- Email: [email protected]
Acute abdominal pain in a separated conjoined twin
Journal of Pediatric Surgery (2005) 40, E39 – E42
www.elsevier.com/locate/jpedsurg
Acute abdominal pain in a separated conjoined twin Denise B. Klinknera, Gregory J. Beilmanb,* a
Department of Surgery, Medical College of Wisconsin, Milwaukee, WI 53226, USA Department of Surgery, University of Minnesota–Twin Cities, Minneapolis, MN 55455, USA
b
Index words: Conjoined twins; Late complications; Acute abdomen; Parasite; Autosite
Abstract Complications of conjoined twins are typically events surrounding the birth and surgical separation. We report an unusual late-onset complication, a grade IV duodenal disruption of the accessory system in a formerly conjoined twin. Because conjoined twins have unique anatomy based on the junction and division of organs, one must always consider a multitude of causes for common complaints. Basic operative principles apply: the acute abdomen must be explored. Finally, to avoid these complications, removal of all or nearly all parasitic structures must be considered at the initial separation. D 2005 Elsevier Inc. All rights reserved.
Surgical complications related to care of conjoined twins usually occur during the initial attempt at separation and reconstruction. Cywes et al reported the results of caring for 25 sets of conjoined twins, with late complications occurring within 14 months of separation [1]. Another review indicated that late deaths occur up to 10 years later, due predominately to cardiac congenital anomalies [2]. Although multiple cases are reported regarding the birth and surgical separation of conjoined twins, there are few publications on the long-term outcome of surgical separation of a conjoined twin [2,3]. Hence, we report our unique encounter.
1. Case report Our patient is a 48-year-old white man who presented to an outside hospital with flulike symptoms and left lower quadrant (LLQ) pain. Because he was known to have
T Corresponding author. Tel.: +1 612 625 7911; fax: +1 612 626 0439. E-mail address: [email protected] (G.J. Beilman). 0022-3468/$ – see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2005.07.039
unusual anatomy as a previously separated conjoined twin, he was transferred to our facility within 24 hours. Upon admission, he continued to complain of fever, chills, nausea, vomiting, and worsening LLQ pain. He denied any diarrhea, constipation, hematochezia, melena, or urinary symptoms. He had no known drug allergies and was on no medications. The significant medical history included birth as a conjoined twin, with his brother being nonviable and removed at birth. He was known to have a left gallbladder and an accessory spleen. The surgical history included 35 prior abdominal operations, with the last occurring in 1978. At that time, he underwent drainage of multiple abdominal abscesses of no known etiology and elective left appendectomy. The majority of the abdominal surgeries were related to the separation itself, but details were unavailable. In the remote past, he had a left below-theknee amputation for club foot. He has fathered one normal daughter. His physical examination was significant for a temperature of 398C and slight tachycardia but normal blood pressure. The abdominal examination revealed normal bowel sounds, no distension, and no rebound tenderness.
E40
Fig. 1 A, Abdominal CT demonstrating the duplicated liver (white arrow) and cystic mass (white dotted arrow) in the edge of the liver. Abnormal-appearing pancreatic body is noted (dashed arrow). Moreover, a distended gallbladder (white outlined arrow) from the native liver is evident. B, Note the horseshoe kidney (white dashed arrow), enlarged spleen (white arrow), and small bowel wall thickening and inflammation to the patient’s right and posterior to the duplicated liver. The edge of the duplicated liver and its gallbladder are noted (white outlined arrow). C, Inflammatory process in the left midabdomen involving the bowel (white arrow). Note the thickened and inflamed bowel wall. There is also localized free fluid and mesenteric stranding.
