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Gastrointestinal perforation in infants
136
group indicated that in children >3 months of age, both an antireflux operation and a Ladd procedure were often required to prevent GER when an intestinal malrotation was present. The Nissen operation alone can be dangerous and prone to failure in the presence of incomplete gastric outlet obstruction.--John D. Orr Assessment of Injection Sclerotherapy in the Management of 152 Children With Oesophageal Varices. E.R. Howard, M.D. Stringer,
andA.P. Mowat. Br J Surg 75:404-408, (May), 1988. The authors report the long-term follow-up of 152 children with esophageal varices undergoing endoscopic sclerotherapy since 1979. One hundred eight of the children presented with variceal bleeding that was managed by injection sclerotherapy. Variceal obliteration was achieved in 92% of the children with extrahepatic portal hypertension, and in 75% of those with intrahepatic portal hypertension. Prophylactic injection sclerotherapy was also used to obliterate large varices in 11 children with no history of hemorrhage. Although bleeding episodes occurred before complete variceal obliteration was achieved, the mortality rate from variceal bleeding was only 1%. The major complications were those of esophageal ulceration and stricture, both of which resolved with conservative management. Recurrent varices occurred in 12% of patients, with rebleeding in 9%. All responded successfully to a second course of treatment. It is concluded that endoscopic sclerotherapy is the treatment of choice for the primary management of esophageal varices in c h i l d r e n . John D. Orr Gastropancreatic Duplications. S. Soundararajan and T.K. Subramaniam. Pediatr Surg Internat 3:288-289, (May), 1988.
The authors report two cases of dual duplication of the stomach and pancreas. They suggest that these occur as a result of defective rotation of the ventral anlage of the pancreas.--Prem Puri Spontaneous Rupture of the Stomach--Low Insertion of the Esophagus on the Lesser Curvature as a Predisposing Factor.
P. Juul and S.A. Pedersen. Pediatr Surg Internat 3:283-284, (May), 1988. The authors report an unusual case in which pneumatic gastric distension due to intake of soda, in association with an abnormally low insertion of the esophagus on the lesser curvature, led to mechanical disruption of the stomach in an 11-year-old girl.--Prem Puri Sonographic Diagnosis of Hypertrophic Pyloric Stenosis. J.D.
Blumhagen, L. Maclin, and D. Krauter. A J R 150:1367-1370, (June), 1988. Many centers have come to prefer the use of ultrasound over the upper gastrointestinal (GI) series if an imaging modality is needed to make the diagnosis of hypertrophic pyloric stenosis. The authors attempted to further analyze the sonographic criteria that are used to determine a positive or negative examination. Retrospectively, they reviewed 323 examinations that had been performed to evaluate the possibility of pyloric stenosis. Of these, 215 had been reported as negative, 107 as positive, and one was suspicious but did not meet the exact criteria for the diagnosis. There were two false-negative studies and no false-positives. The accuracy rate was 99.4%. Independent measurements by four radiologists were made of muscle thickness, muscle length, and channel length. Each significantly correlated with the diagnosis at the P < .01 level. The most reliable of the three measurements is that of muscle thickness. The authors recommend this measurement as the most discriminating and accurate one to use.-- Thomas V. Whalen
INTERNATIONAL ABSTRACTS
Appendicitis in the First Year of Life. A.P. Barker and R.B. Davey.
Aust N Z J Surg 58:491-494, (June), 1988. Thirteen cases of appendicitis in children under the age of 3 years were described from the Adelaide Children's Hospital between 1972 and 1984. This represented an incidence of 1% of all cases of appendicitis. Ages ranged from 16 to 36 months (mean, 25 months). There were eight males and five females. The most common symptoms were vomiting (85%) and abdominal pain (77%). All children were pyrexial (>37~ and evidence of localized or generalized peritonitis was present in 92%. The WBC count was raised in 70% and the abdominal x-ray was abnormal in 84%, suggesting that in patients <3 years of age, these two investigations are important contributors to the diagnosis. The mean time between onset of symptoms and presentation to the hospital was four days, with an additional mean delay time of 24 hours between presentation and appendectomy. Consequently, during the appendicectomy, gangrene with perforation was noted in 70%. The appendicectomy was performed immediately following diagnosis in 12 cases, and in one case, it was delayed. Postoperative complications included wound infection (15%) and bowel obstruction (15%). The authors emphasize the need for a high index of suspicion, a careful history, and a complete examination that may or may not require sedation.-Patricia M. Davidson Use of the Barium Enema in the Diagnosis of Necrotizing Entero-
colitis. P. Uken, W. Smith, E.A. Franken, et al. Pediatr Radiol 18:24-27, (January), 1988.
