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Neonatal “Near Misses” Encountered in the Emergency Department
Abstract: Pediatric surgical diseases may present in a delayed fashion to the emergency department. In addition, these cases may have abnormal presentations when compared with presentations in the setting of the neonatal intensive care unit. A high degree of suspicion on the part of emergency physicians will allow for prompt and definitive consultation and treatment of our youngest patients. Here we present 3 such cases that were encountered in our emergency department that show the importance of a high index of suspicion.
Keywords:
Neonatal “Near Misses” Encountered in the Emergency Department
malrotation; volvulus; Hirschsprung disease; patent ductus arteriosus
Fredrick J. Bohanon, MD*, Brittany M. Graham, BS*, Ravi S. Radhakrishnan, MD *†
N *Department of Surgery, University of Texas Medical Branch, Galveston, TX; †Department of Pediatrics, University of Texas Medical Branch, Galveston, TX. Reprint requests and correspondence: Ravi S. Radhakrishnan, MD, Department of Surgery, The University of Texas Medical Branch, 301 University Boulevard, Galveston, Texas 77555-0353. [email protected] (F.J. Bohanon), [email protected] (B.M. Graham), [email protected] (R.S. Radhakrishnan) 1522-8401 © 2014 Elsevier Inc. All rights reserved.
eonatal surgical “near misses” that present to the emergency department (ED) must be recognized early and treated promptly. Frequently, the presenting symptoms are difficult to assess or are absent in infants and neonates, leading to misdiagnosis or delay in diagnosis. Here we discuss 3 pediatric surgical cases presenting to our ED. The first case is a 1-week-old with an intestinal malrotation with midgut volvulus. The second case is an older infant with delayed diagnosis of Hirschsprung disease. The third case is a 7-week-old infant with failure to thrive and congestive heart failure secondary to a patent-ductus arteriosis.
CASE 1 A female infant was born at 40 weeks gestation via an uneventful cesarean delivery with a birth weight of 3.3 kg. During the first week of life, the mother reported poor feeding and persistent nonbilious vomiting. The baby presented to the ED at 1 week of life with significant weight loss and with the following vital signs: heart rate, 126 beats/min; respiratory rate, 44 breaths/min; temperature, 36.7°C; blood pressure, 83/60 mm Hg; and weight, 3.17 kg. On physical examination, the baby was alert and active and had moist mucus membranes, heart and breaths sounds were normal, and the upper abdomen was distended with bowel loops palpable. An NEONATAL NEAR MISSES IN THE ED / BOHANON ET AL. • VOL. 15, NO. 3 231
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Figure 1. Supine abdominal radiograph demonstrating multiple distended loops of small bowel consistent with intestinal obstruction.
abdominal x-ray was obtained showing dilated proximal small bowel concerning for proximal intestinal obstruction (Figure 1). A barium enema was then conducted demonstrating a displaced cecum in the right upper quadrant. Despite the absence of bilious emesis, these findings were concerning for intestinal malrotation (Figure 2). Pediatric surgery was consulted, and the patient was taken emergently to the operating room for exploratory laparotomy. At surgery, the baby was noted to have Ladd's bands extending from the right colon across the duodenum to the lateral abdominal wall and malrotation with volvulus. The small bowel was viable at the conclusion of surgery. The baby began oral feeds on postoperative day 7 and discharged home soon thereafter with normal bowel movements.
INTESTINAL MALROTATION Intestinal malrotation is a congenital anomaly of intestinal rotation. 1 Intestinal obstruction may occur if cecal (Ladd's) bands are present or if midgut volvulus arises, which is a twisting of the mesentery leading to ischemia and possibly necrosis and perforation. In one series, 80% of patients presented in the first month of life, with bilious emesis (97%) being the primary symptom. 2 They further report that most of these patients had prior contact with health care professionals that may have resulted in an
Figure 2. Contrast enema in the same patient as Fig. 1. Displaced cecum in the right upper quadrant is concerning for malrotation.
