remnant of the appendix after total inversion. Endoscopy 1982;14:66–8.