Ileal Perforation
Due to Arteriovenous in a Premature Infant
By John Munn, Aliya N. Hussain,
Melanie
J. Castelli, Sheila M. Diamond,
Maywood, 0 Gastrointestinal arteriovenous malformations (AVM) in children are most commonly associated with bleeding. Although not previously reported, we present a case of an AVM associated with intestinal perforation in a premature infant. 0 1990 by W.B. Saunders Company. INDEX WORDS: Arteriovenous tinal perforation, infant.
malformation,
infant; intes-
I
N THIS REPORT, a case of ileal perforation in a premature infant suspected of having atypical perforated necrotizing enterocolitis (NEC) is reported. Operative findings indicated spontaneous perforation and histological evaluation disclosed perforation secondary to an arteriovenous malformation (AVM). CASE
REPORT
A 970-g boy, one of twins, was born via cesarean section at 27 weeks’ gestation to a 34-year-old, healthy primigravid. Apgar scores
were 5 and 7. The infant was intubated for mild respiratory distress and ventilated with supplemental oxygen. An umbilical vein catheter was placed and he was transferred to the Loyola University Medical Center Neonatal Intensive Care Unit. Blood, urine, and spinal fluid cultures were obtained and were sterile after 72 hours. Chest roentgenogram and abdomina1 films were normal. Antibiotics were administered. The child rapidly stabilized and was extubated the following day. On the third day of life, the baby became mottled and acidotic. He was reintubated and resuscitated. A generalized seizure followed and phenobarbital was given. Cranial ultrasound demonstrated a grade IV intracranial hemorrhage. Over the next 3 days there was gradual improvement and the child was weaned from the ventilator. Nutrition was maintained with peripheral hyperalimentation. On the sixth day of life, the baby developed abdominal distention and discoloration. There was minimal tenderness. A nasogastric tube was placed. Abdominal radiographs showed pneumoperitoneum without pneumatosis intestinalis or portal vein air. Laboratory studies indicated a prolonged prothrombin time and partial thromboplastin time (15.5 and 68.8, respectively). The platelet count was 77,000 and the fibrinogen was 97. At laparotomy, there was free bile stained fluid and air within the abdominal cavity. There was minimal peritoneal inflammation. A 5 mm perforation of the terminal ileum with adjacent hemorrhagic changes was identified. The remaining small intestine, colon, and stomach were completely normal. Because of the paucity of peritoneal inflammation and the isolation of the lesion, a 2-cm segment of ileum was resected and a primary anastomosis was performed. Intraoperative abdominal fluid cultures grew no organisms. Postoperatively, the baby did well. He was discharged home at 11 weeks of age weighing 2.5 kg on a normal diet. He is doing well on follow-up 7 months later. Pathology
Gross examination of the specimen showed a 5 x 2 mm perforation with irregular margins located on the antimesenteric surface of Journal
of Pediatric
Surgery,
Malformation
Vol 25, No 6 (June), 1990: pp 701-703
and Preston R. Black
Illinois
the ileum. There was gray-black discoloration of the adjacent serosa. Microscopic examination of the intestine surrounding the perforation demonstrated multiple, thin-walled, dilated vessels in the submucosa compatible with AVM (Fig 1). There were multiple foci of recent hemorrhage in the adjoining tissue. No ectopic tissue was noted. Adjacent mucosa showed no necrosis or inflammation. An additional finding was marked thinning of the muscularis propria subjacent to the AVM (Fig 2). Near the site of perforation only a few strands of muscie were remaining (Fig 3). DISCUSSION
Perforation of the gastrointestinal tract in infancy is an infrequent occurrence that carries significant mortality. Newborns at risk for perforation include critically ill neonates with NEC’** and those with obstruction or congenital anomalies of the intestinal tract1.3-5 Iatrogenic intestinal perforation may be related to gastric intubation or oxygen supplementation via mask or cannula.iY6Additionally, intestinal perforations may occur spontaneously related to the stress of sepsis or asphyxia. Over 400 such cases have been collected and reviewed since 1825.1,4,7,8 AVM may be encountered in any part of the body, including the upper and lower extremities, the head and neck, the gut, the liver, the lungs, and the central nervous system. However, symptomatic AVM in infants under 6 months of age generally are located in the central nervous system, in the lungs, or in the liver. They usually present clinically as a mass and may occasionally be associated with refractile congestive heart failure, cyanosis, or a bruit.g Symptomatic AVM of the gastrointestinal tract are unusual lesions in childhood, which if they come to clinical attention, do so most frequently because of bleeding, Typically, patients with gastrointestinal AVM develop acute, massive hematochezia or chronic melena with irondeficiency anemia. Gastrointestinal AVM may become clinically apparent at any age.” Perforation from a gastrointestinal AVM has not been reported.” Careful perusal of the literature has uncovered only one case in which an intestinal AVM was symptomatic in a
From the Division of Pediatric Surgery, the Departments of Surgery and Pathology, The Stritch School of Medicine, Loyola University Medical Center, Maywood. IL. Address reprint requests to Preston R. Black, MD, Department of Surgery, Loyola University Medical Center, 2160 S First Ave. Maywood, IL 60153. Q 1990 by W.B. Saunders Company. 0022-3468/90/2506-0033$03,00/O
701
702
Fig 1. Microscopic section of ileum demonstrating multiple thin-walled blood vessels in the submucoss with adjacent hemorrhege (H&E. original magnification x 100).
