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Appendiceal perforation: A potentially lethal initial mode of presentation of Hirschsprung's disease
Appendiceal Perforation: A Potentially Lethal Initial Mode of Presentation of Hirschsprung’s Disease By Aklle Sario(jlu,
F. Cahit Tanyel,
Nebil Ankara,
0 In Hacettepe University Children’s Hospital, between 1976 and 1993 two patients among 302 with Hirschsprung’s disease were diagnosed with appendiceal perforation (API at initial admission. Both patients were less than 2 months of age. One of them was a boy with total colonic aganglionosis and the latter a girl with long-segment disease. In both cases the site of AP was the base, and periappendicitis without mucosal involvement was detected. The present cases and review of the literature suggest that longer aganglionic segment carries a higher risk of AP. Copyright o 1997 by W.B. Saunders Company INDEX tion.
WORDS:
Hirschsprung’s
disease,
appendiceal
perfora-
and Akgiin
HiGsdnmez
Case 2 A 27-day-old girl presented with abdominal distention, fever, convulsions, sepsis, hyponatremia, and hyperpotassemia. The passage of meconium was delayed for 2 days. Fluid and electrolyte replacement, nasogastric decompression and antibiotic treatment were given. Plain abdominal radiograph and barium enema results were suggestive of intestinal obstruction and Hirschsprung’s disease, respectively. She underwent surgery, and appendiceal perforation at the base of the appendix and HD were detected. The aganglionic segment extended to the descending colon. Appendectomy and transverse colostomy were performed. The pathological examination of the specimen showed penappendicitis without involvement of mucosal layer. She died of sepsis 7 days after the operation.
DISCUSSION
H
IRSCHSPRUNG’S DISEASE (IjD) is one of the common causes of intestinal obstruction in the newborn period.’ Although appendiceal perforation causes symptoms of intestinal obstruction in the newborn period, this is one of the least likely modes of initial presentation of HD.2,3 However, because of the funnel-shaped structure of the appendix in the newborn period, appendicitis is very rare, and is frequently diagnosed after perforation.4s5 If neonatal appendicitis is encountered, HD, cystic fibrosis, and necrotizing enterocolitis should be ruled out.6 However, there are limited number of reports about the appendiceal perforation associated with HD. Two patients with appendiceal perforations among 302 patients with HD who were admitted between 1976 and 1993, form the basis of this report.
CASE
REPORTS
Case I A 40-day-old boy was admitted with the symptoms of poor feeding, vomiting, and abdominal distension. He did not pass stool for the past 4 days. He was dehydrated and malnourished. He had signs and symptoms of mechanical intestinal obstruction on both physical examination and plain abdominal x-ray. Barium enema showed an unused colon suggesting total colonic aganglionosis. Followmg fluid resuscitation, and initiation of broad spectrum antibiotics he underwent a laparotomy. During laparotomy appendiceal perforation at the base of the appendix was detected. The ileum and jejunum were dilated, but he had a narrow colon. Because the frozen section of the appendectomy specimen and colon were aganglionic, and that of the terminal ileum showed normal ganglion cells, ileostomy, and appendectomy were performed. The pathology specimen of the appendix showed perlappendicitis w&out mvolvement of mucosal layer. He had severe respiratory distress after the operation and did not respond to resuscitation. He died a day later. Necropsy findings included atelectasis of both lungs, tracheitls. brain edema, total colonic aganglionosis, enterocolitis, peritonitis, and malnutrition. JournalofPediatricSurgery,
Biiytikpamukr$u, Turkey
Vol 32. No 1 (January),
1997: pp 123-124