earlier diagnosis and treatment, if malrotation had been in the differential diagnosis. 2
CASE 2 A 39-week gestational age boy was born via spontaneous vaginal delivery with a birth weight of 3.9 kg. The baby had a medical history of ABO incompatibility, term large for gestational age, poor feeding, and maternal chorioamionitis. The baby passed meconium on the first day of life and was having 4 yellow-seedy bowel movements per day at discharge. The infant was in his normal state of health, being breast-fed with formula supplementation, until 7 weeks of life. At 7 weeks of age, he presented with his mother to an urgent care facility for a several day history of constipation. The mother reported using apple juice as a laxative with minimal relief. The urgent care facility gave a suppository, and a small hard pellet of stool was expressed with abdominal straining. He was released with the recommendation of 1 oz of apple juice with 1 oz of water twice a day. Four days later, the baby was taken to the ED for a 4-day history of having no bowel movements, poor feeding, and abdominal straining. An abdominal x-ray was obtained showing distention of the large and small bowel (Figure 3). Pediatric surgery was consulted for the constipation, and a barium enema was performed (Figure 4). The barium enema showed no transition point or stenotic bowel. Despite appropriate passage of meconium and
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no radiologic evidence of a transition zone, the diagnosis of Hirschsprung was entertained and consent was obtained for rectal biopsies and fecal disimpaction. Histological examination revealed the absence of ganglion cells in several of the biopsy specimens and calretinin staining was entirely negative, leading to the diagnosis of Hirschsprung disease. The baby then underwent a laparoscopicassisted Swenson-type pull-through procedure with coloanal anastomosis. Postoperatively, the patient did well and is currently having normal bowel movements and undergoing serial anal dilations per protocol. Hirschsprung disease is a motor disorder of the colon and rectum caused by improper cephalocaudal migration of neural crest cells (ganglionic precursors) into the distal bowel. The failure of neural crest cell migration leads to a lack of the myenteric plexus within the bowel wall and results in areas of unopposed sympathetic tone. Aganglionic areas of unopposed sympathetic tone lead to an inability of bowel wall relaxation, resulting in a functional obstruction of the bowel. Patients may present with FTT, bilious emesis, chronic constipation, and abdominal distention. The severity of Hirschsprung disease varies from failure to pass meconium within the first 48 hours after birth to the life-threatening complication of Hirschsprungassociated enterocolitis. The mainstay of treatment is surgery, removing the aganglionic bowel, pulling through ganglionic bowel, and preserving the anal canal and sphincter mechanism. 3
Figure 3. Supine abdominal radiograph illustrating diffuse gaseous distention of large and small bowel with formed fecal material in the rectosigmoid colon.
Figure 4. Contrast enema in the same patient as Fig. 3. Rectum and sigmoid colon were filled with contrast, and no radiographic sign of obstruction is seen.
CASE 3 A 35-week gestational age boy was born via cesarean delivery for breech positioning and was noted to be pale and limp at birth with meconium staining and respiratory failure requiring intubation and resuscitation. Birth weight was 1.9 kg. After initial respiratory failure, the neonate recovered and began oral feeds on day of life 5 and was tolerating full feeds on day of life 17. Additional comorbidities included multiple chromosomal abnormalities, dysmorphic facies, and a systolic murmur. The patient was discharged on day of life 22. At 7 weeks of age, the patient presented to the ED for failure to thrive and respiratory distress. The child was admitted and treated for an upper respiratory tract infection. On physical examination, a 4/6 machinery-like holosystolic-type murmur was identified. Subsequently, an echocardiogram was ordered and found a large patent ductus arteriosus (PDA) and a large secundum atrial septal defect with a left-to-right shunt, but no signs of congestive heart failure. The patient was discharged after 9 days of treatment for the respiratory tract infection. Three weeks after this admission, the infant presented to the ED for failure to thrive and respiratory distress. A
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Figure 5. Decision support algorithm for infants with persistent vomiting.