newborn for a reason other than hemorrhage. This was a child with an ileal AVM that caused intestinal ..? obstruction.“ Although NEC is the most common cause of intestinal perforation in this patient population, the clinical signs and symptoms and the operative findings in the present case were not typical of this disease. Histological analysis was most consistent with the perforation occurring in an area where there was a collection of dilated submucosal blood vessels consistent with an AVM associated with a marked thinning of the muscularis propria.” To our knowledge, there has never been a report of an intestinal AVM causing perforation in a neonate. Therefore, we believe that this report represents the first description of a gastrointestinal arteriovenous malformation resulting in intestinal perforation in a premature infant. The reason that the AVM led to intestinal perforation in this child is not clear. One possibility is that the perforation was initiated by acute hemorrhage of the AVM. There was hemorrhage noted microscopically in
MUNN ET AL
Fig 2. Extreme thinning of muscularis propria subjacent to rubmucossl AVM (H&E, original magnification x 100).
the lesion and preoperative coagulation studies disclosed a mild coagulopathy. The acute hemorrhage could have weakened the intestinal wall and led to rupture of the ileum. Another possibility could be that
Fig 3. High power of area seen in Fig 2 (H&E. original magnification x400).
ILEAL PERFORATION SECONDARY TO AVM
703
there was thinning or absence of the muscle layers of the intestine in the area of the AVM. This could have resulted in an area of inherent weakness in the intestinal wall. Rare cases of absence of intestinal wall musculature in neonates have been described.‘3“5 How-
ever, none of these cases were associated with AVM and in all of the cases there was circumferential absence of muscle over variable lengths of bowel. Most of these children presented with obstruction and none had perforation.
REFERENCES 1. Bell MJ: Perforation of the gastrointestinal tract and peritonitis in the neonate. Surg Gynecol Obstet 160:20-26, 1985 2. Wayne ER, Burrington JD, Hutter J: Neonatal nectrotizing enterocolitis. Arch Surg 110:476-480, 1975 3. Emanuel B, Zlotniv P, Raffensperger JG: Perforation of the intestinal tract in infancy and childhood. Surg Gynecol Obstet 146:926-929,1978 4. Thelander HE: Perforation of the gastrointestinal tract of the newborn infant. Am J Dis Child 58:371-393, 1939 5. Cruze K, Snyder WH: Acute perforation of the alimentary tract in infancy and childhood. Ann Surg 154:93-99,196l 6. Garland JS, Nelson DB, Rice T, et al: Increased risk of gastrointestinal perforations in neonates mechanically ventilated with either face mask or nasal prongs. Pediatrics 76:406-410, 1973 7. Lloyd JR: The etiology of gastrointestinal perforations in the newborn. J Pediatr Surg 4:77-84, 1969 8. Parrish RA, Sherman RT, Wilson H: Spontaneous rupture of the gastroenteric tract in the newborn. Ann Surg 159:244-251, 1964 9. Knudson RP, Alden ER: Symptomatic arteriovenous malfor-
mation in infants less than 6 months of age. Pediatrics 64:238-240, 1979 10. Moore JD, Thompson NW, Appelman HD, et al: Arteriovenous malformations of the gastrointestinal tract. Arch Surg 111:381-389, 1976 11. Richardson JD, Max MH, Flint LM, et al: Bleeding vascular malformations of the intestines. Surgery 84:430-436, 1978 12. Graivier L: Ileal stenosis due to arteriovenous malformation in a newborn infant. J Pediatr Surg 17:78-79, 1982 13. Steiner DH, Maxwell JG, Rasmussen BL, et al: Segmental absence of intestinal musculature: An unusual cause of intestinal obstruction in the neonate. Am J Surg 118:964-967, 1969 14. Carroll RL Jr: Absence of musculature of the distal ileum: A cause of neonatal intestinal obstruction. J Pediatr Surg 8:29-31, 1973 15. Dhall JC, Khatri HL, Jaiswal TS, et al: Congenital segmental absence of intestinal musculature: A rare cause of intestinal obstruction in a neonate. Am J Gastroenterol70:401-403, 1978
Due to Arteriovenous in a Premature Infant
By John Munn, Aliya N. Hussain,
Melanie
J. Castelli, Sheila M. Diamond,
Maywood, 0 Gastrointestinal arteriovenous malformations (AVM) in children are most commonly associated with bleeding. Although not previously reported, we present a case of an AVM associated with intestinal perforation in a premature infant. 0 1990 by W.B. Saunders Company. INDEX WORDS: Arteriovenous tinal perforation, infant.