In an effort to improve diagnostic specificity in necrotizing enterocolitis (NEC), 31 premature neonates who were suspected to have the disease based upon clinical findings underwent a barium enema. The patients did not have clear-cut NEC, but were selected for the ambiguity of their presentation. In 26 of the infants, the examination was interpreted to be normal, and this was considered as ruling out NEC. One of these infants subsequently demonstrated a full clinical picture of the disease. Five infants had a positive study as manifested by ulcerations, intramural extravasation, mucosal irregularity, or persistent spasm. There were no perforations seen as a result of the study. The authors emphasize that the barium enema should not be used in obvious cases of NEC, but advocate its discriminatory use in the ambiguous case.--Thomas V. Whalen Prevention of Necrotizing Enterocolitis in Low-Birth-Weight Infants by IgA-IgG Feeding. M.M. Eibb, H.M. Wolff, H. F~trnkranz,
et al. N Engl J Med 319:1-7, (July 7), 1988. The efficacy of an oral immunoglobulin preparation (73% IgA and 26% IgG) in reducing the incidence of necrotizing enterocolitis (NEC) in infants of low birth weight (for whom no maternal breast milk was available) was studied in a randomized trial. The infants weighed between 800 and 2,000 g. Duration of follow-up was 28 days. There were no cases of N E C among the 88 infants receiving the oral immunoglobulin, as compared with six cases among the 91 control infants ( P = .0143). The authors conclude that the administration of oral immunoglobulin may prevent NEC in infants of low birth weight.--John N. Schullinger Gastrointestinal Perforation in Infants. A.P. Borzotta and D.B.
Groff. Am J Surg 155:477-452, (March), 1988. This series collects data on all infants (< 1 year of age) who were found either during operation or autopsy to have a perforation of the gastrointestinal tract. All patients were neonates except two at 35 and 46 days of age. They, with the rest of the patients in the series were neonatal intensive care unit patients. Most of the patients had necrotizing enterocolitis (NEC). Several had meconium ileus. Of all the infants (29), ten had a patent ductus arteriosus, and all but one
137
INTERNATIONAL ABSTRACTS
died. Overall survival was 59%. Risk with reoperation was felt to be ascribable to the primary disease process and not to the repeat laparotomy itself. Mortality from N E C was no different from that of perforation from other disease processes.--Thomas V. Whalen Intussusception Reduction in Children by Rectal Insufflation of Air.
L. Gu, D.J. Alton, A. Daneman, et al. AJR 150:1345-1348, (June), 1988. This report begins by noting that a textbook published in 1897 recounted the successful reduction of intussusception by use of an ordinary hand bellows. This is followed by a recounting of a prospective study of the use of air insufflation for reduction of intussusception. The initial pressure was set at 80 mmHg, but was carried to 120 mmHg in patients who were not "considered at high risk for perforation." Fluoroscopy was employed. Failure of pneumatic reduction led to an attempt by barium enema. There were 118 intussusceptions identified and entered into the study. Reduction was accomplished in 75%. No patient whose pneumatic reduction was unsuccessful was able to be reduced by the barium enema. Of the 29 patients who were then operated on, 12 were said to be easily reduced, seven were reduced with "great difficulty," and seven required resection. There were three perforations; all were instantly recognized. There were four patients referred to the authors after unsuccessful reduction with a barium enema, who were reduced pneumostatically. The authors also note that the average fluoroscopic time was just 30 seconds, and the average procedure time was five minutes.--Thomas V. Whalen
reduction, with a 50% success. Hydrostatic reduction was requested by the surgeon after careful clinical assessment, and was not attempted where the history was longer than 48 hours or associated with bleeding, peritonism, or perforation. Recurrences were all treated surgically. Bleeding and obstruction were not regarded as contraindications to hydrostatic reduction. Fifty-one patients had a laparotomy; 11 required resection. Only one patient who had a failed hydrostatic reduction required resection. The highest incidence of resection was in infants 3 to 6 months of age with complete obstruction--a group in which hydrostatic reduction is not advised. There was one death from pseudomembranous colitis. The authors recommend hydrostatic reduction of intussusception in selected cases.--W.G. Scobie Individualized Management of Colonic Atresia. E.C. Pohlson, E.L Hatch, P.L. Glick, eta. Am J Surg 155:690-692, (May), 1988.
