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Fetal extraperitoneal rectal perforation presenting after duodenal atresia repair
Journal of Pediatric Surgery (2010) 45, 2447–2449
www.elsevier.com/locate/jpedsurg
Fetal extraperitoneal rectal perforation presenting after duodenal atresia repair Yew-Wei Tan ⁎, Sandeep Motiwale Department of Paediatric Surgery, Queen's Medical Centre, Nottingham University Hospital, Nottingham NG7 2UH, UK Received 9 May 2010; revised 11 August 2010; accepted 30 August 2010
Key words: Fetal extraperitoneal rectal perforation; Duodenal atresia; Diagnosis
Abstract Fetal extraperitoneal rectal perforation (FERP) is a very rare condition, but prompt diagnosis and appropriate treatment produce overall good outcome. We report the first case of FERP known to be associated with duodenal atresia, which only became clinically apparent after duodenal atresia repair and initially presented a diagnostic difficulty. Our case was successfully treated with a defunctioning colostomy and subsequent excision of the pseudocyst cavity. Proximal atresias can mask the presentation of FERP, and this diagnosis should be considered in cases of neonatal perineal and buttock swelling. © 2010 Elsevier Inc. All rights reserved.
Fetal extraperitoneal rectal perforation (FERP) is a terminology first coined by Pitcher et al [1] in 2009. It typically presents in neonates with a gas- or meconium-filled perineal or buttock swelling, leading to a risk of skin rupture, intraperitoneal perforation, and systemic sepsis [1]. On the basis of the 15 reported cases in the literature, it has a good long-term outcome when diagnosed and treated early [1-8]. We present here a case of a newborn known to have duodenal atresia that presented with a concomitant FERP, the latter only became clinically apparent after a duodenoduodenostomy.
1. Case report A 1.76-kg infant girl born by cesarean delivery at 36+6 weeks gestation was admitted to our neonatal unit with an antenatal diagnosis of duodenal atresia. Polyhydramnios and ⁎ Corresponding author. Tel.: +44 7786 960622. E-mail address: [email protected] (Y.-W. Tan). 0022-3468/$ – see front matter © 2010 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2010.08.063
maternal duodenal atresia were noted. At birth, duodenal atresia was confirmed radiologically. A small discolored left labioperineal swelling was also noted, with a normally placed anus and normal female genitalia. This swelling was clinically thought to be of lymphatic or vascular in origin, but ultrasound of the area was inconclusive. She had a duodenoduodenostomy on day 1 of life and passed meconium on day 2. Within 3 days, her left labioperineal swelling rapidly increased in size (Fig. 1). X-ray and computed tomographic scan of pelvis demonstrated an aerocele with air-fluid level and areas of calcification within the swelling (Fig. 2). A contrast enema confirmed the presence of a communication between the rectum and the cavity but not with the spinal canal (Fig. 3). A spinal ultrasound confirmed the presence of calcification within the extradural spinal canal below the thecal sac. Magnetic resonance imaging showed a small locule of air within the extradural spinal canal at the level of S1 but no definite communication with the cyst. The child remained systematically well with no feature of sepsis or meningism. On day 5, decision was taken to perform a divided sigmoid colostomy to divert feces from
2448
Fig. 1
Y.-W. Tan, S. Motiwale
Large left labioperineal swelling on day 5 of life.
the cystic swelling and, potentially, the spinal canal. She made an uneventful recovery, and a subsequent distal stomogram showed no communication between the rectum and the swelling. At 13 weeks of age, she underwent excision of the mass via a posterior sagittal approach. The cyst was found within the left pelvic and buttock area. It extended superiorly toward but outwith the peritoneal fold, inferiorly toward the labia, laterally toward the pelvic brim, medially around the rectum, and posteriorly into the sacrococcygeal space. No obvious communication with the rectum was noted. The posterior aspect of the cyst was adherent to the vertebra, and this was resected. Careful examination did not find any communication between the cyst and the dura mater. Her postoperative course was unremarkable. Histologic examination of the cystic lesion did not show any epithelium-lined wall or colonic tissue therefore confirming that this was a pararectal pseudocyst. In the absence of postnatal iatrogrenic cause (eg, rectal
Fig. 3 Contrast enema showing contrast leak into the sacroperineal space.
examination or per rectum instrumentation), we confirmed the diagnosis of FERP. At 6 months of age, she underwent a further contrast enema to ensure no rectal extravasation before colostomy closure. No recurrence of swelling was noted at 6-month outpatient follow-up.
