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Repair of giant omphalocele by component separation technique
Journal of Pediatric Surgery Case Reports 42 (2019) 32–33
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.elsevier.com/locate/epsc
Repair of giant omphalocele by component separation technique a,∗
Mutua Irene , Swaleh Shahbal a b
T
b
Kampala International University, Uganda Department of Surgery, College of Health Sciences, University of Nairobi, Kenya
A R T I C LE I N FO
A B S T R A C T
Keywords: Omphalocele Component separation technique Escharotic therapy
Giant omphalocele management has always been a challenge because of the large fascial defect and the associated anomalies. We managed successfully a neonate with giant omphalocele and no associated anomalies by delayed repair constituting escharotic therapy initially and later managed the ventral hernia by component separation technique before her preschool years. Her recovery was non-eventful with no recurrence of the hernia on follow up.
1. Introduction An omphalocele is a congenital midline ventral abdominal wall defect with herniation of abdominal viscera into a membrane covered sac consisting of an inner peritoneal layer and an outer layer of amnion [1]. Giant omphalocele is thus characterized by a large opening (diameter of ≥ 5 cm) with herniated abdominal organs including the liver, loss of abdominal cavity volume and other associated congenital anomalies [1,2]. Component separation technique involves the mobilization of the abdominal rectus muscle and fascia with the preservation of innervation for functional reconstruction of the abdominal wall [3]. 2. Case presentation Our case was a female term neonate born via vaginal delivery at 39/ 40 with an Apgar score of 91, 105, 1010 and a birth weight of 3.0 kg. She was born at a peripheral hospital and was later referred to Kenyatta National Hospital having being diagnosed with a congenital abdominal wall defect recognized at birth. She was breastfeeding normally and passed meconium 8 h after birth. She was the first-born child. On examination, the child was alert. Per abdomen, anterior abdominal wall defect was seen at the umbilicus measuring 5 cm in diameter. The small intestines and the liver was visualized via the amnion. A diagnosis of a giant omphalocele was made. Other systems were normal. An echocardiogram done was normal. Child was managed conservatively with daily silver sulfadiazine cream application to allow for granulation tissue to form. Once formed, she was discharged home and follow up done at our Pediatric Surgical
∗
Outpatient Clinic. She was readmitted to hospital when 2 years 6 months old, weighing 14 kgs for secondary closure of abdominal wall defect measuring 5 cm in diameter and 15 cm in length by component separation technique (CST). Intraoperatively, a midline incision was made and skin flaps below the subcutaneous tissue were developed laterally up to the midclavicular line. Adhesiolysis done and the liver mobilized by dividing the falciform ligament. Two relaxing longitudinal incisions on the anterior rectus sheath and external oblique aponeurosis respectively were made bilaterally and one longitudinal incision made on the posterior rectus sheath to gain a total length of 6 cm. This was adequate to achieve medialization without tension. Apposition to the midline was achieved without tension with absorbable Vicryl No 1. suture in a continuous manner. A Portovac Suction drain was placed on the anterior rectus sheath and skin closure done with Nylon 3.0 suture in an interrupted manner. Antibiotics and analgesics were given post operatively. Total drain output was 150 mls. The drain was removed on day 5 postop after being inactive for more than 24 hrs. Her recovery was uneventful and she was discharged on day 6 postop. Follow up at our clinic shows no recurrence of the ventral hernia. 3. Discussion Treatment options for infants with omphalocele depends on size of the defect, presence of associated anomalies, gestational age and if the sac has ruptured or is intact. Anomalies include lung hypoplasia, pulmonary hypertension, congenital heart defects and chromosomal aberrations.1Primary one stage repair, staged closure or delayed repair
Corresponding author. University of Nairobi, P.O Box 19676- 00202, Nairobi, Kenya. E-mail address: [email protected] (M. Irene).
https://doi.org/10.1016/j.epsc.2018.12.015 Received 3 October 2018; Received in revised form 12 December 2018; Accepted 13 December 2018 Available online 19 December 2018 2213-5766/ © 2018 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
Journal of Pediatric Surgery Case Reports 42 (2019) 32–33
M. Irene, S. Shahbal
However, delayed repair by escharotic therapy initially and later component separation technique before the preschool years is feasible with good outcomes.