repeat echocardiogram demonstrated the same PDA and secundum atrial septal defect with further findings of congestive heart failure. Pediatric surgery was consulted and a decision was made to close the PDA. The pediatric surgery team performed an uneventful ligation of the PDA via left thoracotomy. The patient had no postoperative complications. The patient eventually transferred to another institution per family requests, tolerating feeds. Patent ductus arteriosis is a condition in which the ductus arteriosis fails to close after birth. This closure should occur within the first 12 to 18 hours, functionally. Anatomic closure should occur in 2 to 3 weeks. 4 The incidence of PDA is approximately 1 in 2000 live births, accounting for 5 to 10% of congenital heart diseases, making PDA one of the most common congenital cardiovascular malformations. 5 The clinical sequelae of PDA range from asymptomatic to a range of severe conditions including failure to thrive, bronchopulmonary dysplasia, renal failure, necrotizing enterocolitis, and congestive heart failure. 6
SUMMARY Neonatal surgical near misses do present to the ED and with unusual presentations of common conditions. It is paramount to consider these diseases in our differential diagnosis. Figure 5 is a decision support algorithm to consider when evaluating persistent vomiting. The ED is a place that will commonly encounter these atypical surgical patients, and therefore, constant vigilance and appreciation for these diseases are needed to prevent a delay in correct diagnosis and treatment.
REFERENCES 1. Applegate KE, Anderson JM, Klatte EC. Intestinal malrotation in children: a problem-solving approach to the upper gastrointestinal series. Radiographics 2006;26:1485–500. 2. Shalaby MS, Kuti K, Walker G. Intestinal malrotation and volvulus in infants and children. Br Med J 2013;347:f6949.
NEONATAL NEAR MISSES IN THE ED / BOHANON ET AL. • VOL. 15, NO. 3 235
3. Levitt MA, Hamrick MC, Eradi B, et al. Transanal, full-thickness, Swenson-like approach for Hirschsprung disease. J Pediatr Surg 2013;48:2289–95. 4. Anilkumar M. Patent ductus arteriosus. Cardiol Clin 2013;31:417–30.
5. Schneider DJ, Moore JW. Patent ductus arteriosus. Circulation 2006;114:1873–82. 6. Saldeno YP, Favareto V, Mirpuri J. Prolonged persistent patent ductus arteriosus: potential perdurable anomalies in premature infants. J Perinatol 2012;32:953–8.
Keywords:
Neonatal “Near Misses” Encountered in the Emergency Department
malrotation; volvulus; Hirschsprung disease; patent ductus arteriosus
Fredrick J. Bohanon, MD*, Brittany M. Graham, BS*, Ravi S. Radhakrishnan, MD *†
N *Department of Surgery, University of Texas Medical Branch, Galveston, TX; †Department of Pediatrics, University of Texas Medical Branch, Galveston, TX. Reprint requests and correspondence: Ravi S. Radhakrishnan, MD, Department of Surgery, The University of Texas Medical Branch, 301 University Boulevard, Galveston, Texas 77555-0353. [email protected] (F.J. Bohanon), [email protected] (B.M. Graham), [email protected] (R.S. Radhakrishnan) 1522-8401 © 2014 Elsevier Inc. All rights reserved.
eonatal surgical “near misses” that present to the emergency department (ED) must be recognized early and treated promptly. Frequently, the presenting symptoms are difficult to assess or are absent in infants and neonates, leading to misdiagnosis or delay in diagnosis. Here we discuss 3 pediatric surgical cases presenting to our ED. The first case is a 1-week-old with an intestinal malrotation with midgut volvulus. The second case is an older infant with delayed diagnosis of Hirschsprung disease. The third case is a 7-week-old infant with failure to thrive and congestive heart failure secondary to a patent-ductus arteriosis.
