- Email: [email protected]
Pyloric atresia: Comorbidity determines outcome
J Ped Surg Case Reports 2 (2014) 192e195
Contents lists available at ScienceDirect
Journal of Pediatric Surgery CASE REPORTS journal homepage: www.jpscasereports.com
Pyloric atresia: Comorbidity determines outcomeq Linda M. Schutzman a, Meggen Walsh b, John M. Draus Jr. c, * a
College of Medicine, University of Kentucky, Lexington, KY 40536, USA Department of Pathology, University of Kentucky, Lexington, KY 40536, USA c Department of Surgery, Division of Pediatric Surgery, University of Kentucky, 800 Rose Street, MN-102 Chandler Medical Center, Lexington, KY 40536, USA b
a r t i c l e i n f o
a b s t r a c t
Article history: Received 31 December 2013 Received in revised form 20 March 2014 Accepted 20 March 2014 Available online 13 April 2014
Pyloric atresia is a congenital form of gastric outlet obstruction. The obstruction is relieved surgically, but survival is determined by the presence of additional anomalies. Herein, we report two cases of pyloric atresia. Our first patient was a neonate with epidermolysis bullosa who died of sepsis. The second was an infant who presented with postprandial emesis and had an uncomplicated postoperative course. We discuss the current understanding and management of this rare malformation. Ó 2014 The Authors. Published by Elsevier Inc. All rights reserved.
Key words: Pyloric atresia Gastric outlet obstruction Epidermolysis bullosa
Pyloric atresias are uncommon gastrointestinal defects that represent less than 1% of all bowel atresias. Traditionally, pyloric atresias have been classified by their anatomic morphology. Type 1 atresias are characterized by the presence of a mucosal web between the stomach and duodenum. Type 2 atresias contain a solid longitudinal segment at the pylorus with an intact mesentery. Type 3 atresias are gap aplasias with a disruption between the blind-ending segments of the stomach and duodenum. The surgical correction of pyloric atresia results in excellent long-term function with few complications when it is an isolated defect. Unfortunately, more than half of these children are born with concomitant anomaliesemost commonly epidermolysis bullosa and other intestinal atresias. Such confounding disorders drastically alter the morbidity and mortality associated with this diagnosis. 1. Case report 1 A 1290 g girl was delivered by cesarean section at 33 weeks gestation. The pregnancy had been complicated by polyhydramnios, premature prolonged rupture of the membranes, and late decelerations. Admission radiographs showed a large gastric
q This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/3.0/). * Corresponding author. Tel.: þ1 859 323 5625; fax: þ1 859 323 5289. E-mail addresses: [email protected], [email protected] (J.M. Draus).
bubble and absent intestinal gas which was concerning for pyloric atresia (Fig. 1). The baby was found to have fragile skin, especially at areas of friction, which quickly developed into tense vesicles with scattered areas of erosions, bullae, and crusts (Fig. 2A). Early management included placement of an orogastric tube and initiation of parenteral nutrition. A punch biopsy was diagnostic for junctional epidermolysis bullosa (Fig. 2B). Laparotomy was performed at 1 month of age. We identified a type II pyloric atresia with a 1 cm segment of solid pylorus and an intact mesentery (Fig. 3). A pyloric resection and gastroduodenostomy was performed. Orogastric decompression was discontinued on postoperative day 17. Breast milk feeds were initiated on postoperative day 23 but were soon stopped because the infant developed malabsorption and suspected septicemia. A septic workup was initiated, and prophylactic antibiotics were given. Enteral nutrition was restarted on multiple occasions and always held because of similar consequences. Parental nutrition was continued for a suspected protein-losing enteropathy. Our patient developed intermittent fevers between 38 C and 41 C, and several courses of prophylactic antibiotics were administered. Septic workups never identified an organism. Stool cultures, Clostridium difficile assays, and stool leukocytes remained negative. Eventually, she was intubated for a respiratory distress syndrome, and her general condition continued to deteriorate despite maximum respiratory and nutritional support. On postoperative day 66, the medical staff held a care conference with the girl’s family. A do-not-resuscitate order was obtained, and the baby died at 15 weeks of age.
