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Apple-peel intestinal atresia: Enteroplasty for intestinal lengthening and primary anastomosis
Journal of Pediatric Surgery (2013) 48, E5–E7
www.elsevier.com/locate/jpedsurg
Apple-peel intestinal atresia: Enteroplasty for intestinal lengthening and primary anastomosis Luciano Silveira Onofre ⁎, Renato Frota de Albuquerque Maranhão, Elaine Cristina Soares Martins, Camila Girardi Fachin, Jose Luiz Martins Department of Pediatric Surgery, Universidade Federal de São Paulo, Unifesp, Brazil Received 26 November 2012; revised 29 April 2013; accepted 29 April 2013
Key words: Apple-peel intestinal atresia; Intestinal lengthening procedure; Tailoring procedure
Abstract Apple-peel atresia (or Type-IIIb intestinal atresia) is an unusual type of jejunoileal atresia. They present with jejunal atresia near the ligament of Treitz and a foreshortened small bowel. Many surgical options have been used, but the optimal method of repair remains unclear. We present a case of a newborn with apple-peel intestinal atresia managed by enteroplasty for intestinal lengthening and primary anastomosis. © 2013 Elsevier Inc. All rights reserved.
Infants born with jejunoileal atresia have considerable dilatation of the intestine proximal to the obstruction, and the distal segment of intestine is collapsed. When the dilatated proximal intestine is anastomosed to the distal, persistent functional bowel obstruction occurs even though the anastomosis is widely patent [1]. In cases of proximal jejunal atresia with adequate length of intestine, resection of the dilatated atretic segment and end-to-end anastomosis obviates these complications. When there is limitated length of remaining intestine, an antimesenteric reduction-tapering jejunoplasty has proved useful [2]. Nevertheless some cases of intestinal atresia are associated with a loss of a sizeable segment of the small bowel, frequently the case in apple-peel atresia (type-IIIb intestinal atresia). The requisite resection to adjust anastomotic openings and avoid the functional obstruction of the bowel in these patients can compromise the absorptive area of the intestine and increase the incidence of short-bowel syndrome. ⁎ Corresponding author. Rua Baltazar Lisboa, , São Paulo-SP, Brazil CEP 04110–060. Tel.: + 55 11 98209 9938; fax: + 55 11 5908 1665. E-mail address: [email protected] (L.S. Onofre). 0022-3468/$ – see front matter © 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2013.04.024
Intestinal-lengthening procedures are rarely used as the primary correction in these cases. The authors present the correction of apple-peel intestinal atresia through enteroplasty using a flap on the antimesenteric border from the dilated blind proximal segment of intestine for intestinal lengthening and primary anastomosis. This procedure is based on application of the technique initially described by J. Ten Kate to correct long gap esophageal atresia [3]. By using this technique, we simultaneously managed a tailoring procedure that promote intestinal lengthening and primary anastomosis without a significant loss of the absorptive area.
1. Case report A two-day old female newborn with a gestational age of 35 weeks was admitted with a diagnosis compatible with intestinal obstruction. The child's birth weight was 2600 g. The patient underwent a laparotomy, which resulted in a diagnosis of apple-peel intestinal atresia (Fig. 1). The dilated proximal bowel segment from the duodenal junction to the
E6
L.S. Onofre et al.
Fig. 1 Apple-peel intestinal atresia diagnosed in a newborn with intestinal obstruction.
site of atresia measured 30 cm in length and 10 cm in diameter. The distal small bowel segment from the ileocecal valve expressing features of atresia measured approximately 20 cm in length. Due to concern about the length of the intestine, the technique used to correct the defect consisted of creating a flap on the antimesenteric border from the dilated blind proximal segment of intestine. This flap of the proximal intestinal segment measured 10 cm in length and had a base measuring 5 cm in length. The flap was turned down to the lower segment and tabularized employing a funnel-effect that lead to the anastomotic opening with a 1.5 cm diameter. An end-to-end anastomosis was performed from the tubularized proximal segment to the distal intestinal segment, as shown in Fig. 2. This enteroplasty technique enabled to lengthen the dilatated proximal atretic segment by about a third of its initial length, promotes tailoring to avoid functional obstruction, enabling anastomosis with adequate calibers to the distal intestinal segment (Fig. 3). There were no post-operative complications. The patient remained on total parenteral nutrition for a period of 3 months until full enteral feeding was achieved. The patient is now a thriving seven year old, weighs 27 kilograms and measures 1 meter and 26 centimeters, and has 1–2 wellformed bowel movements a day. She is currently in the weight-for-age chart 73,4 percentile and in the body mass index-for-age chart 76,7 percentile, considered eutrophic (WHO child growth standards, reference 2007). No other surgical procedures were required and there were no episodes of bowel obstructions.