Although it is a well-known association, appendiceal perforation caused by Hirschsprung’s disease is one of the rare modes of initial presentation, with only 10 cases described in the literature (Table 1).2,3*7-”In the present series only 0.66% of patients were admitted with this rare mode of presentation. Although HD is mentioned to be ruled out among patients with perforated appendicitis, some features unique to HD suggest this entity to be different from perforated appendicitis. In perforated appendicitis luminal obstruction is mandatory. Inflammation begins from mucosa toward serosa and perforation usually occurs at the tip. However, evaluation of present cases and literature shows that in appendiceal perforation associated with HD, the mucosal infiltration is minimal, and periappendicitis is more severe. Therefore, instead of evaluating under the title of complicated appendicitis, Hirschsprung-associated appendiceal perforations should be considered as a different entity. Most of the patients with HD and appendiceal perforation association are neonates, with the oldest reported as 4 months of age.2 Appendiceal perforation is encountered at the initial admission of patients. Among 10 patients, six were reported to be boys. 2,3~8,9 Addition of a boy and a girl makes the total number twelve. The extent of the aganglionic segment has been total colonic in five, long-segment in five, and short-segment in one. It was unknown in the remaining patient. These data suggest
From the Department of Pediatric Surgec. Faculty of Medicme, Hacettepe University, Ankara, Turkey. Address reprint requests to l? Cahit Tanyel, MD, Hacettepe University Faculty of Medicine, Department of Pediatric Surgery, 06100, Samanpazan. Ankara, Turkey. Copyright 0 1997 by WB. Saunders Company 0022-3468/97/3201-0038$03.00/O
123
124
SARIOGLU
Table 1. Appendiceal Study 7d
Disease Extent of Aganglionic Segment
Outcome
kot recorded Transverse colon
Not recorded Alive
Male
At the base Midportion
Splenic
Died
Id 15d
Martin
et al8
33d 11 d
Male Male
Asch et allo
Not recorded Not recorded
Not recorded Not recorded
Swenson Newman
Neonatal 4mo
Arliss et al3 Present report Present
report
Hhschsprung’s
Not recorded At the base
et al8 et al*
et all1 et al*
with
Not recorded Male
Martin Martin
Gastrin et al9 Asch et allo
in Patients
sne of Appendiceal Perforation
Sex
Age
Wyllie’
Perforations
flexure
At the apex
Splenicflexure Distal half of colon
Died Alive
Not recorded
Total colonic
Not recorded
Not recorded Male
Not recorded Not recorded Not recorded
Total colonic Total colonic Total colonic
Not recorded Not recorded Alive
7d 40d
Male Male
At the base At the base
Alive Died
27 d
Female
At the base
Rectosigmoid Total colonic Descending colon
that the length of aganglionic segment may have a role, and longer aganglionic segment carries a higher risk. The constancy of multiplication of pressure and volume may explain the increase of risk. Because aganglionic segment cannot dilate, intracolonic volume should decrease while the length of aganglionic segment increases. If the ileocecal valve is competent, intracolonic pressure may increase more rapidly in smaller intracolonic volumes, and make the appendix more prone to perforation. The reasons for predilection of appendix as a site of perforation is not clear. The appendix is cone shaped in the neonatal period with a wide appendiceal connection to the cecum.4 The other difference between the appendix of a baby and that of the adult is the fact that although the Gerlach’s fold of adults does not have a muscular layer, the babies appendix usually contains it.4 This might function as a check-valve trapping air and meconium, which in turn might result in perforation. It is also noted that the circular muscle is thickened on the ileal side, of the appendicocecal union whereas no such thickening is present on the lateral side.4 The tenia coli are also shown
ET AL
Died
to take various courses about the base of appendix. Although in some’cases they surround the appendix like a diaphragm or in a Y-shaped arrangement, in some cases this decussation of the fibers of tenia is missing. Arliss et al3 applied Laplace’s law to those cases with appendiceal perforation together with Hirschsprung’s disease to explain the site of perforation without inflammation, and proved that it should be at the base of the appendix. If Laplace’s law and the possibility of a thinner anatomic area at the base of the appendix are combined, the perforation site at the base of appendix can be explained. But there are cases with midportion and apical perforation as we11.8,9Therefore, the proposed mechanisms for appendiceal predilection still remain speculative. The mortality associated with HD seems to be higher among patients admitted With appendiceal perforations. In both of the presented cases and in two cases in the literature, the course of this association has been lethal. Appendiceal perforation in the newborn period is the rare but potentially lethal initial presentation of Hirschsprung’s disease.