2213-5766/$ e see front matter Ó 2014 The Authors. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.epsc.2014.03.014
L.M. Schutzman et al. / J Ped Surg Case Reports 2 (2014) 192e195
193
Fig. 3. Intraoperative photograph showing a type II pyloric atresia. Fig. 1. Abdominal radiograph reveals a large gastric bubble with a paucity of intestinal gas.
2. Case report 2 A 9-month-old boy presented to the emergency room with a several week history of non-bilious emesis after most feedings. The boy’s pediatrician had previously started him on ranitidine for the empiric treatment of gastroesophageal reflux. In the emergency room (ER), an acute abdominal series showed a normal bowel gas pattern with the incidental finding of an opaque foreign body in the left upper quadrant. He tolerated an oral fluid challenge, was discharged home, and instructed to follow-up with his pediatrician. The next week, he returned to the ER with continued emesis. Our pediatric gastroenterologist performed a flexible endoscopy. A zipper slider and pull tab was removed from the child’s stomach (Fig. 4A). The pyloric aperture was found to be quite small, and the
neonatal scope could not be advanced through the pylorus (Fig. 4B). Further workup included an abdominal ultrasound to rule out pyloric stenosis and an upper gastrointestinal series which revealed slow passage of contrast out of the stomach (Fig. 5). A pediatric surgical consultation was obtained, and the child was taken to the operating room for exploration. We identified a type 1 pyloric atresia with small perforation within the pyloric web. The web was resected, and a Heineke-Mikulicz pyloroplasty was performed. Oral feedings were resumed on the fourth postoperative day and advanced without complication. 3. Discussion Pyloric atresias are rare, but well-documented forms of gastric outlet obstruction. The exact etiology of this anomaly remains unknown. An autosomal recessive inheritance pattern is displayed in those with a familial predisposition for pyloric atresia [1]. The
Fig. 2. A) Photograph illustrating the skin lesions of our patient with epidermolysis bullosa. B) Electron micrograph of our patient’s skin biopsy. Note the widened dermal/epidermal space. There are areas of lamina densa present; however, the lamina lucida is missing multifocally.
194
L.M. Schutzman et al. / J Ped Surg Case Reports 2 (2014) 192e195
Fig. 4. A) Endoscopic image showing a gastric foreign body. B) Endoscopic image showing a type I pyloric atresia with a small opening.
atresias may be the consequence of developmental arrest or a vascular insult which results in a failure of pyloric recanalization between the 5th and 12th weeks of gestation. Some speculate that the fibrous membrane forms in response to mechanical or chemical irritation [1e3]. Approximately 30%e55% of children with pyloric atresia are found to have other congenital anomaliesemost commonly epidermolysis bullosa or additional intestinal atresias [4]. Epidermolysis bullosa is a genetic disorder that effectively increases collagenase activity resulting in collagen splitting at various locations within the epidermis or dermis. It is classified according to the specific level of involvement: epidermolysis bullosa simplex involves the epidermis; junctional epidermolysis bullosa involves the lamina lucida of the basement membrane; and dystrophic epidermolysis bullosa involves the lamina densa of the basement membrane. Clinically, epidermolysis bullosa is characterized by skin blistering and denudation after the occurrence of seemingly minor trauma.
Fig. 5. Upper gastrointestinal series shows delayed passage of gastric contrast.