Fig. 2 The line of incision in the dilatated blind proximal segment of intestine is marked, the arrow demonstrating the direction the flap will turn. The proximal segment is tabularized with a funnel-effect leading down to the anastomotic opening. Endto-end anastomosis without significant loss of the absorptive area.
intestinal tract without first somehow adjusting the caliber. Despite the segment’s producing vigorous peristalsis, its walls do not adequately coapt, which results in low intraluminal pressure. The resulting peristalsis is incapable of producing an adequate upstream pressure gradient [4].
2. Discussion When correcting intestinal atresia, we should not use the dilated proximal intestinal segment to reconstruct the
Fig. 3 Enteroplasty for intestinal lengthening and primary anastomosis.
Apple-peel intestinal atresia The use of this segment is associated with an increase in a functional bowel obstruction [3]. Many approaches can be taken to resolve this problem. Functional anastomosis is obtained through resection of the dilatated atretic proximal intestinal segment and end-to-end anastomosis; however this technique sacrifices an important segment of the absorptive intestinal surface [1,3,5]. As an alternative, an antimesenteric reduction-tapering jejuonoplasty can be used to perform anastomosis, but this also negatively affects the absorptive area [6]. Tapering antimesenteric reduction jejuonoplasty of the dilated segment ensures effective anastomosis without the loss of intestinal mucosa, but this technique is more appropriate for obstructions and dehiscience in the suture [7]. Apple-peel atresia (or Type-IIIb intestinal atresia) is an unusual type of jejunoileal atresia. Its etiopathogenesis includes the occurrence of mesenteric vascular accident after the emergence of the middle colic artery. They present with jejunal atresia near the ligament of Treitz, foreshortened bowel, and a large mesenteric gap. The bowel distal to the atresia is precariously supplied in a retrograde fashion by anastomotic arcades from the ileocolic, right colic or inferior mesentery artery. Most of these children had less than half of the normal length of the small bowel and had a physiologically short bowel [7]. Many surgical options have been used, but the optimal method of repair remains unclear. The ideal technique would enable intestinal lengthening and primary anastomosis. In 1952, J. Ten Kate described a technique to correct long gap esophageal atresia using a flap from the anterior wall of the esophagus. He showed that it was possible to lengthen the dilated proximal stump and to decrease anastomotic tension [8,9]. In this clinical case, we apply this principle to correct an apple-peel intestinal atresia. The length of the flap we created measured one-third the length of the dilated proximal intestinal segment, and its base measured half the diameter of the dilated segment. It was designed with a funnel-effect leading to the anastomotic opening. The diameter of the distal portion of the flap was made to adjust the anastomotic openings and ensured anastomosis with adequate diameter to the distal intestinal segment. There was no impairment of vascularization of the flap after its confection. However, had there been an ischemic segment of flap, this would have been resected before the tubulization. Serial transverse enteroplasty procedure (STEP) was described in 2003 by Kim et al for intestinal lengthening of patients with short bowel syndrome [10,11]. The procedure also promotes intestinal lengthening and tapering
E7 and is another viable option in the management of intestinal atresia [12,13]. STEP involves the use of a stapler, which may not be affordable for less affluent countries [14]. Serial transverse enteroplasty in intestinal atresia management also requires an extensive proximal dilated intestinal segment [11]. The proposed technique of enteroplasty with a flap from the antimesenteric border is simpler than STEP, particularly when the stapler is simply not available. Furthermore, enteroplasty with a flap from the antimesenteric border can be performed on any length of proximal intestinal segment, which is not the case for STEP. The technique of enteroplasty with a flap from the antimesenteric border to correct apple-peel intestinal atresia guarantees functional anastomosis without significant loss of the absorptive area. It is a simple operation and can be used as an alternative in correcting intestinal atresia where other techniques favor the occurrence of short bowel syndrome.