CASE 1 A female infant was born at 40 weeks gestation via an uneventful cesarean delivery with a birth weight of 3.3 kg. During the first week of life, the mother reported poor feeding and persistent nonbilious vomiting. The baby presented to the ED at 1 week of life with significant weight loss and with the following vital signs: heart rate, 126 beats/min; respiratory rate, 44 breaths/min; temperature, 36.7°C; blood pressure, 83/60 mm Hg; and weight, 3.17 kg. On physical examination, the baby was alert and active and had moist mucus membranes, heart and breaths sounds were normal, and the upper abdomen was distended with bowel loops palpable. An NEONATAL NEAR MISSES IN THE ED / BOHANON ET AL. • VOL. 15, NO. 3 231
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Figure 1. Supine abdominal radiograph demonstrating multiple distended loops of small bowel consistent with intestinal obstruction.
abdominal x-ray was obtained showing dilated proximal small bowel concerning for proximal intestinal obstruction (Figure 1). A barium enema was then conducted demonstrating a displaced cecum in the right upper quadrant. Despite the absence of bilious emesis, these findings were concerning for intestinal malrotation (Figure 2). Pediatric surgery was consulted, and the patient was taken emergently to the operating room for exploratory laparotomy. At surgery, the baby was noted to have Ladd's bands extending from the right colon across the duodenum to the lateral abdominal wall and malrotation with volvulus. The small bowel was viable at the conclusion of surgery. The baby began oral feeds on postoperative day 7 and discharged home soon thereafter with normal bowel movements.
INTESTINAL MALROTATION Intestinal malrotation is a congenital anomaly of intestinal rotation. 1 Intestinal obstruction may occur if cecal (Ladd's) bands are present or if midgut volvulus arises, which is a twisting of the mesentery leading to ischemia and possibly necrosis and perforation. In one series, 80% of patients presented in the first month of life, with bilious emesis (97%) being the primary symptom. 2 They further report that most of these patients had prior contact with health care professionals that may have resulted in an
Figure 2. Contrast enema in the same patient as Fig. 1. Displaced cecum in the right upper quadrant is concerning for malrotation.
earlier diagnosis and treatment, if malrotation had been in the differential diagnosis. 2
CASE 2 A 39-week gestational age boy was born via spontaneous vaginal delivery with a birth weight of 3.9 kg. The baby had a medical history of ABO incompatibility, term large for gestational age, poor feeding, and maternal chorioamionitis. The baby passed meconium on the first day of life and was having 4 yellow-seedy bowel movements per day at discharge. The infant was in his normal state of health, being breast-fed with formula supplementation, until 7 weeks of life. At 7 weeks of age, he presented with his mother to an urgent care facility for a several day history of constipation. The mother reported using apple juice as a laxative with minimal relief. The urgent care facility gave a suppository, and a small hard pellet of stool was expressed with abdominal straining. He was released with the recommendation of 1 oz of apple juice with 1 oz of water twice a day. Four days later, the baby was taken to the ED for a 4-day history of having no bowel movements, poor feeding, and abdominal straining. An abdominal x-ray was obtained showing distention of the large and small bowel (Figure 3). Pediatric surgery was consulted for the constipation, and a barium enema was performed (Figure 4). The barium enema showed no transition point or stenotic bowel. Despite appropriate passage of meconium and
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no radiologic evidence of a transition zone, the diagnosis of Hirschsprung was entertained and consent was obtained for rectal biopsies and fecal disimpaction. Histological examination revealed the absence of ganglion cells in several of the biopsy specimens and calretinin staining was entirely negative, leading to the diagnosis of Hirschsprung disease. The baby then underwent a laparoscopicassisted Swenson-type pull-through procedure with coloanal anastomosis. Postoperatively, the patient did well and is currently having normal bowel movements and undergoing serial anal dilations per protocol. Hirschsprung disease is a motor disorder of the colon and rectum caused by improper cephalocaudal migration of neural crest cells (ganglionic precursors) into the distal bowel. The failure of neural crest cell migration leads to a lack of the myenteric plexus within the bowel wall and results in areas of unopposed sympathetic tone. Aganglionic areas of unopposed sympathetic tone lead to an inability of bowel wall relaxation, resulting in a functional obstruction of the bowel. Patients may present with FTT, bilious emesis, chronic constipation, and abdominal distention. The severity of Hirschsprung disease varies from failure to pass meconium within the first 48 hours after birth to the life-threatening complication of Hirschsprungassociated enterocolitis. The mainstay of treatment is surgery, removing the aganglionic bowel, pulling through ganglionic bowel, and preserving the anal canal and sphincter mechanism. 3
Figure 3. Supine abdominal radiograph illustrating diffuse gaseous distention of large and small bowel with formed fecal material in the rectosigmoid colon.