Prenatal diagnosis allows for early counseling and improved perinatal management. Ultrasound findings suggesting pyloric atresia include polyhydramnios and gastric dilatation in the absence of the double bubble characteristic of duodenal atresia [3,4]. Due to the significant impact on morbidity and mortality, epidermolysis bullosa must be considered in any fetus suspected of having pyloric atresia. Epidermolysis bullosa can be diagnosed by genetic testing for familial cases, fetal skin biopsy when feasible, or by the presence of characteristic radiologic findings [1,4e6]. Fetal ultrasound findings associated with epidermolysis bullosa include echogenic debris throughout the amniotic fluid, marked narrowing of external orifices, areas of skin blistering or thinning, and malformation of the external ear [4,6]. Fetal MRI may show gastric dilatation, dependent layering of skin debris and bilateral ear defects [4]. Pyloric atresia should be considered in any newborn who presents with clinical manifestations of intestinal obstruction and nonbilious vomiting. As seen in our first case, the diagnosis is confirmed with an abdominal radiograph which shows a single gastric bubble and a paucity of intestinal air. As illustrated by our second case, the diagnosis becomes more challenging when children have a type 1 atresia with a perforated web. These children usually present with feeding intolerance, emesis, and other nonspecific symptoms. Frequently, there is a delay in diagnosis and treatment. Ultrasound and endoscopic exploration may be useful [2]. Children born with epidermolysis bullosa and pyloric atresia face a complicated clinical course, often resulting in death from electrolyte imbalance, protein loss, and septicemia [2,7]. The combination of pyloric atresia and epidermolysis bullosa was traditionally regarded as a subtype of the junctional variant. Recent molecular advancements have elucidated over 70 mutations that result in defective hemidesmosome attachment suggesting that this affiliation is actually a discrete disorder [4,8,9]. Due to variable penetration, children with this disorder express a spectrum of disease severity. There are several reports of long-term survivors, endorsing the aggressive management of this patient population. Current efforts are searching a link between genotype and phenotypic expression which may better predict outcomes [5]. The definitive management of pyloric atresia requires surgical correction of the anatomic blockage. In general, surgery should be delayed until fluid losses and metabolic derangements from the gastric outlet obstruction have been corrected. Durable intravenous access should be obtained, and parenteral nutrition is often necessary. The excessive losses of gastric secretions may result in a
L.M. Schutzman et al. / J Ped Surg Case Reports 2 (2014) 192e195
hypokalemic, hypochloremic metabolic alkalosis which requires aggressive fluid resuscitation and appropriate electrolyte replacement. Early gastric decompression by means of an orogastric or nasogastric sump tube should be initiated to decrease the risk of aspiration and subsequent respiratory complication. The surgical management of pyloric atresias is dictated by the anatomic subtype of atresia identified. Type I atresias are treated with either a Heineke-Mikulicz or Finney pyloroplasty. Type II and type III atresias require excision of the solid pyloric segment with end-to-end gastroduodenostomy. The surgeon must evaluate the remaining bowel for additional atresias. If other intestinal atresias are identified, they typically are repaired during the same operation using standard techniques. Recently, several nonconventional techniques have been described. Type I atresias have been opened with balloon dilation or the utilization of laser technology for either web excision or radical membrane incision [10]. Methods involving pylorus-preserving techniques and pyloric reconstruction and have been described for type II and III atresias. Yokoyama et al. recently presented a surgical alternative in which the atretic pylorus was incised longitudinally and the end-to-end anastomosis was placed anterior to the preserved pylorus [10]. Dessanti et al. has described a method involving the anatomic reconstruction of a pyloric sphincter [11]. These methods have been tried on a few patients in hopes of preventing the alkaline gastritis commonly associated with traditional end-to-end gastroduodenostomy. More data are needed to evaluate their safety and long-term efficacy. The management of pyloric atresia in a patient with epidermolysis bullosa presents significant perioperative challenges for the medical team. Severe metabolic disturbances and septic complications may arise secondarily to the excessive denudation of the skin [1,2,12]. Dedicated attention to electrolyte replacement and nutritional support are essential. A recent report of a successful laparoscopic pyloroplasty presents an attractive alternative for the epidermolysis bullosa patient who is already burdened by the metabolic demands of the disorder [13]. 4. Conclusion Pyloric atresia is a serious congenital gastric outlet obstruction. When found alone, pyloric atresias have favorable outcomes.