References [1] De Lorimier AA, Norman DA, Gooding CA, et al. A model for cinefluoroscopic and manometric study of chronic intestinal obstruction. J Pediatr Surg 1973;8:758-91. [2] Grosfeld JL. Jejunoileal atresia and stenosis. In: O´Neill Jr JA, Rowe MI, Grosfeld JL, Fonkalsrud EW, Coran AG, editors. Pediatric surgery. 5 th ed. St Louis: Missouri Mosby-Year Book; 1998. p. 1145-58. [3] Benson CD. Resection and primary anastomosis of the jejunum and ileum in the newborn. Ann Surg 1955;142:478-85. [4] Thomas Jr CD. Jejunoplasty for the correction of jejunal atresia. Sur Gynecol Obstrect 1969;129:545-6. [5] De Lorimier AA, Harrison MR. J Intestinal plication in the treatment of atresia. J Pediatr Surg 1983;18:734-7. [6] Weitzman JJ, Vanderhoof RS. Jejunal atresia with agenesis of the dorsal mesentery with “Christmas tree” deformity of the small intestine. Am J Surg 1966;11:443-9. [7] Waldhausen JHT, Sawain R. Improved long-term outcome for patients with jejunoileal apple peel atresia. J Pediatr Surg 1997;32:1308-9. [8] Kate JT. A method of suturing in operations for congenital oesophageal atresia. Arch Chir Neerl 1952;4:43-7. [9] Gough MH. Esophageal atresia – Use of an anterior flap in the difficult anastomosis. J Pediatr Surg 1980;15:310-1. [10] Kim HB, Fauza D, Garza J. Serial transverse enteroplasty (STEP): a novel bowel lenghtening procedure. J Pediatr Surg 2003;38:425-9. [11] Kim HB, Lee PW, Garza J. Serial transverse enteroplasty for short bowel syndrome: a case report. J Pediatr Surg 2003;38:881-5. [12] Wales PW, Dutta S. Serial transverse enteroplasty for neonates with proximal jejuna atresia. J Pediatr Surg 2005;40:E31-4. [13] Ismael A, Alkadhi A, Alnagaar O, et al. Serial transverse enteroplasty in intestinal atresia management. J Pediatr Surg 2005;40:E5-6. [14] Sheth NP. Serial transverse enteroplasty.J Pediatr Surg 2005;40:1060 [author reply 1060].
www.elsevier.com/locate/jpedsurg
Apple-peel intestinal atresia: Enteroplasty for intestinal lengthening and primary anastomosis Luciano Silveira Onofre ⁎, Renato Frota de Albuquerque Maranhão, Elaine Cristina Soares Martins, Camila Girardi Fachin, Jose Luiz Martins Department of Pediatric Surgery, Universidade Federal de São Paulo, Unifesp, Brazil Received 26 November 2012; revised 29 April 2013; accepted 29 April 2013
Key words: Apple-peel intestinal atresia; Intestinal lengthening procedure; Tailoring procedure
Abstract Apple-peel atresia (or Type-IIIb intestinal atresia) is an unusual type of jejunoileal atresia. They present with jejunal atresia near the ligament of Treitz and a foreshortened small bowel. Many surgical options have been used, but the optimal method of repair remains unclear. We present a case of a newborn with apple-peel intestinal atresia managed by enteroplasty for intestinal lengthening and primary anastomosis. © 2013 Elsevier Inc. All rights reserved.
Infants born with jejunoileal atresia have considerable dilatation of the intestine proximal to the obstruction, and the distal segment of intestine is collapsed. When the dilatated proximal intestine is anastomosed to the distal, persistent functional bowel obstruction occurs even though the anastomosis is widely patent [1]. In cases of proximal jejunal atresia with adequate length of intestine, resection of the dilatated atretic segment and end-to-end anastomosis obviates these complications. When there is limitated length of remaining intestine, an antimesenteric reduction-tapering jejunoplasty has proved useful [2]. Nevertheless some cases of intestinal atresia are associated with a loss of a sizeable segment of the small bowel, frequently the case in apple-peel atresia (type-IIIb intestinal atresia). The requisite resection to adjust anastomotic openings and avoid the functional obstruction of the bowel in these patients can compromise the absorptive area of the intestine and increase the incidence of short-bowel syndrome. ⁎ Corresponding author. Rua Baltazar Lisboa, , São Paulo-SP, Brazil CEP 04110–060. Tel.: + 55 11 98209 9938; fax: + 55 11 5908 1665. E-mail address: [email protected] (L.S. Onofre). 0022-3468/$ – see front matter © 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2013.04.024
Intestinal-lengthening procedures are rarely used as the primary correction in these cases. The authors present the correction of apple-peel intestinal atresia through enteroplasty using a flap on the antimesenteric border from the dilated blind proximal segment of intestine for intestinal lengthening and primary anastomosis. This procedure is based on application of the technique initially described by J. Ten Kate to correct long gap esophageal atresia [3]. By using this technique, we simultaneously managed a tailoring procedure that promote intestinal lengthening and primary anastomosis without a significant loss of the absorptive area.
