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Neonatal appendiceal perforation and Hirschsprung's disease
Neonatal
Appendiceal
Perforation
and Hirschsprung’s
Disease
By Jeffrey Arliss and Leif 0. Holgersen New York, New 9 The case of a boy infant with neonatal appendiceal perforation who presented at 9 years of age with Hirschsprung’s disease is reported. A retrospective review of the microscopic sections of the appendix demonstrated periappendicitis without transmural inflammation. The importance of differentiating between true appendicitis and periappendicitis in a neonate with appendiceal perforation is stressed, in order to avoid a missed diagnosis of Hirschsprung’s disease. @ 1990 by W.B. Saunders Company.
York
tion, and the diagnosis was confirmed by rectal biopsy. Four weeks following a sigmoid colostomy, a Duhamel pull-through was performed. Review of the microscopic sections of the appendix removed as a neonate showed periappendicitis with no evidence of transmural inflammation. DISCUSSION
From the Section of Pediatric Surgery, Department of Surgery, St LukeS-Roosevelt Hospital Center, New York. NY. Address reprint requests to Lxif 0. Holgersen. MD. St Luke’sRoosevelt Hospital Center, Amsterdam Ave and 114th St, New York, NY 10025. o 1990 by W.B. Saunders Company. 0022-3468/90/2506-0030$03.00/0
Martin and Perrin* reported three cases of appendiceal perforation in neonates in whom Hirschsprung’s disease was later confirmed. In two of the three patients there was only minimal evidence of gross inflammation of the appendix. The perforation occured at the base of the appendix in two patients and the midportion in one. An additional report by Gastrin and Josephson3 described a neonate with an apical appendiceal perforation in whom Hirschsprung’s disease was diagnosed at 5 years of age. Microscopic sections of the appendix demonstrated periappendicitis. The appendix in the patient described here was perforated at the base and microscopically there was periappendicitis without transmural inflammation. Neonatal appendiceal perforation in the absence of transmural inflammation invites the conclusion that these perforations may be mechanical. The anatomy of the cecum and appendix was classified by Treves, in 1885,5 with the neonatal appendix having a definite funnel-shaped opening into the cecum. This configuration was confirmed by Snyder and Chaffin and Wangenstein et al7 who also demonstrated a sharp decrease in this configuration after 1 year of age. Because the base of the appendix would therefore be incorporated in the cecal circumference in the neonate, in the presence of distal obstruction it would also represent the potential point of maximum tension based on Laplace’s law, where the relationship between pressure (P), tension (T), and radius (R) is expressed as T = V2 PR. This phenomenon might explain the predilection for perforation at the base of the appendix in neonates with Hirschsprung’s disease. However, it does not explain the apical perforation of the appendix described by Gastrin, unless inspissated meconium in the lumen of the appendix was a factor, as in a case of appendiceal perforation and meconium plug syndrome described by Grosfeld et a1.s Inflammatory perforations of the neonatal appendix usually produce apical perforations. In a review of five cases of neonatal appendicitis by Schaupp et al,’ perforation in all five occurred at the apex of the appendix. The mortality in this series was lOO%, and autopsies performed on all
694
Journalof
INDEX WORDS: disease.
Appendicitis,
neonatal; Hirschsprung’s
N
EONATAL appendicitis is rare and apical perforation usually occurs prior to diagnosis.’ The association of neonatal appendiceal perforation with Hirschsprung’s disease has been detailed in two reports of four patients.29 The perforations in these patients occurred in the absence of transmural appendiceal inflammation. A previously published case of a patient with neonatal appendiceal perforation4 who subsequently presented with Hirschsprung’s disease, is the basis for this report. CASE REPORT A 7-day-old boy presented with fever, poor feeding, abdominal
distention, and obstipation for 24 hours. Examination included a plain abdominal x-ray that showed an early partial small bowel obstruction. A barium enema was reported as normal except for retained meconium in the transverse colon. Following nasogastric tube decompression, hydration, and antibiotics the child improved, and a right flank mass was noted. A sonogram and intravenous pyelogram showed right hydronephrosis consistent with a congenital ureteropelvic junction (UPJ) obstruction. The child underwent extraperitoneal repair of the UPJ obstruction at aged 14 days. At surgery, following completion of a right pyeloplasty, the peritoneum was opened. Unexpectedly, a grossly edematous and inflamed appendix was found, with a perforation at the base and a small retrocecal abscess. An appendectomy was performed with inversion and closure of the appendiceal stump. He made an uneventful recovery and at the time of discharge was having five to eight soft yellow stools a day. He was lost to follow-up until 9 years of age when he presented with a history of constipation that had begun 4 weeks following his appendectomy. A barium enema was consistent with Hirschsprung’s disease with a transition at the rectosigmoid junc-
Pediatric Surgery, Vol 25, No 6 (June), 1990: pp 694-695
NEONATAL
APPENDICITIS;
HIRSCHSPRUNG’S
DISEASE
695
neonates did not show any evidence of colon obstruction. In the presence of neonatal appendiceal perforation without the gross appearance of appendicitis, Martin and Perrin stressed appendectomy with exteriorization of the cecum, because the likelihood of coexistent Hirschsprung’s disease was so great. At laporatomy it may not be possible to distinguish between true transmural appendicitis and the periappendiceal reaction that accompanies perforation. The patient we treated and the patient described by Gastrin did well following appendectomy without cecostomy, despite the missed diagnosis of Hirschsprung’s disease. The presence of
five
healthy cecal tissue is probably the more important determinant of whether or not a decompression procedure is necessary. Despite the gross appearance of the appendix, careful microscopic review should be performed on all perforated appendices in the neonate. The absence of transmural inflammation should alert the clinician to the possibility of Hirschsprung’s disease and rectal biopsy should be considered. Transmural appendiceal inflammation has not been a feature in the patients reported with Hirschsprung’s disease and appendiceal perforation, and the implication that there is an association with true neonatal appendicitis and Hirschsprung’s disease should be dispelled.