2. Discussion
Fig. 2 Large aerocele in the left labioperineal area, with areas of calcification within the cavity.
To our knowledge, this is the first report of duodenal atresia that has been associated with FERP. Although FERP can present as late as 5 days after birth, our case had an atypical presentation of FERP and demonstrates the potential challenge in making this diagnosis, especially when there is a proximal gastrointestinal obstruction. The discoloration and mild swelling around the labioperineal area at birth made assessment of this lesion difficult and its significance uncertain. After duodenal atresia repair however, a rapidly enlarging cystic swelling arising from this lesion declared itself as being a significant perineal pathologic condition. Other differential diagnoses included a neuroenteric cyst (because of calcification found on the extradural spinal canal) and a rectal duplication anomaly. A sacrococcygeal teratoma may also be a differential diagnosis in other cases. Contrary to other authors' experience [1,3], our case
FERP after duodenal atresia repair demonstrates that imaging alone may not be sufficient to distinguish between these possibilities, and therefore, the initial management should be directed at minimizing further complications. In the absence of neurologic features and signs of definite spinal involvement on magnetic resonance imaging, we would anticipate that a defunctioning colostomy will be sufficient to achieve diversion to prevent further complication, followed by a formal posterior sagittal exploration on a later date. If the patient is unwell at presentation and neurosepsis is suspected (eg, in neuroenteric fistula), one has to be prepared to also perform a laminectomy and debridement [9]. If peritonitis is the cause of systemic sepsis, peritoneal washout during laparotomy and colostomy formation can be performed. If perineal sepsis is suspected (eg, in FERP), a course of antibiotic after diverting colostomy is expected to treat the infection. We would advocate this safe and logical approach in managing such challenging case. The contemporary understanding is that FERP is a late prenatal event [1]. However, the pathogenesis of this condition remains unclear. The concept of intestinal perforation occurs as a result of distal obstruction is logical; however, like Pitcher et al [1], we could not identify any evidence of distal obstruction, and it seems unlikely that intestinal transit would have generated enough pressure to cause rectal perforation given the duodenal obstruction, even if transient intrauterine rectal/anal obstruction did occur to cause the rectal perforation. Therefore, we are in agreement with Davies et al [2] that the antenatal rectal perforation was more likely a result of a localized ischemic insult after herniation of supralevator rectum through a pelvic floor defect, resembling a Richter's hernia. Fetal extraperitoneal rectal perforation can be a difficult diagnosis, especially when proximal obstruction has oc-
2449 curred, and needs to be considered in all cases of neonatal perineal or buttock swelling.
Acknowledgments The authors would like to thank Mr M Shenoy, consultant pediatric urologist, for being directly involved in the management of this child, and Mr R Lindley, specialist registrar in pediatric surgery, for reviewing the manuscript.
References [1] Pitcher GJ, Davies MR, Bowley DM, et al. Fetal extraperitoneal rectal perforation: a rare neonatal emergency. J Pediatr Surg 2009;44:1405-9. [2] Davies MR, Cywes S, Rode H. Prenatal perforation of the extraperitoneal part of the rectum, associated with a developmental defect of the pelvic floor. Z Kinderchir 1984;39:271-3. [3] Sundararajan L, Patel D, Jawaheer G. Antenatal rectal perforation presenting in the neonate. Pediatr Surg Int 2008;24:601-3. [4] Mitsudo SM, Boley SJ, Rosenzweig MJ, et al. Extraperitoneal pelvic meconium extravasation in a newborn infant. J Pediatr 1983;103: 598-600. [5] Dickinson SJ, Moumgis B. Prenatal volvulus with pseudocyst perforating the perineum. J Pediatr Surg 1966;1:288-91. [6] Delarue A, Garcia-Meric P, Martin C, et al. Antenatal rupture of a diverticular rectal duplication with neonatal perineal fistulisation. Pediatr Surg Int 1998;13:288-9. [7] West DK, Touloukian RJ. Meconium pseudocyst presenting as a buttock mass. J Pediatr Surg 1988;23:864-5. [8] Lockwood C, Ghidini A, Romero R, et al. Fetal bowel perforation simulating sacrococcygeal teratoma. J Ultrasound Med 1988;7:227-9. [9] Darwish B, Stanley TV, Wickremesekera A, et al. Presacral neuroenteric fistula in a newborn presenting with an epidural abscess: case report and review of the literature. Pediatrics 2004;114: 527-31.