1. Case report A two-day old female newborn with a gestational age of 35 weeks was admitted with a diagnosis compatible with intestinal obstruction. The child's birth weight was 2600 g. The patient underwent a laparotomy, which resulted in a diagnosis of apple-peel intestinal atresia (Fig. 1). The dilated proximal bowel segment from the duodenal junction to the
E6
L.S. Onofre et al.
Fig. 1 Apple-peel intestinal atresia diagnosed in a newborn with intestinal obstruction.
site of atresia measured 30 cm in length and 10 cm in diameter. The distal small bowel segment from the ileocecal valve expressing features of atresia measured approximately 20 cm in length. Due to concern about the length of the intestine, the technique used to correct the defect consisted of creating a flap on the antimesenteric border from the dilated blind proximal segment of intestine. This flap of the proximal intestinal segment measured 10 cm in length and had a base measuring 5 cm in length. The flap was turned down to the lower segment and tabularized employing a funnel-effect that lead to the anastomotic opening with a 1.5 cm diameter. An end-to-end anastomosis was performed from the tubularized proximal segment to the distal intestinal segment, as shown in Fig. 2. This enteroplasty technique enabled to lengthen the dilatated proximal atretic segment by about a third of its initial length, promotes tailoring to avoid functional obstruction, enabling anastomosis with adequate calibers to the distal intestinal segment (Fig. 3). There were no post-operative complications. The patient remained on total parenteral nutrition for a period of 3 months until full enteral feeding was achieved. The patient is now a thriving seven year old, weighs 27 kilograms and measures 1 meter and 26 centimeters, and has 1–2 wellformed bowel movements a day. She is currently in the weight-for-age chart 73,4 percentile and in the body mass index-for-age chart 76,7 percentile, considered eutrophic (WHO child growth standards, reference 2007). No other surgical procedures were required and there were no episodes of bowel obstructions.
Fig. 2 The line of incision in the dilatated blind proximal segment of intestine is marked, the arrow demonstrating the direction the flap will turn. The proximal segment is tabularized with a funnel-effect leading down to the anastomotic opening. Endto-end anastomosis without significant loss of the absorptive area.
intestinal tract without first somehow adjusting the caliber. Despite the segment’s producing vigorous peristalsis, its walls do not adequately coapt, which results in low intraluminal pressure. The resulting peristalsis is incapable of producing an adequate upstream pressure gradient [4].
2. Discussion When correcting intestinal atresia, we should not use the dilated proximal intestinal segment to reconstruct the
Fig. 3 Enteroplasty for intestinal lengthening and primary anastomosis.