Eleven patients with colonic atresia were surgically treated over a 5-year period. Two patients had associated gastroschisis, one of whom had associated ileal atresia. Multiple atresias were seen in two patients, and arthrogryposis and malrotation were each seen in one patient. Four patients (one with type I and three with type III atresia) had primary repair and did well. Three deaths occurred in three premature neonates, Each of them had associated anomalies that were the proximate cause of death. Associated conditions, gestational age, and the patient's general condition should be taken into account when planning operative correction of colonic atresia.-Thomas V. Whalen
Pneumatic Reduction of Ileocolic Intussusception in Children. S.G.
Miles, W.A. Cumming, and J.L. Williams. Pediatr Radiol 18:3-5, (January), 1988. While acknowledging that colleagues in Asia and South America have used pneumatic reduction in the therapy of intussusception for many years, the authors ~'eport four cases of use of this technique as a new option in the United States. Each of the four briefly recounted cases had prompt and successful reduction of an ileocolic intussusception. Pressure in the system was regulated so as not to exceed 120 mmHg, and fluoroscopic observation was constantly used. The authors enthusiastically endorse this technique, stating it will now become their preferred method of treatment.--Thomas V. Whalen Comparison of Oxygen and Barium Reduction of Ileocolic Intussusception. E. Phelan, J.F. de Campo, and G. Malecky. A JR 150:1349-
1352, (June), 1988. This Australian hospital treated 57 patients with 61 episodes of intussusception over an 8-month period beginning in late 1986. Six patients were thought to be unacceptable risks for attempted reduction, leaving 55 attempted reductions. The authors used oxygen at a flow rate of 2 L/rain, and at a pressure of 80 mmHg. This is stated to be equivalent to a l-m column of barium. Fluoroscopy was employed and reduction was attempted in a similar fashion as with barium (three attempts of three minutes each). Reduction was successful in 40 of 55 attempts, for a success rate of 73%. This compares quite favorably with the authors' own series of attempted barium reductions (379 attempts over 15 years, with a 53% success rate). There were no perforations during the oxygen enemas. The authors state that oxygen reduction has gained "unanimous acceptance" among the pediatric surgeons at their institution.--Thomas V. Whalen Intussusception: The Case for Barium Reduction. J.H.R. Winstan-
ley, C.M. Doig, and H. Brydon. J R Coil Surg Edinb 32:285-287, (November), 1987. The authors report a 5-year experience with 78 cases of intussusception. Fifty-four patients were treated initially by hydrostatic
Hirschsprung's Disease: Alpha-naphthylesterase Activity for Enzyme-Histochemical Evaluation of the Extent of the Aganglionic Segment During Surgery. P. Dodero and G. Martucciello. Pediatr
Surg Internat 3:269-274, (May), 1988. The authors present their experience with the histotopochemical a-naphthylesterase reaction for intraoperative evaluation of the length of the aganglionic segment in Hirschsprung's disease (HD). The activity of nonspecific esterases was used to demonstrate the presence or absence of intramural ganglion cells in seromuscular intestinal biopsies taken at various levels in 50 patients with the preoperative diagnosis of HD. In 44 cases, the biopsies were taken during a definitive HD operation (endorectal pull-through,' Spare technique). In the remaining six patients, who presented with intestinal obstruction, the biopsies were taken during a laparotomy. The authors consider this histochemical technique a reliable, quick, simple, and economical method for intraoperative evaluation of the length of the aganglionic segment. It is also useful for confirming the diagnosis of HD, and is of help in the study of so-called pseudoHD.--Prem Purl of 4 Cases. C. Janneck and W. Holthusen. Z Kinderchir 43:112-116, (April), 1988.