www.elsevier.com/locate/jpedsurg
Fetal extraperitoneal rectal perforation presenting after duodenal atresia repair Yew-Wei Tan ⁎, Sandeep Motiwale Department of Paediatric Surgery, Queen's Medical Centre, Nottingham University Hospital, Nottingham NG7 2UH, UK Received 9 May 2010; revised 11 August 2010; accepted 30 August 2010
Key words: Fetal extraperitoneal rectal perforation; Duodenal atresia; Diagnosis
Abstract Fetal extraperitoneal rectal perforation (FERP) is a very rare condition, but prompt diagnosis and appropriate treatment produce overall good outcome. We report the first case of FERP known to be associated with duodenal atresia, which only became clinically apparent after duodenal atresia repair and initially presented a diagnostic difficulty. Our case was successfully treated with a defunctioning colostomy and subsequent excision of the pseudocyst cavity. Proximal atresias can mask the presentation of FERP, and this diagnosis should be considered in cases of neonatal perineal and buttock swelling. © 2010 Elsevier Inc. All rights reserved.
Fetal extraperitoneal rectal perforation (FERP) is a terminology first coined by Pitcher et al [1] in 2009. It typically presents in neonates with a gas- or meconium-filled perineal or buttock swelling, leading to a risk of skin rupture, intraperitoneal perforation, and systemic sepsis [1]. On the basis of the 15 reported cases in the literature, it has a good long-term outcome when diagnosed and treated early [1-8]. We present here a case of a newborn known to have duodenal atresia that presented with a concomitant FERP, the latter only became clinically apparent after a duodenoduodenostomy.
1. Case report A 1.76-kg infant girl born by cesarean delivery at 36+6 weeks gestation was admitted to our neonatal unit with an antenatal diagnosis of duodenal atresia. Polyhydramnios and ⁎ Corresponding author. Tel.: +44 7786 960622. E-mail address: [email protected] (Y.-W. Tan). 0022-3468/$ – see front matter © 2010 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2010.08.063
maternal duodenal atresia were noted. At birth, duodenal atresia was confirmed radiologically. A small discolored left labioperineal swelling was also noted, with a normally placed anus and normal female genitalia. This swelling was clinically thought to be of lymphatic or vascular in origin, but ultrasound of the area was inconclusive. She had a duodenoduodenostomy on day 1 of life and passed meconium on day 2. Within 3 days, her left labioperineal swelling rapidly increased in size (Fig. 1). X-ray and computed tomographic scan of pelvis demonstrated an aerocele with air-fluid level and areas of calcification within the swelling (Fig. 2). A contrast enema confirmed the presence of a communication between the rectum and the cavity but not with the spinal canal (Fig. 3). A spinal ultrasound confirmed the presence of calcification within the extradural spinal canal below the thecal sac. Magnetic resonance imaging showed a small locule of air within the extradural spinal canal at the level of S1 but no definite communication with the cyst. The child remained systematically well with no feature of sepsis or meningism. On day 5, decision was taken to perform a divided sigmoid colostomy to divert feces from
2448
Fig. 1
Y.-W. Tan, S. Motiwale
Large left labioperineal swelling on day 5 of life.
the cystic swelling and, potentially, the spinal canal. She made an uneventful recovery, and a subsequent distal stomogram showed no communication between the rectum and the swelling. At 13 weeks of age, she underwent excision of the mass via a posterior sagittal approach. The cyst was found within the left pelvic and buttock area. It extended superiorly toward but outwith the peritoneal fold, inferiorly toward the labia, laterally toward the pelvic brim, medially around the rectum, and posteriorly into the sacrococcygeal space. No obvious communication with the rectum was noted. The posterior aspect of the cyst was adherent to the vertebra, and this was resected. Careful examination did not find any communication between the cyst and the dura mater. Her postoperative course was unremarkable. Histologic examination of the cystic lesion did not show any epithelium-lined wall or colonic tissue therefore confirming that this was a pararectal pseudocyst. In the absence of postnatal iatrogrenic cause (eg, rectal
Fig. 3 Contrast enema showing contrast leak into the sacroperineal space.
examination or per rectum instrumentation), we confirmed the diagnosis of FERP. At 6 months of age, she underwent a further contrast enema to ensure no rectal extravasation before colostomy closure. No recurrence of swelling was noted at 6-month outpatient follow-up.