is done based on the above factors after stabilization of the patient. In our case, the patient had a giant omphalocele with a fascial defect of 5 cm in diameter and the sac was intact. She did not have any associated anomaly. Delayed closure was thus opted due to the loss of abdominal domain. Escharotic therapy which results in gradual epithelialization and granulation of the omphalocele sac over several months was done [4]. In a study by Kouame et al. found that the average duration for achieving complete epithelialization using topical application of 2% disodium aqueous eosin was 70 ± 7 days [5]. Early surgery for giant omphalocele have a risk of increased intra-abdominal pressure and direct trauma to viscera especially the liver and small intestines [4]. We allowed the child to grow up to the age of 30 months to avoid these complications during component separation technique. A ventral hernia formed thereafter and we repaired it by component separation technique (CST). CST as described by Ramirez et al. is done as a means of closure of large abdominal wall defects by separation of the abdominal muscle wall components to allow mobilization of each unit over a greater distance achieving medialization [6]. Other operative management include the use of prosthetic materials such as synthetic meshes whose disadvantage include increased risk of infection, adhesion to the abdominal viscera and reduced intensity and elasticity which might cause abdominal wall hernia [3]. Biological meshes derived from human, bovine or porcine tissue have been used either as an overlay, underlay or sandwich technique to reinforce fascial closure [7,8]. The mesh integrates into the host tissue by causing an inflammatory response initially then it is infiltrated by cellular and vascular components and finally matrix remodeling [7]. This method was used successfully by Shauna Levy et al. to repair large abdominal wall defects in children [9]. In our set-up however, the cost of biological mesh was high and we managed to achieve medialization without tension. Mean age of pediatric patients treated using CST repair without reinforcement using a mesh was 13.3 ± 18.5months with a range of 6 days–69 months in the study by Yoshiaki et al. [3] while the median age in another study by Floortje et al. [10] was 6.5 months with a range of 5months–69 months. Our patient was 30 months at the time of repair. Timing of repair is important before the individual's body image begins to develop from the preschool age of 5 years. Complications of CST include seroma, hematoma, wound infection and skin necrosis [3,10]. Our patient had no complications post-operatively and no recurrence of ventral hernia on follow up.
Patient consent Consent to publish the case was not obtained. This report does not contain any personal information that could lead to the identification of the patient. Funding No funding or grant support. Authorship All authors attest that they meet the current ICMJE criteria for Authorship. Conflict of interest The following authors have no financial disclosures: (MI, SS.) References [1] Alexander JM, Bjarte R. Giant omphalocele: current perspectives. Res Rep Neonatol 2016;6:33–9. [2] Kumar HR, Jester AL, Ladd AP. Impact of omphalocele size on associated conditions. J Pediatr Surg 2008;43(12):2216–9. [3] Yoshiaki T, Kouji N, Kina M, Yokihiro T, Yoshirou M, Toshiharu M, et al. A new abdominal wall reconstruction strategy for giant omphalocele. J Pediatr Surg Case Rep 2018(31):90–4. [4] Emily RCL, Cassandra MK, Jacob CL. Neonatal abdominal wall defects. Semin Fetal Neonatal Med 2011(16):164–72. [5] Kouame DB, Odehouri- Koudou TH, Sounkere M. Techniques and results of the conservative treatment of giant omphalocele with 2% disodium Aqueous eosin. Clin Mother Child Health 2013;11:157. [6] Ramirez OM, Ruas E, Dellon AL. “Components separation” method for closure of abdominal- wall defects: an anatomic and clinical study. Plast Reconstr Surg 1990;86(3):519–26. [7] James FF, Anjali SK. Biologic versus synthetic mesh reinforcement: what are the pros and cons? Clin Colon Rectal Surg 2014;27(4):140–8. [8] Hufford T, Tremblay JF, Mustafa Sheikh MT, et al. Local parastomal hernia repair with biological mesh is safe and effective. Am J Surg 2018;215(1):88–90. [9] Shauna L, Kuojen T, Charles S, et al. Component separation for complex congenital abdominal wall defects: not just for adults anymore. J Pediatr Surg 2013;48(12):2525–9. [10] Floortje CVE, Ivo DB, Robert PB, et al. Closure of giant omphaloceles by the abdominal wall component separation technique in infants. J Pediatr Surg 2008(43):246–50.