malformation,
infant; intes-
I
N THIS REPORT, a case of ileal perforation in a premature infant suspected of having atypical perforated necrotizing enterocolitis (NEC) is reported. Operative findings indicated spontaneous perforation and histological evaluation disclosed perforation secondary to an arteriovenous malformation (AVM). CASE
REPORT
A 970-g boy, one of twins, was born via cesarean section at 27 weeks’ gestation to a 34-year-old, healthy primigravid. Apgar scores
were 5 and 7. The infant was intubated for mild respiratory distress and ventilated with supplemental oxygen. An umbilical vein catheter was placed and he was transferred to the Loyola University Medical Center Neonatal Intensive Care Unit. Blood, urine, and spinal fluid cultures were obtained and were sterile after 72 hours. Chest roentgenogram and abdomina1 films were normal. Antibiotics were administered. The child rapidly stabilized and was extubated the following day. On the third day of life, the baby became mottled and acidotic. He was reintubated and resuscitated. A generalized seizure followed and phenobarbital was given. Cranial ultrasound demonstrated a grade IV intracranial hemorrhage. Over the next 3 days there was gradual improvement and the child was weaned from the ventilator. Nutrition was maintained with peripheral hyperalimentation. On the sixth day of life, the baby developed abdominal distention and discoloration. There was minimal tenderness. A nasogastric tube was placed. Abdominal radiographs showed pneumoperitoneum without pneumatosis intestinalis or portal vein air. Laboratory studies indicated a prolonged prothrombin time and partial thromboplastin time (15.5 and 68.8, respectively). The platelet count was 77,000 and the fibrinogen was 97. At laparotomy, there was free bile stained fluid and air within the abdominal cavity. There was minimal peritoneal inflammation. A 5 mm perforation of the terminal ileum with adjacent hemorrhagic changes was identified. The remaining small intestine, colon, and stomach were completely normal. Because of the paucity of peritoneal inflammation and the isolation of the lesion, a 2-cm segment of ileum was resected and a primary anastomosis was performed. Intraoperative abdominal fluid cultures grew no organisms. Postoperatively, the baby did well. He was discharged home at 11 weeks of age weighing 2.5 kg on a normal diet. He is doing well on follow-up 7 months later. Pathology
Gross examination of the specimen showed a 5 x 2 mm perforation with irregular margins located on the antimesenteric surface of Journal
of Pediatric
Surgery,
Malformation
Vol 25, No 6 (June), 1990: pp 701-703
and Preston R. Black
Illinois
the ileum. There was gray-black discoloration of the adjacent serosa. Microscopic examination of the intestine surrounding the perforation demonstrated multiple, thin-walled, dilated vessels in the submucosa compatible with AVM (Fig 1). There were multiple foci of recent hemorrhage in the adjoining tissue. No ectopic tissue was noted. Adjacent mucosa showed no necrosis or inflammation. An additional finding was marked thinning of the muscularis propria subjacent to the AVM (Fig 2). Near the site of perforation only a few strands of muscie were remaining (Fig 3). DISCUSSION
Perforation of the gastrointestinal tract in infancy is an infrequent occurrence that carries significant mortality. Newborns at risk for perforation include critically ill neonates with NEC’** and those with obstruction or congenital anomalies of the intestinal tract1.3-5 Iatrogenic intestinal perforation may be related to gastric intubation or oxygen supplementation via mask or cannula.iY6Additionally, intestinal perforations may occur spontaneously related to the stress of sepsis or asphyxia. Over 400 such cases have been collected and reviewed since 1825.1,4,7,8 AVM may be encountered in any part of the body, including the upper and lower extremities, the head and neck, the gut, the liver, the lungs, and the central nervous system. However, symptomatic AVM in infants under 6 months of age generally are located in the central nervous system, in the lungs, or in the liver. They usually present clinically as a mass and may occasionally be associated with refractile congestive heart failure, cyanosis, or a bruit.g Symptomatic AVM of the gastrointestinal tract are unusual lesions in childhood, which if they come to clinical attention, do so most frequently because of bleeding, Typically, patients with gastrointestinal AVM develop acute, massive hematochezia or chronic melena with irondeficiency anemia. Gastrointestinal AVM may become clinically apparent at any age.” Perforation from a gastrointestinal AVM has not been reported.” Careful perusal of the literature has uncovered only one case in which an intestinal AVM was symptomatic in a
From the Division of Pediatric Surgery, the Departments of Surgery and Pathology, The Stritch School of Medicine, Loyola University Medical Center, Maywood. IL. Address reprint requests to Preston R. Black, MD, Department of Surgery, Loyola University Medical Center, 2160 S First Ave. Maywood, IL 60153. Q 1990 by W.B. Saunders Company. 0022-3468/90/2506-0033$03,00/O
701
702
Fig 1. Microscopic section of ileum demonstrating multiple thin-walled blood vessels in the submucoss with adjacent hemorrhege (H&E. original magnification x 100).