D.B. Klinkner, G.J. Beilman LLQ pain and referred LLQ tenderness were present. Also noted were a supernumerary left areola and breast bud in the LLQ. Finally, he had midline and left lateral incisions, well healed. Diagnostic studies revealed normal electrolytes, white blood cell count, liver function tests, and urinalysis. Abdominal computed tomography (CT) demonstrated a localized fluid collection in the area of the localized pain, as well as aberrant anatomy, including a horseshoe kidney, accessory spleen, and liver (Fig. 1A, B, and C). He was placed on a broad-spectrum intravenous antibiotic but developed worsening LLQ abdominal pain, continued fevers, and peritoneal signs. He was taken to the operating room for exploratory laparotomy. Upon exploration, the patient was found to have an accessory system on the left comprised of a liver, gallbladder, stomach, duodenum, jejunum, and pancreas (Fig. 2A). This system communicated with the native gastrointestinal system via a natural bifurcation of the jejunum. Also discovered was a grade IV duodenal disruption in the accessory system which was caused by an obstruction at a distal fibrous ring lesion in the accessory jejunum. Finally, the left gallbladder had palpable gallstones. The operative procedures included right and left cholecystectomies, left duodenectomy with resection of the stricture, left gastrojejunostomy, and left choledochojejunostomy (Fig. 2B). Intraoperative upper gastrointestinal endoscopy revealed no visible communication between the 2 systems other than the bifurcated jejunum. The pathology revealed mesenteritis and peritonitis of the small bowel and chronic calculous and acalculous cholecystitis of the left and right gallbladders, respectively. The postoperative course was smooth, with removal of the nasogastric tube on postoperative day (POD) 4, regular diet on POD 5, and drain removal and discharge to home on POD 7. On follow-up 6 weeks postoperative, the patient reported no abdominal pain and an improving appetite and diet. The patient’s long-term course was complicated by poor emptying of his duplicate stomach, becoming symptomatic 1 year after the surgery. After preoperative localization of vascular structures, the patient underwent elective resection of the duplicate stomach, small bowel, and liver without perioperative complication. He is currently asymptomatic and employed at his usual occupation 4 years after the initial operation described previously.
2. Discussion Conjoined twins occur from 1 in 50,000 to 1 in 100,000 births, with a female to male ratio of 3:1 [2,4]. They are monozygotic twins, sharing homologous sites, same sex, and same parentage [5]. Survival is bleak, with 40% of conjoined twins being stillborn and an additional 35%
Acute abdominal pain in a separated conjoined twin
Fig. 2 A, Diagrammatic depiction of the patient’s anatomy. The accessory system is noted on the patient’s left: liver, gallbladder, stomach, pancreas, duodenum, and jejunum. The systems communicated through a natural bifurcation of the jejunum. The accessory system had its own blood supply, largely undefined. Pathology included a grade IV (100%) duodenal disruption on the left, a distal fibrous stenosis in the accessory jejunum, gallstones in the left gallbladder, and distention of the right gallbladder. B, The operative procedure. Right and left cholecystectomies, left duodenectomy with removal of the stricture, left gastrojejunostomy, and left choledochojejunostomy.
dying within the first 24 hours [4]. Environment and genetics are implicated in the etiology, but not definitely linked [4]. Classification is according to the site of junction: craniopagus (Greek for helmet joining), cephalopagus (head), thoracopagus (chest), omphalopagus (umbilicus), parapagus (side), rachipagus (spine), pygopagus (rump), and ischiopagus (hip) [5,6]. In addition, there is a range of
E41 anomalies or degree of development of the twins. Twins may be minimally attached, or they may consist of one nearly normal infant with a poorly developed twin, otherwise known as an autosite with a parasite [5,6]. In cases of parasites, the accessory tissue is analyzed for vital structures before removal [1,2,7,8]. The technology available today may have directed the original surgeons to proceed with complete resection of the extraneous structures and thus avoidance of this outcome. As noted in previous cases [1,2,7,8,11], resection should be undertaken in an elective fashion, unless the status of one twin severely affects the outcome of the other. However, the circumstances of his initial operations are unknown. Surgical issues usually surround the question of separation or correction of various anomalies, such as imperforate anus. In several series and case reports, the preoperative assessment and planning with a multidisciplinary approach were emphasized [1,2,4,8,9]. The surgical care of these patients after separation exacts the same careful attention to anatomy. Our patient