2. Discussion
Fig. 2 Large aerocele in the left labioperineal area, with areas of calcification within the cavity.
To our knowledge, this is the first report of duodenal atresia that has been associated with FERP. Although FERP can present as late as 5 days after birth, our case had an atypical presentation of FERP and demonstrates the potential challenge in making this diagnosis, especially when there is a proximal gastrointestinal obstruction. The discoloration and mild swelling around the labioperineal area at birth made assessment of this lesion difficult and its significance uncertain. After duodenal atresia repair however, a rapidly enlarging cystic swelling arising from this lesion declared itself as being a significant perineal pathologic condition. Other differential diagnoses included a neuroenteric cyst (because of calcification found on the extradural spinal canal) and a rectal duplication anomaly. A sacrococcygeal teratoma may also be a differential diagnosis in other cases. Contrary to other authors' experience [1,3], our case
FERP after duodenal atresia repair demonstrates that imaging alone may not be sufficient to distinguish between these possibilities, and therefore, the initial management should be directed at minimizing further complications. In the absence of neurologic features and signs of definite spinal involvement on magnetic resonance imaging, we would anticipate that a defunctioning colostomy will be sufficient to achieve diversion to prevent further complication, followed by a formal posterior sagittal exploration on a later date. If the patient is unwell at presentation and neurosepsis is suspected (eg, in neuroenteric fistula), one has to be prepared to also perform a laminectomy and debridement [9]. If peritonitis is the cause of systemic sepsis, peritoneal washout during laparotomy and colostomy formation can be performed. If perineal sepsis is suspected (eg, in FERP), a course of antibiotic after diverting colostomy is expected to treat the infection. We would advocate this safe and logical approach in managing such challenging case. The contemporary understanding is that FERP is a late prenatal event [1]. However, the pathogenesis of this condition remains unclear. The concept of intestinal perforation occurs as a result of distal obstruction is logical; however, like Pitcher et al [1], we could not identify any evidence of distal obstruction, and it seems unlikely that intestinal transit would have generated enough pressure to cause rectal perforation given the duodenal obstruction, even if transient intrauterine rectal/anal obstruction did occur to cause the rectal perforation. Therefore, we are in agreement with Davies et al [2] that the antenatal rectal perforation was more likely a result of a localized ischemic insult after herniation of supralevator rectum through a pelvic floor defect, resembling a Richter's hernia. Fetal extraperitoneal rectal perforation can be a difficult diagnosis, especially when proximal obstruction has oc-
2449 curred, and needs to be considered in all cases of neonatal perineal or buttock swelling.
Acknowledgments The authors would like to thank Mr M Shenoy, consultant pediatric urologist, for being directly involved in the management of this child, and Mr R Lindley, specialist registrar in pediatric surgery, for reviewing the manuscript.
References [1] Pitcher GJ, Davies MR, Bowley DM, et al. Fetal extraperitoneal rectal perforation: a rare neonatal emergency. J Pediatr Surg 2009;44:1405-9. [2] Davies MR, Cywes S, Rode H. Prenatal perforation of the extraperitoneal part of the rectum, associated with a developmental defect of the pelvic floor. Z Kinderchir 1984;39:271-3. [3] Sundararajan L, Patel D, Jawaheer G. Antenatal rectal perforation presenting in the neonate. Pediatr Surg Int 2008;24:601-3. [4] Mitsudo SM, Boley SJ, Rosenzweig MJ, et al. Extraperitoneal pelvic meconium extravasation in a newborn infant. J Pediatr 1983;103: 598-600. [5] Dickinson SJ, Moumgis B. Prenatal volvulus with pseudocyst perforating the perineum. J Pediatr Surg 1966;1:288-91. [6] Delarue A, Garcia-Meric P, Martin C, et al. Antenatal rupture of a diverticular rectal duplication with neonatal perineal fistulisation. Pediatr Surg Int 1998;13:288-9. [7] West DK, Touloukian RJ. Meconium pseudocyst presenting as a buttock mass. J Pediatr Surg 1988;23:864-5. [8] Lockwood C, Ghidini A, Romero R, et al. Fetal bowel perforation simulating sacrococcygeal teratoma. J Ultrasound Med 1988;7:227-9. [9] Darwish B, Stanley TV, Wickremesekera A, et al. Presacral neuroenteric fistula in a newborn presenting with an epidural abscess: case report and review of the literature. Pediatrics 2004;114: 527-31.