REFERENCES 1. Swenson 0, Davidson FZ: Similarities of mechanical intestinal obstruction and aganglionic megacolon in the newborn infant: Areview of 64 cases. N Engl J Med 262&I-67,1960 2. Newman B, Nussbaum A, Kirkpatrick JA, et al: Appendiceal perforation, pneumoperitoneum, and Hirschsprung’s disease. J Pediatr Surg 23:854-856,198s 3. Arliss J, Holgersen LO: Neonatal appendiceal perforation and Hirschsprung’s disease. J Pediatr Surg 25:694-695,199O 4. Wangenstein OH, Buirge RE, Dennis C. et al: Studies on the etiology of acute appendicitis. Ann Surg 106:910-942, 1937 5. Shaupp W, Clausen EG, Ferrier PR: Appendicitis during the first month of life. Surgery 48:805-811, 1960
6. Grosfeld JL, Weinberger M, Clatworthy W: Acute appendicitis in the first two years of life. J Pediatr Surg 8285293, 1973 7. Wyllie GG: Course and management of Hirschsprung’s disease. Laricet 2:847-855, 1957 8. Martin LW, Perrin EV: Neonatal perforation of the appendix in association with Hirschsprung’s disease. AM Surg 166:799-802, 1967 9. Gastrin U, Josephson S: Appendiceal peritonitis and megacolon in the neonatal period. Acta Chir Stand 136:153-155.1970 10. Asch MJ, Weitzmann JJ, Hays DM, et al: Total colon aganglionosis. Arch Surg 105:74-78, 1972 11. Swenson 0, Sherman JO, Fisher JH: Diagnosis of congenital megacolon: An analysis of 501 patients. J Pediatr Surg 8587-594, 1973
F. Cahit Tanyel,
Nebil Ankara,
0 In Hacettepe University Children’s Hospital, between 1976 and 1993 two patients among 302 with Hirschsprung’s disease were diagnosed with appendiceal perforation (API at initial admission. Both patients were less than 2 months of age. One of them was a boy with total colonic aganglionosis and the latter a girl with long-segment disease. In both cases the site of AP was the base, and periappendicitis without mucosal involvement was detected. The present cases and review of the literature suggest that longer aganglionic segment carries a higher risk of AP. Copyright o 1997 by W.B. Saunders Company INDEX tion.
WORDS:
Hirschsprung’s
disease,
appendiceal
perfora-
and Akgiin
HiGsdnmez
Case 2 A 27-day-old girl presented with abdominal distention, fever, convulsions, sepsis, hyponatremia, and hyperpotassemia. The passage of meconium was delayed for 2 days. Fluid and electrolyte replacement, nasogastric decompression and antibiotic treatment were given. Plain abdominal radiograph and barium enema results were suggestive of intestinal obstruction and Hirschsprung’s disease, respectively. She underwent surgery, and appendiceal perforation at the base of the appendix and HD were detected. The aganglionic segment extended to the descending colon. Appendectomy and transverse colostomy were performed. The pathological examination of the specimen showed penappendicitis without involvement of mucosal layer. She died of sepsis 7 days after the operation.
DISCUSSION
H
IRSCHSPRUNG’S DISEASE (IjD) is one of the common causes of intestinal obstruction in the newborn period.’ Although appendiceal perforation causes symptoms of intestinal obstruction in the newborn period, this is one of the least likely modes of initial presentation of HD.2,3 However, because of the funnel-shaped structure of the appendix in the newborn period, appendicitis is very rare, and is frequently diagnosed after perforation.4s5 If neonatal appendicitis is encountered, HD, cystic fibrosis, and necrotizing enterocolitis should be ruled out.6 However, there are limited number of reports about the appendiceal perforation associated with HD. Two patients with appendiceal perforations among 302 patients with HD who were admitted between 1976 and 1993, form the basis of this report.