REFERENCES 1. Schaupp W, Clausen EC, Ferrier PK: Appendicitis during the first month of life. Surgery 48805811, 1960 2. Martin LW, Perrin EV: Neonatal perforation of the appendix in association with Hirschsprung’s disease. Ann Surg 166:799-802, 1967 3. Gastrin U, Josephson S: Appendiceal perforation and megacolon in the neonatal period. Acta Chir Stand 136:153-155, 1970 4. Tucci P, Holgersen LO, Doctor D, et al: Congenital ureteropelvie junction obstruction associated with unsuspected acute perforated appendicitis in a neonate. J Urol 120:247-248, 1978
5. Treves F: The anatomy of the intestinal canal and peritoneum in man. Br Med J 1:470-474, 1885 6. Snyder WH, Chatlin L: Appendicitis during the first two years of life. Arch Surg 64549-560, 1952 7. Wangenstein OH, Buirge RE, Dennis C, et al: Studies in the etiology of acute appendicitis. Ann Surg 106:910-942, 1937 8. Grosfeld JL, Weinberger M, Clatworthy HW: Acute appendicitis in the first two years of life. J Pediatr Surg 8:285-293, 1973
Appendiceal
Perforation
and Hirschsprung’s
Disease
By Jeffrey Arliss and Leif 0. Holgersen New York, New 9 The case of a boy infant with neonatal appendiceal perforation who presented at 9 years of age with Hirschsprung’s disease is reported. A retrospective review of the microscopic sections of the appendix demonstrated periappendicitis without transmural inflammation. The importance of differentiating between true appendicitis and periappendicitis in a neonate with appendiceal perforation is stressed, in order to avoid a missed diagnosis of Hirschsprung’s disease. @ 1990 by W.B. Saunders Company.
York
tion, and the diagnosis was confirmed by rectal biopsy. Four weeks following a sigmoid colostomy, a Duhamel pull-through was performed. Review of the microscopic sections of the appendix removed as a neonate showed periappendicitis with no evidence of transmural inflammation. DISCUSSION
From the Section of Pediatric Surgery, Department of Surgery, St LukeS-Roosevelt Hospital Center, New York. NY. Address reprint requests to Lxif 0. Holgersen. MD. St Luke’sRoosevelt Hospital Center, Amsterdam Ave and 114th St, New York, NY 10025. o 1990 by W.B. Saunders Company. 0022-3468/90/2506-0030$03.00/0
Martin and Perrin* reported three cases of appendiceal perforation in neonates in whom Hirschsprung’s disease was later confirmed. In two of the three patients there was only minimal evidence of gross inflammation of the appendix. The perforation occured at the base of the appendix in two patients and the midportion in one. An additional report by Gastrin and Josephson3 described a neonate with an apical appendiceal perforation in whom Hirschsprung’s disease was diagnosed at 5 years of age. Microscopic sections of the appendix demonstrated periappendicitis. The appendix in the patient described here was perforated at the base and microscopically there was periappendicitis without transmural inflammation. Neonatal appendiceal perforation in the absence of transmural inflammation invites the conclusion that these perforations may be mechanical. The anatomy of the cecum and appendix was classified by Treves, in 1885,5 with the neonatal appendix having a definite funnel-shaped opening into the cecum. This configuration was confirmed by Snyder and Chaffin and Wangenstein et al7 who also demonstrated a sharp decrease in this configuration after 1 year of age. Because the base of the appendix would therefore be incorporated in the cecal circumference in the neonate, in the presence of distal obstruction it would also represent the potential point of maximum tension based on Laplace’s law, where the relationship between pressure (P), tension (T), and radius (R) is expressed as T = V2 PR. This phenomenon might explain the predilection for perforation at the base of the appendix in neonates with Hirschsprung’s disease. However, it does not explain the apical perforation of the appendix described by Gastrin, unless inspissated meconium in the lumen of the appendix was a factor, as in a case of appendiceal perforation and meconium plug syndrome described by Grosfeld et a1.s Inflammatory perforations of the neonatal appendix usually produce apical perforations. In a review of five cases of neonatal appendicitis by Schaupp et al,’ perforation in all five occurred at the apex of the appendix. The mortality in this series was lOO%, and autopsies performed on all
694
Journalof
INDEX WORDS: disease.