Figure 4. Contrast enema in the same patient as Fig. 3. Rectum and sigmoid colon were filled with contrast, and no radiographic sign of obstruction is seen.
CASE 3 A 35-week gestational age boy was born via cesarean delivery for breech positioning and was noted to be pale and limp at birth with meconium staining and respiratory failure requiring intubation and resuscitation. Birth weight was 1.9 kg. After initial respiratory failure, the neonate recovered and began oral feeds on day of life 5 and was tolerating full feeds on day of life 17. Additional comorbidities included multiple chromosomal abnormalities, dysmorphic facies, and a systolic murmur. The patient was discharged on day of life 22. At 7 weeks of age, the patient presented to the ED for failure to thrive and respiratory distress. The child was admitted and treated for an upper respiratory tract infection. On physical examination, a 4/6 machinery-like holosystolic-type murmur was identified. Subsequently, an echocardiogram was ordered and found a large patent ductus arteriosus (PDA) and a large secundum atrial septal defect with a left-to-right shunt, but no signs of congestive heart failure. The patient was discharged after 9 days of treatment for the respiratory tract infection. Three weeks after this admission, the infant presented to the ED for failure to thrive and respiratory distress. A
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Figure 5. Decision support algorithm for infants with persistent vomiting.
repeat echocardiogram demonstrated the same PDA and secundum atrial septal defect with further findings of congestive heart failure. Pediatric surgery was consulted and a decision was made to close the PDA. The pediatric surgery team performed an uneventful ligation of the PDA via left thoracotomy. The patient had no postoperative complications. The patient eventually transferred to another institution per family requests, tolerating feeds. Patent ductus arteriosis is a condition in which the ductus arteriosis fails to close after birth. This closure should occur within the first 12 to 18 hours, functionally. Anatomic closure should occur in 2 to 3 weeks. 4 The incidence of PDA is approximately 1 in 2000 live births, accounting for 5 to 10% of congenital heart diseases, making PDA one of the most common congenital cardiovascular malformations. 5 The clinical sequelae of PDA range from asymptomatic to a range of severe conditions including failure to thrive, bronchopulmonary dysplasia, renal failure, necrotizing enterocolitis, and congestive heart failure. 6
SUMMARY Neonatal surgical near misses do present to the ED and with unusual presentations of common conditions. It is paramount to consider these diseases in our differential diagnosis. Figure 5 is a decision support algorithm to consider when evaluating persistent vomiting. The ED is a place that will commonly encounter these atypical surgical patients, and therefore, constant vigilance and appreciation for these diseases are needed to prevent a delay in correct diagnosis and treatment.
REFERENCES 1. Applegate KE, Anderson JM, Klatte EC. Intestinal malrotation in children: a problem-solving approach to the upper gastrointestinal series. Radiographics 2006;26:1485–500. 2. Shalaby MS, Kuti K, Walker G. Intestinal malrotation and volvulus in infants and children. Br Med J 2013;347:f6949.
NEONATAL NEAR MISSES IN THE ED / BOHANON ET AL. • VOL. 15, NO. 3 235
3. Levitt MA, Hamrick MC, Eradi B, et al. Transanal, full-thickness, Swenson-like approach for Hirschsprung disease. J Pediatr Surg 2013;48:2289–95. 4. Anilkumar M. Patent ductus arteriosus. Cardiol Clin 2013;31:417–30.
5. Schneider DJ, Moore JW. Patent ductus arteriosus. Circulation 2006;114:1873–82. 6. Saldeno YP, Favareto V, Mirpuri J. Prolonged persistent patent ductus arteriosus: potential perdurable anomalies in premature infants. J Perinatol 2012;32:953–8.