4. Conclusion Giant omphalocele management has always been a challenge.
33
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.elsevier.com/locate/epsc
Repair of giant omphalocele by component separation technique a,∗
Mutua Irene , Swaleh Shahbal a b
T
b
Kampala International University, Uganda Department of Surgery, College of Health Sciences, University of Nairobi, Kenya
A R T I C LE I N FO
A B S T R A C T
Keywords: Omphalocele Component separation technique Escharotic therapy
Giant omphalocele management has always been a challenge because of the large fascial defect and the associated anomalies. We managed successfully a neonate with giant omphalocele and no associated anomalies by delayed repair constituting escharotic therapy initially and later managed the ventral hernia by component separation technique before her preschool years. Her recovery was non-eventful with no recurrence of the hernia on follow up.
1. Introduction An omphalocele is a congenital midline ventral abdominal wall defect with herniation of abdominal viscera into a membrane covered sac consisting of an inner peritoneal layer and an outer layer of amnion [1]. Giant omphalocele is thus characterized by a large opening (diameter of ≥ 5 cm) with herniated abdominal organs including the liver, loss of abdominal cavity volume and other associated congenital anomalies [1,2]. Component separation technique involves the mobilization of the abdominal rectus muscle and fascia with the preservation of innervation for functional reconstruction of the abdominal wall [3]. 2. Case presentation Our case was a female term neonate born via vaginal delivery at 39/ 40 with an Apgar score of 91, 105, 1010 and a birth weight of 3.0 kg. She was born at a peripheral hospital and was later referred to Kenyatta National Hospital having being diagnosed with a congenital abdominal wall defect recognized at birth. She was breastfeeding normally and passed meconium 8 h after birth. She was the first-born child. On examination, the child was alert. Per abdomen, anterior abdominal wall defect was seen at the umbilicus measuring 5 cm in diameter. The small intestines and the liver was visualized via the amnion. A diagnosis of a giant omphalocele was made. Other systems were normal. An echocardiogram done was normal. Child was managed conservatively with daily silver sulfadiazine cream application to allow for granulation tissue to form. Once formed, she was discharged home and follow up done at our Pediatric Surgical
∗
Outpatient Clinic. She was readmitted to hospital when 2 years 6 months old, weighing 14 kgs for secondary closure of abdominal wall defect measuring 5 cm in diameter and 15 cm in length by component separation technique (CST). Intraoperatively, a midline incision was made and skin flaps below the subcutaneous tissue were developed laterally up to the midclavicular line. Adhesiolysis done and the liver mobilized by dividing the falciform ligament. Two relaxing longitudinal incisions on the anterior rectus sheath and external oblique aponeurosis respectively were made bilaterally and one longitudinal incision made on the posterior rectus sheath to gain a total length of 6 cm. This was adequate to achieve medialization without tension. Apposition to the midline was achieved without tension with absorbable Vicryl No 1. suture in a continuous manner. A Portovac Suction drain was placed on the anterior rectus sheath and skin closure done with Nylon 3.0 suture in an interrupted manner. Antibiotics and analgesics were given post operatively. Total drain output was 150 mls. The drain was removed on day 5 postop after being inactive for more than 24 hrs. Her recovery was uneventful and she was discharged on day 6 postop. Follow up at our clinic shows no recurrence of the ventral hernia. 3. Discussion Treatment options for infants with omphalocele depends on size of the defect, presence of associated anomalies, gestational age and if the sac has ruptured or is intact. Anomalies include lung hypoplasia, pulmonary hypertension, congenital heart defects and chromosomal aberrations.1Primary one stage repair, staged closure or delayed repair
Corresponding author. University of Nairobi, P.O Box 19676- 00202, Nairobi, Kenya. E-mail address: [email protected] (M. Irene).
https://doi.org/10.1016/j.epsc.2018.12.015 Received 3 October 2018; Received in revised form 12 December 2018; Accepted 13 December 2018 Available online 19 December 2018 2213-5766/ © 2018 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
Journal of Pediatric Surgery Case Reports 42 (2019) 32–33
M. Irene, S. Shahbal
However, delayed repair by escharotic therapy initially and later component separation technique before the preschool years is feasible with good outcomes.