195
However, the clinical course is drastically altered by the coexistence of epidermolysis bullosa. We have described two patients who received care at our institution. Furthermore, we have discussed the current knowledge regarding etiology, classification, diagnosis and treatment of this rare anomaly. Conflict of interest statement The authors of the case report entitled “Pyloric Atresia: Comorbidity Determines Outcome” have no conflicts of interest to report. References [1] Al-Salem AH. Congenital pyloric atresia and associated anomalies. Pediatr Surg Int 2007;23:559e63. [2] Ilce Z, Erdogan E, Kara C, Celayir S, Sarimurat N, Senyuz OF, et al. Pyloric atresia: 15 year review from a single institution. J Pediatr Surg 2003;38: 1581e4. [3] Andriessen MJG, Matthyssens LE, Heij HA. Pyloric atresia. J Pediatr Surg 2010; 45:2470e2. [4] Merrow AC, Frischer JS, Lucky AW. Pyloric atresia with epidermolysis bullosa: fetal MRI diagnosis with postnatal correlation. Pediatr Radiol 2013;43: 1656e61. [5] Sicot BDJ, Derueele P, Kacet N, Vaillant C, Subtil D. Prenatal findings in epidermolysis bullosa with pyloric atresia in a family not known to be at risk. Ultrasound Obstet Gynecol 2005;25:607e9. [6] Maurice P, Eyrolle-Guignot D, Dhombres F, Carel C, Gonzales M, Muller F, et al. The key role of ultrasound examination in the prenatal diagnosis of epidermolysis bullosa with pyloric atresia. Prenat Diagn 2013;33:908e9. [7] Samad L, Siddiqui EF, Arain MA, Atif M, Parkash J, Ahmed S. Pyloric atresia associated with epidermolysis bullosa - three cases presenting in three months. J Pediatr Surg 2004;39:1267e9. [8] Schumann H, Kiritsi D, Pigors M, Kahlhas J, Peters J, Ott H, et al. Phenotypic spectrum of epidermolysis bullosa associated with a6b4 integrin mutations. Br J Dermatol 2013;169:115e24. [9] Fu AC, Hon KL, Choi PC. A neonate with generalized bullae and pyloric atresia. Hong Kong Med J 2013;19(188):e1e2. [10] Yokoyama S, Utsunomiya H. A case of successful surgical repair for solid segment type pyloric atresia using a novel gastroduodenostomy procedure. J Pediatr Surg 2012;47:2158e60. [11] Dessanti A, Di Benetto V, Iannuccelli M, Cossu Rocca P, Di Benedetto A. Pyloric atresia: a new operation to reconstruct the pyloric sphincter. J Pediatr Surg 2004;39:297e301. [12] Dank JP, Kim S, Parisi MA, Brown T, Smith LT, Waldhousen J, et al. Outcome after surgical repair of junctional epidermolysis bullosa-pyloric atresia syndrome: a report of 3 cases and review of the literature. Arch Dermatol 1999; 135:1243e7. [13] Son TN, Hoan VX. Laparoscopic management of pyloric atresia in a neonate with epidermolysis bullosa. J Laparoendosc Adv Surg Tech A 2013;23:649e50.
Contents lists available at ScienceDirect
Journal of Pediatric Surgery CASE REPORTS journal homepage: www.jpscasereports.com
Pyloric atresia: Comorbidity determines outcomeq Linda M. Schutzman a, Meggen Walsh b, John M. Draus Jr. c, * a
College of Medicine, University of Kentucky, Lexington, KY 40536, USA Department of Pathology, University of Kentucky, Lexington, KY 40536, USA c Department of Surgery, Division of Pediatric Surgery, University of Kentucky, 800 Rose Street, MN-102 Chandler Medical Center, Lexington, KY 40536, USA b
a r t i c l e i n f o
a b s t r a c t
Article history: Received 31 December 2013 Received in revised form 20 March 2014 Accepted 20 March 2014 Available online 13 April 2014
Pyloric atresia is a congenital form of gastric outlet obstruction. The obstruction is relieved surgically, but survival is determined by the presence of additional anomalies. Herein, we report two cases of pyloric atresia. Our first patient was a neonate with epidermolysis bullosa who died of sepsis. The second was an infant who presented with postprandial emesis and had an uncomplicated postoperative course. We discuss the current understanding and management of this rare malformation. Ó 2014 The Authors. Published by Elsevier Inc. All rights reserved.
Key words: Pyloric atresia Gastric outlet obstruction Epidermolysis bullosa
Pyloric atresias are uncommon gastrointestinal defects that represent less than 1% of all bowel atresias. Traditionally, pyloric atresias have been classified by their anatomic morphology. Type 1 atresias are characterized by the presence of a mucosal web between the stomach and duodenum. Type 2 atresias contain a solid longitudinal segment at the pylorus with an intact mesentery. Type 3 atresias are gap aplasias with a disruption between the blind-ending segments of the stomach and duodenum. The surgical correction of pyloric atresia results in excellent long-term function with few complications when it is an isolated defect. Unfortunately, more than half of these children are born with concomitant anomaliesemost commonly epidermolysis bullosa and other intestinal atresias. Such confounding disorders drastically alter the morbidity and mortality associated with this diagnosis. 1. Case report 1 A 1290 g girl was delivered by cesarean section at 33 weeks gestation. The pregnancy had been complicated by polyhydramnios, premature prolonged rupture of the membranes, and late decelerations. Admission radiographs showed a large gastric
q This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/3.0/). * Corresponding author. Tel.: þ1 859 323 5625; fax: þ1 859 323 5289. E-mail addresses: [email protected], [email protected] (J.M. Draus).