required multiple operations without the benefit of previous operative reports. We attempted to define the anatomy preoperatively with an abdominal CT, but the clinical deterioration mandated an exploration without definitive information. The timing of the final operation allowed the necessary workup, which has led to a satisfactory outcome. Given the rarity of conjoined twins, encounters such as this are unique to the patient population. From the patient’s history and physical examination, we deduced that he had been an omphalopagus twin, with his brother most likely a parasitic twin: the second twin’s structures deriving its blood supply from the more normal twin, our patient. In this case, the structures for the parasitic twin had not been completely removed, and the accessory system was responsible for the pathology found. We could not have predicted the operative findings based on the diagnostic studies or even the abdominal CT. Once the pathology was discovered, a variety of surgical options were considered. We could have removed all extraneous organs and thus avoid the risk of further complications with the accessory system. However, the second set of organs appeared to have a well-established, but largely undefined, vascular system. The patient had tolerated the presence of the organs before the obstruction, and removal of them would subject him to a longer procedure and significant blood loss. In the setting of the acute abdomen, a procedure of this undertaking would be inappropriate. Thus, we chose to essentially perform a Whipple procedure on the accessory system, providing an outlet for drainage of the left liver. In an effort to avoid future acute abdominal surgeries, we removed both gallbladders and ensured that the appendix had also been removed. The procedure originally performed allowed the patient to proceed with elective resection of the duplicate liver, stomach, and small bowel with preoperative workup to define the vascular system. Resection in this setting avoided
E42 the complications of operating in the acute abdomen and the potential for significant blood loss from liver resection. The care of conjoined twins inevitably includes surgical, medical, and ethical issues. The conflict surrounding omphalopagus twins Jodie and Mary in the United Kingdom readdresses the issues of care: attempting to maintain the best interests of the children and respect for the wishes of the family [10]. Is separation always necessary? Each case must be considered individually [11]. In our patient, the issues of separation were long ago debated. However, other questions are raised. One may argue that the accessory organs should be transplanted into another patient, because the second set may only cause complications later and benefit another. On the other hand, using his organs would then be exploiting his unique nature. Ultimately, care of the patient must always be in his or her best interest. Long-term follow-up and care of conjoined twins depend on the specific reconstruction after the initial separation. Separated conjoined twins may have a normal life expectancy, as our patient demonstrates. Finally, the lessons learned in caring for this patient are that, first, a typical complaint may have either an unusual presentation or cause, and second, for the pediatric surgeon caring for parasitic twins, one must consider the removal of all or nearly all of the parasitic structures. However, after separation, ultimately, principles of surgical management do not change.
D.B. Klinkner, G.J. Beilman
Acknowledgments The authors thank Jeffery Wu, MD, and Allison S. Veser for editorial advice and Jerry Vincent for his technical skills in preparing this manuscript.
References [1] Cywes S, Millar AJ, Rode H, et al. Conjoined twins—the Cape Town experience. Pediatr Surg Int 1997;12:234 - 48. [2] O’Neill Jr J, Holcomb III GW, Schnaufer L, et al. Surgical experience with thirteen conjoined twins. Ann Surg 1988;208:299 - 312. [3] Hoyle RM, Thomas CG. Twenty-three–year follow-up of separated ischiopagus tetrapus conjoined twins. Ann Surg 1989;210:673 - 9. [4] Wittich AC. Conjoined twins: report of a case and review of literature. J Am Osteopath Assoc 1989;89:1175 - 9. [5] Spencer R. Theoretical and analytical embryology of conjoined twins: part I: embryology. Clin Anat 2000;13:36 - 53. [6] Spencer R. Anatomic description of conjoined twins: a plea for standardized terminology. J Pediatr Surg 1996;31:941 - 4. [7] Cury EK, Schraibman V. Epigastric heteropagus twinning. J Pediatr Surg 2001;36:1 - 3. [8] Kato T, Yoshino H, Hebiguchi T, et al. Experience with treatment of three pairs of conjoined twins. Am J Perinatol 1997;14:25 - 30. [9] Hilfiker ML, Hart M, Holmes R, et al. Expansion and division of conjoined twins. J Pediatr Surg 1998;33:768 - 70. [10] Separation of conjoined twins [editorial]. Lancet 2000;356:953. [11] Raffensperger J. A philosophical approach to conjoined twins. Pediatr Surg Int 1997;12:249 - 55.