is done based on the above factors after stabilization of the patient. In our case, the patient had a giant omphalocele with a fascial defect of 5 cm in diameter and the sac was intact. She did not have any associated anomaly. Delayed closure was thus opted due to the loss of abdominal domain. Escharotic therapy which results in gradual epithelialization and granulation of the omphalocele sac over several months was done [4]. In a study by Kouame et al. found that the average duration for achieving complete epithelialization using topical application of 2% disodium aqueous eosin was 70 ± 7 days [5]. Early surgery for giant omphalocele have a risk of increased intra-abdominal pressure and direct trauma to viscera especially the liver and small intestines [4]. We allowed the child to grow up to the age of 30 months to avoid these complications during component separation technique. A ventral hernia formed thereafter and we repaired it by component separation technique (CST). CST as described by Ramirez et al. is done as a means of closure of large abdominal wall defects by separation of the abdominal muscle wall components to allow mobilization of each unit over a greater distance achieving medialization [6]. Other operative management include the use of prosthetic materials such as synthetic meshes whose disadvantage include increased risk of infection, adhesion to the abdominal viscera and reduced intensity and elasticity which might cause abdominal wall hernia [3]. Biological meshes derived from human, bovine or porcine tissue have been used either as an overlay, underlay or sandwich technique to reinforce fascial closure [7,8]. The mesh integrates into the host tissue by causing an inflammatory response initially then it is infiltrated by cellular and vascular components and finally matrix remodeling [7]. This method was used successfully by Shauna Levy et al. to repair large abdominal wall defects in children [9]. In our set-up however, the cost of biological mesh was high and we managed to achieve medialization without tension. Mean age of pediatric patients treated using CST repair without reinforcement using a mesh was 13.3 ± 18.5months with a range of 6 days–69 months in the study by Yoshiaki et al. [3] while the median age in another study by Floortje et al. [10] was 6.5 months with a range of 5months–69 months. Our patient was 30 months at the time of repair. Timing of repair is important before the individual's body image begins to develop from the preschool age of 5 years. Complications of CST include seroma, hematoma, wound infection and skin necrosis [3,10]. Our patient had no complications post-operatively and no recurrence of ventral hernia on follow up.
Patient consent Consent to publish the case was not obtained. This report does not contain any personal information that could lead to the identification of the patient. Funding No funding or grant support. Authorship All authors attest that they meet the current ICMJE criteria for Authorship. Conflict of interest The following authors have no financial disclosures: (MI, SS.) References [1] Alexander JM, Bjarte R. Giant omphalocele: current perspectives. Res Rep Neonatol 2016;6:33–9. [2] Kumar HR, Jester AL, Ladd AP. Impact of omphalocele size on associated conditions. J Pediatr Surg 2008;43(12):2216–9. [3] Yoshiaki T, Kouji N, Kina M, Yokihiro T, Yoshirou M, Toshiharu M, et al. A new abdominal wall reconstruction strategy for giant omphalocele. J Pediatr Surg Case Rep 2018(31):90–4. [4] Emily RCL, Cassandra MK, Jacob CL. Neonatal abdominal wall defects. Semin Fetal Neonatal Med 2011(16):164–72. [5] Kouame DB, Odehouri- Koudou TH, Sounkere M. Techniques and results of the conservative treatment of giant omphalocele with 2% disodium Aqueous eosin. Clin Mother Child Health 2013;11:157. [6] Ramirez OM, Ruas E, Dellon AL. “Components separation” method for closure of abdominal- wall defects: an anatomic and clinical study. Plast Reconstr Surg 1990;86(3):519–26. [7] James FF, Anjali SK. Biologic versus synthetic mesh reinforcement: what are the pros and cons? Clin Colon Rectal Surg 2014;27(4):140–8. [8] Hufford T, Tremblay JF, Mustafa Sheikh MT, et al. Local parastomal hernia repair with biological mesh is safe and effective. Am J Surg 2018;215(1):88–90. [9] Shauna L, Kuojen T, Charles S, et al. Component separation for complex congenital abdominal wall defects: not just for adults anymore. J Pediatr Surg 2013;48(12):2525–9. [10] Floortje CVE, Ivo DB, Robert PB, et al. Closure of giant omphaloceles by the abdominal wall component separation technique in infants. J Pediatr Surg 2008(43):246–50.
4. Conclusion Giant omphalocele management has always been a challenge.
33