The Currarino Triad--Review
Four cases of the rare syndrome known as Currarino Triad are reported, consisting of a coincidence between anorectal malformation, presacral mass, and a curved defect of the sacrum (scimitar sacrum). Each congenital or chronic constipation should prompt early radiologic examination of the sacrum and the anorectum. The finding of a scimitar sacrum necessitates a contrast enema and a CT scan in the patient and his family, since autosomal inheritance is known in 50% of the Currarino Triad. The surgical problems are discussed. It is emphasized that a careful division of the rectal and spinal tissues must be achieved to prevent a dangerous and lifethreatening infection of the meningeal sac.--Thomas A. Angerpointner
group indicated that in children >3 months of age, both an antireflux operation and a Ladd procedure were often required to prevent GER when an intestinal malrotation was present. The Nissen operation alone can be dangerous and prone to failure in the presence of incomplete gastric outlet obstruction.--John D. Orr Assessment of Injection Sclerotherapy in the Management of 152 Children With Oesophageal Varices. E.R. Howard, M.D. Stringer,
andA.P. Mowat. Br J Surg 75:404-408, (May), 1988. The authors report the long-term follow-up of 152 children with esophageal varices undergoing endoscopic sclerotherapy since 1979. One hundred eight of the children presented with variceal bleeding that was managed by injection sclerotherapy. Variceal obliteration was achieved in 92% of the children with extrahepatic portal hypertension, and in 75% of those with intrahepatic portal hypertension. Prophylactic injection sclerotherapy was also used to obliterate large varices in 11 children with no history of hemorrhage. Although bleeding episodes occurred before complete variceal obliteration was achieved, the mortality rate from variceal bleeding was only 1%. The major complications were those of esophageal ulceration and stricture, both of which resolved with conservative management. Recurrent varices occurred in 12% of patients, with rebleeding in 9%. All responded successfully to a second course of treatment. It is concluded that endoscopic sclerotherapy is the treatment of choice for the primary management of esophageal varices in c h i l d r e n . John D. Orr Gastropancreatic Duplications. S. Soundararajan and T.K. Subramaniam. Pediatr Surg Internat 3:288-289, (May), 1988.
The authors report two cases of dual duplication of the stomach and pancreas. They suggest that these occur as a result of defective rotation of the ventral anlage of the pancreas.--Prem Puri Spontaneous Rupture of the Stomach--Low Insertion of the Esophagus on the Lesser Curvature as a Predisposing Factor.
P. Juul and S.A. Pedersen. Pediatr Surg Internat 3:283-284, (May), 1988. The authors report an unusual case in which pneumatic gastric distension due to intake of soda, in association with an abnormally low insertion of the esophagus on the lesser curvature, led to mechanical disruption of the stomach in an 11-year-old girl.--Prem Puri Sonographic Diagnosis of Hypertrophic Pyloric Stenosis. J.D.
Blumhagen, L. Maclin, and D. Krauter. A J R 150:1367-1370, (June), 1988. Many centers have come to prefer the use of ultrasound over the upper gastrointestinal (GI) series if an imaging modality is needed to make the diagnosis of hypertrophic pyloric stenosis. The authors attempted to further analyze the sonographic criteria that are used to determine a positive or negative examination. Retrospectively, they reviewed 323 examinations that had been performed to evaluate the possibility of pyloric stenosis. Of these, 215 had been reported as negative, 107 as positive, and one was suspicious but did not meet the exact criteria for the diagnosis. There were two false-negative studies and no false-positives. The accuracy rate was 99.4%. Independent measurements by four radiologists were made of muscle thickness, muscle length, and channel length. Each significantly correlated with the diagnosis at the P < .01 level. The most reliable of the three measurements is that of muscle thickness. The authors recommend this measurement as the most discriminating and accurate one to use.-- Thomas V. Whalen
INTERNATIONAL ABSTRACTS
Appendicitis in the First Year of Life. A.P. Barker and R.B. Davey.
Aust N Z J Surg 58:491-494, (June), 1988. Thirteen cases of appendicitis in children under the age of 3 years were described from the Adelaide Children's Hospital between 1972 and 1984. This represented an incidence of 1% of all cases of appendicitis. Ages ranged from 16 to 36 months (mean, 25 months). There were eight males and five females. The most common symptoms were vomiting (85%) and abdominal pain (77%). All children were pyrexial (>37~ and evidence of localized or generalized peritonitis was present in 92%. The WBC count was raised in 70% and the abdominal x-ray was abnormal in 84%, suggesting that in patients <3 years of age, these two investigations are important contributors to the diagnosis. The mean time between onset of symptoms and presentation to the hospital was four days, with an additional mean delay time of 24 hours between presentation and appendectomy. Consequently, during the appendicectomy, gangrene with perforation was noted in 70%. The appendicectomy was performed immediately following diagnosis in 12 cases, and in one case, it was delayed. Postoperative complications included wound infection (15%) and bowel obstruction (15%). The authors emphasize the need for a high index of suspicion, a careful history, and a complete examination that may or may not require sedation.-Patricia M. Davidson Use of the Barium Enema in the Diagnosis of Necrotizing Entero-
colitis. P. Uken, W. Smith, E.A. Franken, et al. Pediatr Radiol 18:24-27, (January), 1988.