CASE
REPORTS
Case I A 40-day-old boy was admitted with the symptoms of poor feeding, vomiting, and abdominal distension. He did not pass stool for the past 4 days. He was dehydrated and malnourished. He had signs and symptoms of mechanical intestinal obstruction on both physical examination and plain abdominal x-ray. Barium enema showed an unused colon suggesting total colonic aganglionosis. Followmg fluid resuscitation, and initiation of broad spectrum antibiotics he underwent a laparotomy. During laparotomy appendiceal perforation at the base of the appendix was detected. The ileum and jejunum were dilated, but he had a narrow colon. Because the frozen section of the appendectomy specimen and colon were aganglionic, and that of the terminal ileum showed normal ganglion cells, ileostomy, and appendectomy were performed. The pathology specimen of the appendix showed perlappendicitis w&out mvolvement of mucosal layer. He had severe respiratory distress after the operation and did not respond to resuscitation. He died a day later. Necropsy findings included atelectasis of both lungs, tracheitls. brain edema, total colonic aganglionosis, enterocolitis, peritonitis, and malnutrition. JournalofPediatricSurgery,
Biiytikpamukr$u, Turkey
Vol 32. No 1 (January),
1997: pp 123-124
Although it is a well-known association, appendiceal perforation caused by Hirschsprung’s disease is one of the rare modes of initial presentation, with only 10 cases described in the literature (Table 1).2,3*7-”In the present series only 0.66% of patients were admitted with this rare mode of presentation. Although HD is mentioned to be ruled out among patients with perforated appendicitis, some features unique to HD suggest this entity to be different from perforated appendicitis. In perforated appendicitis luminal obstruction is mandatory. Inflammation begins from mucosa toward serosa and perforation usually occurs at the tip. However, evaluation of present cases and literature shows that in appendiceal perforation associated with HD, the mucosal infiltration is minimal, and periappendicitis is more severe. Therefore, instead of evaluating under the title of complicated appendicitis, Hirschsprung-associated appendiceal perforations should be considered as a different entity. Most of the patients with HD and appendiceal perforation association are neonates, with the oldest reported as 4 months of age.2 Appendiceal perforation is encountered at the initial admission of patients. Among 10 patients, six were reported to be boys. 2,3~8,9 Addition of a boy and a girl makes the total number twelve. The extent of the aganglionic segment has been total colonic in five, long-segment in five, and short-segment in one. It was unknown in the remaining patient. These data suggest
From the Department of Pediatric Surgec. Faculty of Medicme, Hacettepe University, Ankara, Turkey. Address reprint requests to l? Cahit Tanyel, MD, Hacettepe University Faculty of Medicine, Department of Pediatric Surgery, 06100, Samanpazan. Ankara, Turkey. Copyright 0 1997 by WB. Saunders Company 0022-3468/97/3201-0038$03.00/O
123
124
SARIOGLU
Table 1. Appendiceal Study 7d
Disease Extent of Aganglionic Segment
Outcome
kot recorded Transverse colon
Not recorded Alive
Male
At the base Midportion
Splenic
Died
Id 15d
Martin
et al8
33d 11 d
Male Male
Asch et allo
Not recorded Not recorded
Not recorded Not recorded
Swenson Newman
Neonatal 4mo
Arliss et al3 Present report Present
report
Hhschsprung’s
Not recorded At the base
et al8 et al*
et all1 et al*
with
Not recorded Male
Martin Martin
Gastrin et al9 Asch et allo
in Patients
sne of Appendiceal Perforation
Sex
Age
Wyllie’
Perforations
flexure
At the apex
Splenicflexure Distal half of colon
Died Alive
Not recorded
Total colonic
Not recorded
Not recorded Male
Not recorded Not recorded Not recorded