www.elsevier.com/locate/jpedsurg
Acute abdominal pain in a separated conjoined twin Denise B. Klinknera, Gregory J. Beilmanb,* a
Department of Surgery, Medical College of Wisconsin, Milwaukee, WI 53226, USA Department of Surgery, University of Minnesota–Twin Cities, Minneapolis, MN 55455, USA
b
Index words: Conjoined twins; Late complications; Acute abdomen; Parasite; Autosite
Abstract Complications of conjoined twins are typically events surrounding the birth and surgical separation. We report an unusual late-onset complication, a grade IV duodenal disruption of the accessory system in a formerly conjoined twin. Because conjoined twins have unique anatomy based on the junction and division of organs, one must always consider a multitude of causes for common complaints. Basic operative principles apply: the acute abdomen must be explored. Finally, to avoid these complications, removal of all or nearly all parasitic structures must be considered at the initial separation. D 2005 Elsevier Inc. All rights reserved.
Surgical complications related to care of conjoined twins usually occur during the initial attempt at separation and reconstruction. Cywes et al reported the results of caring for 25 sets of conjoined twins, with late complications occurring within 14 months of separation [1]. Another review indicated that late deaths occur up to 10 years later, due predominately to cardiac congenital anomalies [2]. Although multiple cases are reported regarding the birth and surgical separation of conjoined twins, there are few publications on the long-term outcome of surgical separation of a conjoined twin [2,3]. Hence, we report our unique encounter.
1. Case report Our patient is a 48-year-old white man who presented to an outside hospital with flulike symptoms and left lower quadrant (LLQ) pain. Because he was known to have
T Corresponding author. Tel.: +1 612 625 7911; fax: +1 612 626 0439. E-mail address: [email protected] (G.J. Beilman). 0022-3468/$ – see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2005.07.039
unusual anatomy as a previously separated conjoined twin, he was transferred to our facility within 24 hours. Upon admission, he continued to complain of fever, chills, nausea, vomiting, and worsening LLQ pain. He denied any diarrhea, constipation, hematochezia, melena, or urinary symptoms. He had no known drug allergies and was on no medications. The significant medical history included birth as a conjoined twin, with his brother being nonviable and removed at birth. He was known to have a left gallbladder and an accessory spleen. The surgical history included 35 prior abdominal operations, with the last occurring in 1978. At that time, he underwent drainage of multiple abdominal abscesses of no known etiology and elective left appendectomy. The majority of the abdominal surgeries were related to the separation itself, but details were unavailable. In the remote past, he had a left below-theknee amputation for club foot. He has fathered one normal daughter. His physical examination was significant for a temperature of 398C and slight tachycardia but normal blood pressure. The abdominal examination revealed normal bowel sounds, no distension, and no rebound tenderness.
E40
Fig. 1 A, Abdominal CT demonstrating the duplicated liver (white arrow) and cystic mass (white dotted arrow) in the edge of the liver. Abnormal-appearing pancreatic body is noted (dashed arrow). Moreover, a distended gallbladder (white outlined arrow) from the native liver is evident. B, Note the horseshoe kidney (white dashed arrow), enlarged spleen (white arrow), and small bowel wall thickening and inflammation to the patient’s right and posterior to the duplicated liver. The edge of the duplicated liver and its gallbladder are noted (white outlined arrow). C, Inflammatory process in the left midabdomen involving the bowel (white arrow). Note the thickened and inflamed bowel wall. There is also localized free fluid and mesenteric stranding.