In an effort to improve diagnostic specificity in necrotizing enterocolitis (NEC), 31 premature neonates who were suspected to have the disease based upon clinical findings underwent a barium enema. The patients did not have clear-cut NEC, but were selected for the ambiguity of their presentation. In 26 of the infants, the examination was interpreted to be normal, and this was considered as ruling out NEC. One of these infants subsequently demonstrated a full clinical picture of the disease. Five infants had a positive study as manifested by ulcerations, intramural extravasation, mucosal irregularity, or persistent spasm. There were no perforations seen as a result of the study. The authors emphasize that the barium enema should not be used in obvious cases of NEC, but advocate its discriminatory use in the ambiguous case.--Thomas V. Whalen Prevention of Necrotizing Enterocolitis in Low-Birth-Weight Infants by IgA-IgG Feeding. M.M. Eibb, H.M. Wolff, H. F~trnkranz,
et al. N Engl J Med 319:1-7, (July 7), 1988. The efficacy of an oral immunoglobulin preparation (73% IgA and 26% IgG) in reducing the incidence of necrotizing enterocolitis (NEC) in infants of low birth weight (for whom no maternal breast milk was available) was studied in a randomized trial. The infants weighed between 800 and 2,000 g. Duration of follow-up was 28 days. There were no cases of N E C among the 88 infants receiving the oral immunoglobulin, as compared with six cases among the 91 control infants ( P = .0143). The authors conclude that the administration of oral immunoglobulin may prevent NEC in infants of low birth weight.--John N. Schullinger Gastrointestinal Perforation in Infants. A.P. Borzotta and D.B.
Groff. Am J Surg 155:477-452, (March), 1988. This series collects data on all infants (< 1 year of age) who were found either during operation or autopsy to have a perforation of the gastrointestinal tract. All patients were neonates except two at 35 and 46 days of age. They, with the rest of the patients in the series were neonatal intensive care unit patients. Most of the patients had necrotizing enterocolitis (NEC). Several had meconium ileus. Of all the infants (29), ten had a patent ductus arteriosus, and all but one
137
INTERNATIONAL ABSTRACTS
died. Overall survival was 59%. Risk with reoperation was felt to be ascribable to the primary disease process and not to the repeat laparotomy itself. Mortality from N E C was no different from that of perforation from other disease processes.--Thomas V. Whalen Intussusception Reduction in Children by Rectal Insufflation of Air.
L. Gu, D.J. Alton, A. Daneman, et al. AJR 150:1345-1348, (June), 1988. This report begins by noting that a textbook published in 1897 recounted the successful reduction of intussusception by use of an ordinary hand bellows. This is followed by a recounting of a prospective study of the use of air insufflation for reduction of intussusception. The initial pressure was set at 80 mmHg, but was carried to 120 mmHg in patients who were not "considered at high risk for perforation." Fluoroscopy was employed. Failure of pneumatic reduction led to an attempt by barium enema. There were 118 intussusceptions identified and entered into the study. Reduction was accomplished in 75%. No patient whose pneumatic reduction was unsuccessful was able to be reduced by the barium enema. Of the 29 patients who were then operated on, 12 were said to be easily reduced, seven were reduced with "great difficulty," and seven required resection. There were three perforations; all were instantly recognized. There were four patients referred to the authors after unsuccessful reduction with a barium enema, who were reduced pneumostatically. The authors also note that the average fluoroscopic time was just 30 seconds, and the average procedure time was five minutes.--Thomas V. Whalen
reduction, with a 50% success. Hydrostatic reduction was requested by the surgeon after careful clinical assessment, and was not attempted where the history was longer than 48 hours or associated with bleeding, peritonism, or perforation. Recurrences were all treated surgically. Bleeding and obstruction were not regarded as contraindications to hydrostatic reduction. Fifty-one patients had a laparotomy; 11 required resection. Only one patient who had a failed hydrostatic reduction required resection. The highest incidence of resection was in infants 3 to 6 months of age with complete obstruction--a group in which hydrostatic reduction is not advised. There was one death from pseudomembranous colitis. The authors recommend hydrostatic reduction of intussusception in selected cases.--W.G. Scobie Individualized Management of Colonic Atresia. E.C. Pohlson, E.L Hatch, P.L. Glick, eta. Am J Surg 155:690-692, (May), 1988.