newborn for a reason other than hemorrhage. This was a child with an ileal AVM that caused intestinal ..? obstruction.“ Although NEC is the most common cause of intestinal perforation in this patient population, the clinical signs and symptoms and the operative findings in the present case were not typical of this disease. Histological analysis was most consistent with the perforation occurring in an area where there was a collection of dilated submucosal blood vessels consistent with an AVM associated with a marked thinning of the muscularis propria.” To our knowledge, there has never been a report of an intestinal AVM causing perforation in a neonate. Therefore, we believe that this report represents the first description of a gastrointestinal arteriovenous malformation resulting in intestinal perforation in a premature infant. The reason that the AVM led to intestinal perforation in this child is not clear. One possibility is that the perforation was initiated by acute hemorrhage of the AVM. There was hemorrhage noted microscopically in
MUNN ET AL
Fig 2. Extreme thinning of muscularis propria subjacent to rubmucossl AVM (H&E, original magnification x 100).
the lesion and preoperative coagulation studies disclosed a mild coagulopathy. The acute hemorrhage could have weakened the intestinal wall and led to rupture of the ileum. Another possibility could be that
Fig 3. High power of area seen in Fig 2 (H&E. original magnification x400).
ILEAL PERFORATION SECONDARY TO AVM
703
there was thinning or absence of the muscle layers of the intestine in the area of the AVM. This could have resulted in an area of inherent weakness in the intestinal wall. Rare cases of absence of intestinal wall musculature in neonates have been described.‘3“5 How-
ever, none of these cases were associated with AVM and in all of the cases there was circumferential absence of muscle over variable lengths of bowel. Most of these children presented with obstruction and none had perforation.
REFERENCES 1. Bell MJ: Perforation of the gastrointestinal tract and peritonitis in the neonate. Surg Gynecol Obstet 160:20-26, 1985 2. Wayne ER, Burrington JD, Hutter J: Neonatal nectrotizing enterocolitis. Arch Surg 110:476-480, 1975 3. Emanuel B, Zlotniv P, Raffensperger JG: Perforation of the intestinal tract in infancy and childhood. Surg Gynecol Obstet 146:926-929,1978 4. Thelander HE: Perforation of the gastrointestinal tract of the newborn infant. Am J Dis Child 58:371-393, 1939 5. Cruze K, Snyder WH: Acute perforation of the alimentary tract in infancy and childhood. Ann Surg 154:93-99,196l 6. Garland JS, Nelson DB, Rice T, et al: Increased risk of gastrointestinal perforations in neonates mechanically ventilated with either face mask or nasal prongs. Pediatrics 76:406-410, 1973 7. Lloyd JR: The etiology of gastrointestinal perforations in the newborn. J Pediatr Surg 4:77-84, 1969 8. Parrish RA, Sherman RT, Wilson H: Spontaneous rupture of the gastroenteric tract in the newborn. Ann Surg 159:244-251, 1964 9. Knudson RP, Alden ER: Symptomatic arteriovenous malfor-
mation in infants less than 6 months of age. Pediatrics 64:238-240, 1979 10. Moore JD, Thompson NW, Appelman HD, et al: Arteriovenous malformations of the gastrointestinal tract. Arch Surg 111:381-389, 1976 11. Richardson JD, Max MH, Flint LM, et al: Bleeding vascular malformations of the intestines. Surgery 84:430-436, 1978 12. Graivier L: Ileal stenosis due to arteriovenous malformation in a newborn infant. J Pediatr Surg 17:78-79, 1982 13. Steiner DH, Maxwell JG, Rasmussen BL, et al: Segmental absence of intestinal musculature: An unusual cause of intestinal obstruction in the neonate. Am J Surg 118:964-967, 1969 14. Carroll RL Jr: Absence of musculature of the distal ileum: A cause of neonatal intestinal obstruction. J Pediatr Surg 8:29-31, 1973 15. Dhall JC, Khatri HL, Jaiswal TS, et al: Congenital segmental absence of intestinal musculature: A rare cause of intestinal obstruction in a neonate. Am J Gastroenterol70:401-403, 1978