bubble and absent intestinal gas which was concerning for pyloric atresia (Fig. 1). The baby was found to have fragile skin, especially at areas of friction, which quickly developed into tense vesicles with scattered areas of erosions, bullae, and crusts (Fig. 2A). Early management included placement of an orogastric tube and initiation of parenteral nutrition. A punch biopsy was diagnostic for junctional epidermolysis bullosa (Fig. 2B). Laparotomy was performed at 1 month of age. We identified a type II pyloric atresia with a 1 cm segment of solid pylorus and an intact mesentery (Fig. 3). A pyloric resection and gastroduodenostomy was performed. Orogastric decompression was discontinued on postoperative day 17. Breast milk feeds were initiated on postoperative day 23 but were soon stopped because the infant developed malabsorption and suspected septicemia. A septic workup was initiated, and prophylactic antibiotics were given. Enteral nutrition was restarted on multiple occasions and always held because of similar consequences. Parental nutrition was continued for a suspected protein-losing enteropathy. Our patient developed intermittent fevers between 38 C and 41 C, and several courses of prophylactic antibiotics were administered. Septic workups never identified an organism. Stool cultures, Clostridium difficile assays, and stool leukocytes remained negative. Eventually, she was intubated for a respiratory distress syndrome, and her general condition continued to deteriorate despite maximum respiratory and nutritional support. On postoperative day 66, the medical staff held a care conference with the girl’s family. A do-not-resuscitate order was obtained, and the baby died at 15 weeks of age.
2213-5766/$ e see front matter Ó 2014 The Authors. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.epsc.2014.03.014
L.M. Schutzman et al. / J Ped Surg Case Reports 2 (2014) 192e195
193
Fig. 3. Intraoperative photograph showing a type II pyloric atresia. Fig. 1. Abdominal radiograph reveals a large gastric bubble with a paucity of intestinal gas.
2. Case report 2 A 9-month-old boy presented to the emergency room with a several week history of non-bilious emesis after most feedings. The boy’s pediatrician had previously started him on ranitidine for the empiric treatment of gastroesophageal reflux. In the emergency room (ER), an acute abdominal series showed a normal bowel gas pattern with the incidental finding of an opaque foreign body in the left upper quadrant. He tolerated an oral fluid challenge, was discharged home, and instructed to follow-up with his pediatrician. The next week, he returned to the ER with continued emesis. Our pediatric gastroenterologist performed a flexible endoscopy. A zipper slider and pull tab was removed from the child’s stomach (Fig. 4A). The pyloric aperture was found to be quite small, and the
neonatal scope could not be advanced through the pylorus (Fig. 4B). Further workup included an abdominal ultrasound to rule out pyloric stenosis and an upper gastrointestinal series which revealed slow passage of contrast out of the stomach (Fig. 5). A pediatric surgical consultation was obtained, and the child was taken to the operating room for exploration. We identified a type 1 pyloric atresia with small perforation within the pyloric web. The web was resected, and a Heineke-Mikulicz pyloroplasty was performed. Oral feedings were resumed on the fourth postoperative day and advanced without complication. 3. Discussion Pyloric atresias are rare, but well-documented forms of gastric outlet obstruction. The exact etiology of this anomaly remains unknown. An autosomal recessive inheritance pattern is displayed in those with a familial predisposition for pyloric atresia [1]. The
Fig. 2. A) Photograph illustrating the skin lesions of our patient with epidermolysis bullosa. B) Electron micrograph of our patient’s skin biopsy. Note the widened dermal/epidermal space. There are areas of lamina densa present; however, the lamina lucida is missing multifocally.