Eleven patients with colonic atresia were surgically treated over a 5-year period. Two patients had associated gastroschisis, one of whom had associated ileal atresia. Multiple atresias were seen in two patients, and arthrogryposis and malrotation were each seen in one patient. Four patients (one with type I and three with type III atresia) had primary repair and did well. Three deaths occurred in three premature neonates, Each of them had associated anomalies that were the proximate cause of death. Associated conditions, gestational age, and the patient's general condition should be taken into account when planning operative correction of colonic atresia.-Thomas V. Whalen
Pneumatic Reduction of Ileocolic Intussusception in Children. S.G.
Miles, W.A. Cumming, and J.L. Williams. Pediatr Radiol 18:3-5, (January), 1988. While acknowledging that colleagues in Asia and South America have used pneumatic reduction in the therapy of intussusception for many years, the authors ~'eport four cases of use of this technique as a new option in the United States. Each of the four briefly recounted cases had prompt and successful reduction of an ileocolic intussusception. Pressure in the system was regulated so as not to exceed 120 mmHg, and fluoroscopic observation was constantly used. The authors enthusiastically endorse this technique, stating it will now become their preferred method of treatment.--Thomas V. Whalen Comparison of Oxygen and Barium Reduction of Ileocolic Intussusception. E. Phelan, J.F. de Campo, and G. Malecky. A JR 150:1349-
1352, (June), 1988. This Australian hospital treated 57 patients with 61 episodes of intussusception over an 8-month period beginning in late 1986. Six patients were thought to be unacceptable risks for attempted reduction, leaving 55 attempted reductions. The authors used oxygen at a flow rate of 2 L/rain, and at a pressure of 80 mmHg. This is stated to be equivalent to a l-m column of barium. Fluoroscopy was employed and reduction was attempted in a similar fashion as with barium (three attempts of three minutes each). Reduction was successful in 40 of 55 attempts, for a success rate of 73%. This compares quite favorably with the authors' own series of attempted barium reductions (379 attempts over 15 years, with a 53% success rate). There were no perforations during the oxygen enemas. The authors state that oxygen reduction has gained "unanimous acceptance" among the pediatric surgeons at their institution.--Thomas V. Whalen Intussusception: The Case for Barium Reduction. J.H.R. Winstan-
ley, C.M. Doig, and H. Brydon. J R Coil Surg Edinb 32:285-287, (November), 1987. The authors report a 5-year experience with 78 cases of intussusception. Fifty-four patients were treated initially by hydrostatic
Hirschsprung's Disease: Alpha-naphthylesterase Activity for Enzyme-Histochemical Evaluation of the Extent of the Aganglionic Segment During Surgery. P. Dodero and G. Martucciello. Pediatr
Surg Internat 3:269-274, (May), 1988. The authors present their experience with the histotopochemical a-naphthylesterase reaction for intraoperative evaluation of the length of the aganglionic segment in Hirschsprung's disease (HD). The activity of nonspecific esterases was used to demonstrate the presence or absence of intramural ganglion cells in seromuscular intestinal biopsies taken at various levels in 50 patients with the preoperative diagnosis of HD. In 44 cases, the biopsies were taken during a definitive HD operation (endorectal pull-through,' Spare technique). In the remaining six patients, who presented with intestinal obstruction, the biopsies were taken during a laparotomy. The authors consider this histochemical technique a reliable, quick, simple, and economical method for intraoperative evaluation of the length of the aganglionic segment. It is also useful for confirming the diagnosis of HD, and is of help in the study of so-called pseudoHD.--Prem Purl of 4 Cases. C. Janneck and W. Holthusen. Z Kinderchir 43:112-116, (April), 1988.
The Currarino Triad--Review
Four cases of the rare syndrome known as Currarino Triad are reported, consisting of a coincidence between anorectal malformation, presacral mass, and a curved defect of the sacrum (scimitar sacrum). Each congenital or chronic constipation should prompt early radiologic examination of the sacrum and the anorectum. The finding of a scimitar sacrum necessitates a contrast enema and a CT scan in the patient and his family, since autosomal inheritance is known in 50% of the Currarino Triad. The surgical problems are discussed. It is emphasized that a careful division of the rectal and spinal tissues must be achieved to prevent a dangerous and lifethreatening infection of the meningeal sac.--Thomas A. Angerpointner