Apple-peel intestinal atresia The use of this segment is associated with an increase in a functional bowel obstruction [3]. Many approaches can be taken to resolve this problem. Functional anastomosis is obtained through resection of the dilatated atretic proximal intestinal segment and end-to-end anastomosis; however this technique sacrifices an important segment of the absorptive intestinal surface [1,3,5]. As an alternative, an antimesenteric reduction-tapering jejuonoplasty can be used to perform anastomosis, but this also negatively affects the absorptive area [6]. Tapering antimesenteric reduction jejuonoplasty of the dilated segment ensures effective anastomosis without the loss of intestinal mucosa, but this technique is more appropriate for obstructions and dehiscience in the suture [7]. Apple-peel atresia (or Type-IIIb intestinal atresia) is an unusual type of jejunoileal atresia. Its etiopathogenesis includes the occurrence of mesenteric vascular accident after the emergence of the middle colic artery. They present with jejunal atresia near the ligament of Treitz, foreshortened bowel, and a large mesenteric gap. The bowel distal to the atresia is precariously supplied in a retrograde fashion by anastomotic arcades from the ileocolic, right colic or inferior mesentery artery. Most of these children had less than half of the normal length of the small bowel and had a physiologically short bowel [7]. Many surgical options have been used, but the optimal method of repair remains unclear. The ideal technique would enable intestinal lengthening and primary anastomosis. In 1952, J. Ten Kate described a technique to correct long gap esophageal atresia using a flap from the anterior wall of the esophagus. He showed that it was possible to lengthen the dilated proximal stump and to decrease anastomotic tension [8,9]. In this clinical case, we apply this principle to correct an apple-peel intestinal atresia. The length of the flap we created measured one-third the length of the dilated proximal intestinal segment, and its base measured half the diameter of the dilated segment. It was designed with a funnel-effect leading to the anastomotic opening. The diameter of the distal portion of the flap was made to adjust the anastomotic openings and ensured anastomosis with adequate diameter to the distal intestinal segment. There was no impairment of vascularization of the flap after its confection. However, had there been an ischemic segment of flap, this would have been resected before the tubulization. Serial transverse enteroplasty procedure (STEP) was described in 2003 by Kim et al for intestinal lengthening of patients with short bowel syndrome [10,11]. The procedure also promotes intestinal lengthening and tapering
E7 and is another viable option in the management of intestinal atresia [12,13]. STEP involves the use of a stapler, which may not be affordable for less affluent countries [14]. Serial transverse enteroplasty in intestinal atresia management also requires an extensive proximal dilated intestinal segment [11]. The proposed technique of enteroplasty with a flap from the antimesenteric border is simpler than STEP, particularly when the stapler is simply not available. Furthermore, enteroplasty with a flap from the antimesenteric border can be performed on any length of proximal intestinal segment, which is not the case for STEP. The technique of enteroplasty with a flap from the antimesenteric border to correct apple-peel intestinal atresia guarantees functional anastomosis without significant loss of the absorptive area. It is a simple operation and can be used as an alternative in correcting intestinal atresia where other techniques favor the occurrence of short bowel syndrome.
References [1] De Lorimier AA, Norman DA, Gooding CA, et al. A model for cinefluoroscopic and manometric study of chronic intestinal obstruction. J Pediatr Surg 1973;8:758-91. [2] Grosfeld JL. Jejunoileal atresia and stenosis. In: O´Neill Jr JA, Rowe MI, Grosfeld JL, Fonkalsrud EW, Coran AG, editors. Pediatric surgery. 5 th ed. St Louis: Missouri Mosby-Year Book; 1998. p. 1145-58. [3] Benson CD. Resection and primary anastomosis of the jejunum and ileum in the newborn. Ann Surg 1955;142:478-85. [4] Thomas Jr CD. Jejunoplasty for the correction of jejunal atresia. Sur Gynecol Obstrect 1969;129:545-6. [5] De Lorimier AA, Harrison MR. J Intestinal plication in the treatment of atresia. J Pediatr Surg 1983;18:734-7. [6] Weitzman JJ, Vanderhoof RS. Jejunal atresia with agenesis of the dorsal mesentery with “Christmas tree” deformity of the small intestine. Am J Surg 1966;11:443-9. [7] Waldhausen JHT, Sawain R. Improved long-term outcome for patients with jejunoileal apple peel atresia. J Pediatr Surg 1997;32:1308-9. [8] Kate JT. A method of suturing in operations for congenital oesophageal atresia. Arch Chir Neerl 1952;4:43-7. [9] Gough MH. Esophageal atresia – Use of an anterior flap in the difficult anastomosis. J Pediatr Surg 1980;15:310-1. [10] Kim HB, Fauza D, Garza J. Serial transverse enteroplasty (STEP): a novel bowel lenghtening procedure. J Pediatr Surg 2003;38:425-9. [11] Kim HB, Lee PW, Garza J. Serial transverse enteroplasty for short bowel syndrome: a case report. J Pediatr Surg 2003;38:881-5. [12] Wales PW, Dutta S. Serial transverse enteroplasty for neonates with proximal jejuna atresia. J Pediatr Surg 2005;40:E31-4. [13] Ismael A, Alkadhi A, Alnagaar O, et al. Serial transverse enteroplasty in intestinal atresia management. J Pediatr Surg 2005;40:E5-6. [14] Sheth NP. Serial transverse enteroplasty.J Pediatr Surg 2005;40:1060 [author reply 1060].