D.B. Klinkner, G.J. Beilman LLQ pain and referred LLQ tenderness were present. Also noted were a supernumerary left areola and breast bud in the LLQ. Finally, he had midline and left lateral incisions, well healed. Diagnostic studies revealed normal electrolytes, white blood cell count, liver function tests, and urinalysis. Abdominal computed tomography (CT) demonstrated a localized fluid collection in the area of the localized pain, as well as aberrant anatomy, including a horseshoe kidney, accessory spleen, and liver (Fig. 1A, B, and C). He was placed on a broad-spectrum intravenous antibiotic but developed worsening LLQ abdominal pain, continued fevers, and peritoneal signs. He was taken to the operating room for exploratory laparotomy. Upon exploration, the patient was found to have an accessory system on the left comprised of a liver, gallbladder, stomach, duodenum, jejunum, and pancreas (Fig. 2A). This system communicated with the native gastrointestinal system via a natural bifurcation of the jejunum. Also discovered was a grade IV duodenal disruption in the accessory system which was caused by an obstruction at a distal fibrous ring lesion in the accessory jejunum. Finally, the left gallbladder had palpable gallstones. The operative procedures included right and left cholecystectomies, left duodenectomy with resection of the stricture, left gastrojejunostomy, and left choledochojejunostomy (Fig. 2B). Intraoperative upper gastrointestinal endoscopy revealed no visible communication between the 2 systems other than the bifurcated jejunum. The pathology revealed mesenteritis and peritonitis of the small bowel and chronic calculous and acalculous cholecystitis of the left and right gallbladders, respectively. The postoperative course was smooth, with removal of the nasogastric tube on postoperative day (POD) 4, regular diet on POD 5, and drain removal and discharge to home on POD 7. On follow-up 6 weeks postoperative, the patient reported no abdominal pain and an improving appetite and diet. The patient’s long-term course was complicated by poor emptying of his duplicate stomach, becoming symptomatic 1 year after the surgery. After preoperative localization of vascular structures, the patient underwent elective resection of the duplicate stomach, small bowel, and liver without perioperative complication. He is currently asymptomatic and employed at his usual occupation 4 years after the initial operation described previously.
2. Discussion Conjoined twins occur from 1 in 50,000 to 1 in 100,000 births, with a female to male ratio of 3:1 [2,4]. They are monozygotic twins, sharing homologous sites, same sex, and same parentage [5]. Survival is bleak, with 40% of conjoined twins being stillborn and an additional 35%
Acute abdominal pain in a separated conjoined twin
Fig. 2 A, Diagrammatic depiction of the patient’s anatomy. The accessory system is noted on the patient’s left: liver, gallbladder, stomach, pancreas, duodenum, and jejunum. The systems communicated through a natural bifurcation of the jejunum. The accessory system had its own blood supply, largely undefined. Pathology included a grade IV (100%) duodenal disruption on the left, a distal fibrous stenosis in the accessory jejunum, gallstones in the left gallbladder, and distention of the right gallbladder. B, The operative procedure. Right and left cholecystectomies, left duodenectomy with removal of the stricture, left gastrojejunostomy, and left choledochojejunostomy.
dying within the first 24 hours [4]. Environment and genetics are implicated in the etiology, but not definitely linked [4]. Classification is according to the site of junction: craniopagus (Greek for helmet joining), cephalopagus (head), thoracopagus (chest), omphalopagus (umbilicus), parapagus (side), rachipagus (spine), pygopagus (rump), and ischiopagus (hip) [5,6]. In addition, there is a range of
E41 anomalies or degree of development of the twins. Twins may be minimally attached, or they may consist of one nearly normal infant with a poorly developed twin, otherwise known as an autosite with a parasite [5,6]. In cases of parasites, the accessory tissue is analyzed for vital structures before removal [1,2,7,8]. The technology available today may have directed the original surgeons to proceed with complete resection of the extraneous structures and thus avoidance of this outcome. As noted in previous cases [1,2,7,8,11], resection should be undertaken in an elective fashion, unless the status of one twin severely affects the outcome of the other. However, the circumstances of his initial operations are unknown. Surgical issues usually surround the question of separation or correction of various anomalies, such as imperforate anus. In several series and case reports, the preoperative assessment and planning with a multidisciplinary approach were emphasized [1,2,4,8,9]. The surgical care of these patients after separation exacts the same careful attention to anatomy. Our patient