194
L.M. Schutzman et al. / J Ped Surg Case Reports 2 (2014) 192e195
Fig. 4. A) Endoscopic image showing a gastric foreign body. B) Endoscopic image showing a type I pyloric atresia with a small opening.
atresias may be the consequence of developmental arrest or a vascular insult which results in a failure of pyloric recanalization between the 5th and 12th weeks of gestation. Some speculate that the fibrous membrane forms in response to mechanical or chemical irritation [1e3]. Approximately 30%e55% of children with pyloric atresia are found to have other congenital anomaliesemost commonly epidermolysis bullosa or additional intestinal atresias [4]. Epidermolysis bullosa is a genetic disorder that effectively increases collagenase activity resulting in collagen splitting at various locations within the epidermis or dermis. It is classified according to the specific level of involvement: epidermolysis bullosa simplex involves the epidermis; junctional epidermolysis bullosa involves the lamina lucida of the basement membrane; and dystrophic epidermolysis bullosa involves the lamina densa of the basement membrane. Clinically, epidermolysis bullosa is characterized by skin blistering and denudation after the occurrence of seemingly minor trauma.
Fig. 5. Upper gastrointestinal series shows delayed passage of gastric contrast.
Prenatal diagnosis allows for early counseling and improved perinatal management. Ultrasound findings suggesting pyloric atresia include polyhydramnios and gastric dilatation in the absence of the double bubble characteristic of duodenal atresia [3,4]. Due to the significant impact on morbidity and mortality, epidermolysis bullosa must be considered in any fetus suspected of having pyloric atresia. Epidermolysis bullosa can be diagnosed by genetic testing for familial cases, fetal skin biopsy when feasible, or by the presence of characteristic radiologic findings [1,4e6]. Fetal ultrasound findings associated with epidermolysis bullosa include echogenic debris throughout the amniotic fluid, marked narrowing of external orifices, areas of skin blistering or thinning, and malformation of the external ear [4,6]. Fetal MRI may show gastric dilatation, dependent layering of skin debris and bilateral ear defects [4]. Pyloric atresia should be considered in any newborn who presents with clinical manifestations of intestinal obstruction and nonbilious vomiting. As seen in our first case, the diagnosis is confirmed with an abdominal radiograph which shows a single gastric bubble and a paucity of intestinal air. As illustrated by our second case, the diagnosis becomes more challenging when children have a type 1 atresia with a perforated web. These children usually present with feeding intolerance, emesis, and other nonspecific symptoms. Frequently, there is a delay in diagnosis and treatment. Ultrasound and endoscopic exploration may be useful [2]. Children born with epidermolysis bullosa and pyloric atresia face a complicated clinical course, often resulting in death from electrolyte imbalance, protein loss, and septicemia [2,7]. The combination of pyloric atresia and epidermolysis bullosa was traditionally regarded as a subtype of the junctional variant. Recent molecular advancements have elucidated over 70 mutations that result in defective hemidesmosome attachment suggesting that this affiliation is actually a discrete disorder [4,8,9]. Due to variable penetration, children with this disorder express a spectrum of disease severity. There are several reports of long-term survivors, endorsing the aggressive management of this patient population. Current efforts are searching a link between genotype and phenotypic expression which may better predict outcomes [5]. The definitive management of pyloric atresia requires surgical correction of the anatomic blockage. In general, surgery should be delayed until fluid losses and metabolic derangements from the gastric outlet obstruction have been corrected. Durable intravenous access should be obtained, and parenteral nutrition is often necessary. The excessive losses of gastric secretions may result in a
L.M. Schutzman et al. / J Ped Surg Case Reports 2 (2014) 192e195
hypokalemic, hypochloremic metabolic alkalosis which requires aggressive fluid resuscitation and appropriate electrolyte replacement. Early gastric decompression by means of an orogastric or nasogastric sump tube should be initiated to decrease the risk of aspiration and subsequent respiratory complication. The surgical management of pyloric atresias is dictated by the anatomic subtype of atresia identified. Type I atresias are treated with either a Heineke-Mikulicz or Finney pyloroplasty. Type II and type III atresias require excision of the solid pyloric segment with end-to-end gastroduodenostomy. The surgeon must evaluate the remaining bowel for additional atresias. If other intestinal atresias are identified, they typically are repaired during the same operation using standard techniques. Recently, several nonconventional techniques have been described. Type I atresias have been opened with balloon dilation or the utilization of laser technology for either web excision or radical membrane incision [10]. Methods involving pylorus-preserving techniques and pyloric reconstruction and have been described for type II and III atresias. Yokoyama et al. recently presented a surgical alternative in which the atretic pylorus was incised longitudinally and the end-to-end anastomosis was placed anterior to the preserved pylorus [10]. Dessanti et al. has described a method involving the anatomic reconstruction of a pyloric sphincter [11]. These methods have been tried on a few patients in hopes of preventing the alkaline gastritis commonly associated with traditional end-to-end gastroduodenostomy. More data are needed to evaluate their safety and long-term efficacy. The management of pyloric atresia in a patient with epidermolysis bullosa presents significant perioperative challenges for the medical team. Severe metabolic disturbances and septic complications may arise secondarily to the excessive denudation of the skin [1,2,12]. Dedicated attention to electrolyte replacement and nutritional support are essential. A recent report of a successful laparoscopic pyloroplasty presents an attractive alternative for the epidermolysis bullosa patient who is already burdened by the metabolic demands of the disorder [13]. 4. Conclusion Pyloric atresia is a serious congenital gastric outlet obstruction. When found alone, pyloric atresias have favorable outcomes.