Appendicitis,
neonatal; Hirschsprung’s
N
EONATAL appendicitis is rare and apical perforation usually occurs prior to diagnosis.’ The association of neonatal appendiceal perforation with Hirschsprung’s disease has been detailed in two reports of four patients.29 The perforations in these patients occurred in the absence of transmural appendiceal inflammation. A previously published case of a patient with neonatal appendiceal perforation4 who subsequently presented with Hirschsprung’s disease, is the basis for this report. CASE REPORT A 7-day-old boy presented with fever, poor feeding, abdominal
distention, and obstipation for 24 hours. Examination included a plain abdominal x-ray that showed an early partial small bowel obstruction. A barium enema was reported as normal except for retained meconium in the transverse colon. Following nasogastric tube decompression, hydration, and antibiotics the child improved, and a right flank mass was noted. A sonogram and intravenous pyelogram showed right hydronephrosis consistent with a congenital ureteropelvic junction (UPJ) obstruction. The child underwent extraperitoneal repair of the UPJ obstruction at aged 14 days. At surgery, following completion of a right pyeloplasty, the peritoneum was opened. Unexpectedly, a grossly edematous and inflamed appendix was found, with a perforation at the base and a small retrocecal abscess. An appendectomy was performed with inversion and closure of the appendiceal stump. He made an uneventful recovery and at the time of discharge was having five to eight soft yellow stools a day. He was lost to follow-up until 9 years of age when he presented with a history of constipation that had begun 4 weeks following his appendectomy. A barium enema was consistent with Hirschsprung’s disease with a transition at the rectosigmoid junc-
Pediatric Surgery, Vol 25, No 6 (June), 1990: pp 694-695
NEONATAL
APPENDICITIS;
HIRSCHSPRUNG’S
DISEASE
695
neonates did not show any evidence of colon obstruction. In the presence of neonatal appendiceal perforation without the gross appearance of appendicitis, Martin and Perrin stressed appendectomy with exteriorization of the cecum, because the likelihood of coexistent Hirschsprung’s disease was so great. At laporatomy it may not be possible to distinguish between true transmural appendicitis and the periappendiceal reaction that accompanies perforation. The patient we treated and the patient described by Gastrin did well following appendectomy without cecostomy, despite the missed diagnosis of Hirschsprung’s disease. The presence of
five
healthy cecal tissue is probably the more important determinant of whether or not a decompression procedure is necessary. Despite the gross appearance of the appendix, careful microscopic review should be performed on all perforated appendices in the neonate. The absence of transmural inflammation should alert the clinician to the possibility of Hirschsprung’s disease and rectal biopsy should be considered. Transmural appendiceal inflammation has not been a feature in the patients reported with Hirschsprung’s disease and appendiceal perforation, and the implication that there is an association with true neonatal appendicitis and Hirschsprung’s disease should be dispelled.
REFERENCES 1. Schaupp W, Clausen EC, Ferrier PK: Appendicitis during the first month of life. Surgery 48805811, 1960 2. Martin LW, Perrin EV: Neonatal perforation of the appendix in association with Hirschsprung’s disease. Ann Surg 166:799-802, 1967 3. Gastrin U, Josephson S: Appendiceal perforation and megacolon in the neonatal period. Acta Chir Stand 136:153-155, 1970 4. Tucci P, Holgersen LO, Doctor D, et al: Congenital ureteropelvie junction obstruction associated with unsuspected acute perforated appendicitis in a neonate. J Urol 120:247-248, 1978
5. Treves F: The anatomy of the intestinal canal and peritoneum in man. Br Med J 1:470-474, 1885 6. Snyder WH, Chatlin L: Appendicitis during the first two years of life. Arch Surg 64549-560, 1952 7. Wangenstein OH, Buirge RE, Dennis C, et al: Studies in the etiology of acute appendicitis. Ann Surg 106:910-942, 1937 8. Grosfeld JL, Weinberger M, Clatworthy HW: Acute appendicitis in the first two years of life. J Pediatr Surg 8:285-293, 1973