required multiple operations without the benefit of previous operative reports. We attempted to define the anatomy preoperatively with an abdominal CT, but the clinical deterioration mandated an exploration without definitive information. The timing of the final operation allowed the necessary workup, which has led to a satisfactory outcome. Given the rarity of conjoined twins, encounters such as this are unique to the patient population. From the patient’s history and physical examination, we deduced that he had been an omphalopagus twin, with his brother most likely a parasitic twin: the second twin’s structures deriving its blood supply from the more normal twin, our patient. In this case, the structures for the parasitic twin had not been completely removed, and the accessory system was responsible for the pathology found. We could not have predicted the operative findings based on the diagnostic studies or even the abdominal CT. Once the pathology was discovered, a variety of surgical options were considered. We could have removed all extraneous organs and thus avoid the risk of further complications with the accessory system. However, the second set of organs appeared to have a well-established, but largely undefined, vascular system. The patient had tolerated the presence of the organs before the obstruction, and removal of them would subject him to a longer procedure and significant blood loss. In the setting of the acute abdomen, a procedure of this undertaking would be inappropriate. Thus, we chose to essentially perform a Whipple procedure on the accessory system, providing an outlet for drainage of the left liver. In an effort to avoid future acute abdominal surgeries, we removed both gallbladders and ensured that the appendix had also been removed. The procedure originally performed allowed the patient to proceed with elective resection of the duplicate liver, stomach, and small bowel with preoperative workup to define the vascular system. Resection in this setting avoided
E42 the complications of operating in the acute abdomen and the potential for significant blood loss from liver resection. The care of conjoined twins inevitably includes surgical, medical, and ethical issues. The conflict surrounding omphalopagus twins Jodie and Mary in the United Kingdom readdresses the issues of care: attempting to maintain the best interests of the children and respect for the wishes of the family [10]. Is separation always necessary? Each case must be considered individually [11]. In our patient, the issues of separation were long ago debated. However, other questions are raised. One may argue that the accessory organs should be transplanted into another patient, because the second set may only cause complications later and benefit another. On the other hand, using his organs would then be exploiting his unique nature. Ultimately, care of the patient must always be in his or her best interest. Long-term follow-up and care of conjoined twins depend on the specific reconstruction after the initial separation. Separated conjoined twins may have a normal life expectancy, as our patient demonstrates. Finally, the lessons learned in caring for this patient are that, first, a typical complaint may have either an unusual presentation or cause, and second, for the pediatric surgeon caring for parasitic twins, one must consider the removal of all or nearly all of the parasitic structures. However, after separation, ultimately, principles of surgical management do not change.
D.B. Klinkner, G.J. Beilman
Acknowledgments The authors thank Jeffery Wu, MD, and Allison S. Veser for editorial advice and Jerry Vincent for his technical skills in preparing this manuscript.
References [1] Cywes S, Millar AJ, Rode H, et al. Conjoined twins—the Cape Town experience. Pediatr Surg Int 1997;12:234 - 48. [2] O’Neill Jr J, Holcomb III GW, Schnaufer L, et al. Surgical experience with thirteen conjoined twins. Ann Surg 1988;208:299 - 312. [3] Hoyle RM, Thomas CG. Twenty-three–year follow-up of separated ischiopagus tetrapus conjoined twins. Ann Surg 1989;210:673 - 9. [4] Wittich AC. Conjoined twins: report of a case and review of literature. J Am Osteopath Assoc 1989;89:1175 - 9. [5] Spencer R. Theoretical and analytical embryology of conjoined twins: part I: embryology. Clin Anat 2000;13:36 - 53. [6] Spencer R. Anatomic description of conjoined twins: a plea for standardized terminology. J Pediatr Surg 1996;31:941 - 4. [7] Cury EK, Schraibman V. Epigastric heteropagus twinning. J Pediatr Surg 2001;36:1 - 3. [8] Kato T, Yoshino H, Hebiguchi T, et al. Experience with treatment of three pairs of conjoined twins. Am J Perinatol 1997;14:25 - 30. [9] Hilfiker ML, Hart M, Holmes R, et al. Expansion and division of conjoined twins. J Pediatr Surg 1998;33:768 - 70. [10] Separation of conjoined twins [editorial]. Lancet 2000;356:953. [11] Raffensperger J. A philosophical approach to conjoined twins. Pediatr Surg Int 1997;12:249 - 55.