195
However, the clinical course is drastically altered by the coexistence of epidermolysis bullosa. We have described two patients who received care at our institution. Furthermore, we have discussed the current knowledge regarding etiology, classification, diagnosis and treatment of this rare anomaly. Conflict of interest statement The authors of the case report entitled “Pyloric Atresia: Comorbidity Determines Outcome” have no conflicts of interest to report. References [1] Al-Salem AH. Congenital pyloric atresia and associated anomalies. Pediatr Surg Int 2007;23:559e63. [2] Ilce Z, Erdogan E, Kara C, Celayir S, Sarimurat N, Senyuz OF, et al. Pyloric atresia: 15 year review from a single institution. J Pediatr Surg 2003;38: 1581e4. [3] Andriessen MJG, Matthyssens LE, Heij HA. Pyloric atresia. J Pediatr Surg 2010; 45:2470e2. [4] Merrow AC, Frischer JS, Lucky AW. Pyloric atresia with epidermolysis bullosa: fetal MRI diagnosis with postnatal correlation. Pediatr Radiol 2013;43: 1656e61. [5] Sicot BDJ, Derueele P, Kacet N, Vaillant C, Subtil D. Prenatal findings in epidermolysis bullosa with pyloric atresia in a family not known to be at risk. Ultrasound Obstet Gynecol 2005;25:607e9. [6] Maurice P, Eyrolle-Guignot D, Dhombres F, Carel C, Gonzales M, Muller F, et al. The key role of ultrasound examination in the prenatal diagnosis of epidermolysis bullosa with pyloric atresia. Prenat Diagn 2013;33:908e9. [7] Samad L, Siddiqui EF, Arain MA, Atif M, Parkash J, Ahmed S. Pyloric atresia associated with epidermolysis bullosa - three cases presenting in three months. J Pediatr Surg 2004;39:1267e9. [8] Schumann H, Kiritsi D, Pigors M, Kahlhas J, Peters J, Ott H, et al. Phenotypic spectrum of epidermolysis bullosa associated with a6b4 integrin mutations. Br J Dermatol 2013;169:115e24. [9] Fu AC, Hon KL, Choi PC. A neonate with generalized bullae and pyloric atresia. Hong Kong Med J 2013;19(188):e1e2. [10] Yokoyama S, Utsunomiya H. A case of successful surgical repair for solid segment type pyloric atresia using a novel gastroduodenostomy procedure. J Pediatr Surg 2012;47:2158e60. [11] Dessanti A, Di Benetto V, Iannuccelli M, Cossu Rocca P, Di Benedetto A. Pyloric atresia: a new operation to reconstruct the pyloric sphincter. J Pediatr Surg 2004;39:297e301. [12] Dank JP, Kim S, Parisi MA, Brown T, Smith LT, Waldhousen J, et al. Outcome after surgical repair of junctional epidermolysis bullosa-pyloric atresia syndrome: a report of 3 cases and review of the literature. Arch Dermatol 1999; 135:1243e7. [13] Son TN, Hoan VX. Laparoscopic management of pyloric atresia in a neonate with epidermolysis bullosa. J Laparoendosc Adv Surg Tech A 2013;23:649e50.