Total colonic Total colonic Total colonic
Not recorded Not recorded Alive
7d 40d
Male Male
At the base At the base
Alive Died
27 d
Female
At the base
Rectosigmoid Total colonic Descending colon
that the length of aganglionic segment may have a role, and longer aganglionic segment carries a higher risk. The constancy of multiplication of pressure and volume may explain the increase of risk. Because aganglionic segment cannot dilate, intracolonic volume should decrease while the length of aganglionic segment increases. If the ileocecal valve is competent, intracolonic pressure may increase more rapidly in smaller intracolonic volumes, and make the appendix more prone to perforation. The reasons for predilection of appendix as a site of perforation is not clear. The appendix is cone shaped in the neonatal period with a wide appendiceal connection to the cecum.4 The other difference between the appendix of a baby and that of the adult is the fact that although the Gerlach’s fold of adults does not have a muscular layer, the babies appendix usually contains it.4 This might function as a check-valve trapping air and meconium, which in turn might result in perforation. It is also noted that the circular muscle is thickened on the ileal side, of the appendicocecal union whereas no such thickening is present on the lateral side.4 The tenia coli are also shown
ET AL
Died
to take various courses about the base of appendix. Although in some’cases they surround the appendix like a diaphragm or in a Y-shaped arrangement, in some cases this decussation of the fibers of tenia is missing. Arliss et al3 applied Laplace’s law to those cases with appendiceal perforation together with Hirschsprung’s disease to explain the site of perforation without inflammation, and proved that it should be at the base of the appendix. If Laplace’s law and the possibility of a thinner anatomic area at the base of the appendix are combined, the perforation site at the base of appendix can be explained. But there are cases with midportion and apical perforation as we11.8,9Therefore, the proposed mechanisms for appendiceal predilection still remain speculative. The mortality associated with HD seems to be higher among patients admitted With appendiceal perforations. In both of the presented cases and in two cases in the literature, the course of this association has been lethal. Appendiceal perforation in the newborn period is the rare but potentially lethal initial presentation of Hirschsprung’s disease.
REFERENCES 1. Swenson 0, Davidson FZ: Similarities of mechanical intestinal obstruction and aganglionic megacolon in the newborn infant: Areview of 64 cases. N Engl J Med 262&I-67,1960 2. Newman B, Nussbaum A, Kirkpatrick JA, et al: Appendiceal perforation, pneumoperitoneum, and Hirschsprung’s disease. J Pediatr Surg 23:854-856,198s 3. Arliss J, Holgersen LO: Neonatal appendiceal perforation and Hirschsprung’s disease. J Pediatr Surg 25:694-695,199O 4. Wangenstein OH, Buirge RE, Dennis C. et al: Studies on the etiology of acute appendicitis. Ann Surg 106:910-942, 1937 5. Shaupp W, Clausen EG, Ferrier PR: Appendicitis during the first month of life. Surgery 48:805-811, 1960
6. Grosfeld JL, Weinberger M, Clatworthy W: Acute appendicitis in the first two years of life. J Pediatr Surg 8285293, 1973 7. Wyllie GG: Course and management of Hirschsprung’s disease. Laricet 2:847-855, 1957 8. Martin LW, Perrin EV: Neonatal perforation of the appendix in association with Hirschsprung’s disease. AM Surg 166:799-802, 1967 9. Gastrin U, Josephson S: Appendiceal peritonitis and megacolon in the neonatal period. Acta Chir Stand 136:153-155.1970 10. Asch MJ, Weitzmann JJ, Hays DM, et al: Total colon aganglionosis. Arch Surg 105:74-78, 1972 11. Swenson 0, Sherman JO, Fisher JH: Diagnosis of congenital megacolon: An analysis of 501 patients. J Pediatr Surg 8587-594, 1973