Advances in the management of infected urachal cysts

Advances in the management of infected urachal cysts

Advances in the Management of Infected Urachal Cysts By Barry M. Newman, Melvyn P. Karp, Theodore C. Jewett, and Donald R. Cooney Buffalo, New Y...

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Advances in the Management

of Infected

Urachal

Cysts

By Barry M. Newman, Melvyn P. Karp, Theodore C. Jewett, and Donald R. Cooney Buffalo,

New

York and Charlottesville,

0 Persistent urachal remnants are uncommon congenital anomalies. Unless an umbilical fistula exists, infection may be the first indication of this abnormality. Five children received initial treatment for this problem at the Children’s Hospital of Buffalo during a 20-year period, 1964 to 1994, and a sixth was seen secondarily. There were four boys end two girls; their ages ranged from 8 months to 9 years. Lo;wer abdominal mass with fever and local tenderness were the most common presenting signs. Ultrasound was the most accurate study, correctly diagnosing the cyst in both patients so examined. Incision end drainage alone was performed in one patient. The other five were managed with antibiotic therapy and complete excision as the primary procedure. Cultures were obtained in five patients and were positive in four, growing Staphylococcus aureus in three and Escherichia co/i in one. Significant genitourinary abnormalities were discovered in four of the five patients evaluated. It is concluded that the previously recommended therapy of incision and drainage followed by delayed resection was developed in the preantibiotic era to minimize the mortality from sepsis and the morbidity from recurrence. Our experience indicates that the use of appropriate antibiotics followed promptly by complete cyst excision as a primary procedure is both possible and safe in most cases. Furthermore. the large number of associated genitourinary abnormalities suggests that a complete work-up for these conditions should be performed. D 1986 by Grune & Stratton, Inc. INDEX WORDS: Urachus; urachal cysts.

Y MPTOMATIC PERSISTENCE of the urachus is uncommon; however, when it does occur, it most frequently presents in infancy or childhood. Most urachal anomalies have been classified into four major groups: (1) the completely patent urachus; (2) the urachal sinus (opening to the umbilicus); (3) the urachal diverticulum (opening into the bladder); and (4) the urachal cyst.’ Diagnosis and management of the first two groups are usually straightforward; treatment consists of complete excision of the draining sinus. The diverticula are often silent, unless complicated by stone formation,* and again the treatment is excision when necessary. The last group, urachal cysts, may occasionally give rise to unusual problems, such as carcinoma developing in the residual urachus.3 More often, though, the initial presentation is one of infection. Management of these infected urachal cysts has varied from simple drainage to radical excision.4 This article presents a simplified and safe approach to the management of these potentially morbid anomalies and draws attention to the possibility of associated genitourinary abnormalities.

S

JournalofPediatric

Surgery, Vol 21, No 12 (December), 1986: pp 1051-1054

Virginia MATERIALS AND

METHODS

A retrospective review included all children treated for infected urachal remnants at the Children’s Hospital of Buffalo during a 20-year period, 1964 through 1984. There were six patients (four boys and two girls), aged between 8 months and 9 years (8 months and 1, 2, 3,6, and 9 years). The charts were reviewed for presenting symptoms and physical findings; past medical history, especially involving any genitourinary problems; and laboratory and radiographic studies. Management of the urachal remnant was evaluated, including the use of antibiotics and the type of surgical procedure. Histopathologic studies were noted, and the results of the culture and the sensitivity of any drainage were determined. RESULTS

Infected urachal cysts presented as a lower midline abdominal mass in three of the six children. The infection caused right lower quadrant pain, simulating an acute abdomen in two children. In the last child, umbilical erythema was the only problem. Symptoms lasted from three days to 7 months, with a median of seven days. All children complained of pain, and four had a history of fever higher than 38 “C. A history of umbilical drainage or a complaint of dysuria was present in two children each. On examination, a tender lower abdominal mass was palpable in four children. Fever higher than 38 OC was noted in only two children, and umbilical drainage was actually seen in only 1 child. Initial laboratory evaluation included normal hematocrit determinations in all patients. The leukocyte counts, however, were markedly elevated in five children (range, 8,000 to 26,000 WBC/pL; average, 16,950). The urinalysis was within normal limits in all six children, and the urine culture had no growth in the three instances where it had been obtained. Routine abdominal roengtenograms were not judged to be helpful. Intravenous pyelography was performed in three children, and an abnormality (right hydronephrosis) was seen in only one study. Voiding

From the Departments of Surgery, School of Medicine, State University of New York at Buflalo. and University of Virginia Medical Center, Charlottesville. Va. Presented before the 17th Annual Meeting of the American Pediatric Surgical Association, Toronto, Ontario, May 14-I 7, 1986. Address reprint requests to Barry M. Newman. MD, Box 181. Department of Surgery, University of Virginia Medical Center, Charlottesville, VA 22908. a I986 by Grune & Stratton, Inc. 0022-3468/86/2112-0006$03.00/0

1051

1052

NEWMAN

ET AL

cystourethrograms, obtained in five children, were abnormal in three (Fig 1). Ultrasound, performed in two patients (Fig 2), was the most successful study and was diagnostic for urachal cysts in both instances. Incision and drainage alone was the initial treatment of the infected cyst in one patient. In another, who had had initial drainage elsewhere, a primary resection of the cyst with antibiotic coverage was performed for a recurrent abscess. Two children were managed with antibiotics and complete cyst excision.

a. ¶

r&

B&&5

4

PI

aaE

a‘

4Ilil

Fig 2. Ultrasound examination in a l-year-old child identifies a mass (c) in the midline. immediately cephalad to the bladder (B).

The remaining two children underwent radical excision of the cyst, including a cuff of bladder (cystostomy was performed in one of these children), as the initial procedure with antibiotic coverage. Triple antibiotic therapy of oxacillin, gentamycin, and clindamycin was used in three patients; oxacillin alone was used in one; and chloramphenicol alone was used in the other. Results from therapy were good. There were no postoperative infections. One cyst initially treated elsewhere became reinfected, but no patient treated primarily at this institution developed recurrent disease. One patient who had undergone a radical excision had prolonged urinary drainage from the cystostomy site, but this fistula closed spontaneously without further surgery. Histopathology, available for five patients, demonstrated transitional epithelium in only two specimens. The other three showed granulation tissue in an infected cyst, which was consistent with infected urachal cyst. Cultures of the fluid drained from these cysts grew Staphylococcus aureus in three of these five Table 1. Associated

Genitourinary

Tract Anomalies

in Five Patients Patient NO.

Condition

Therapy

1

Bilateral VU reflux: meatal

Bilateral ureteral reimplants

2

Bilateral VU reflux; basal

Bilateral ureteral reimplants;

bladder diverticulum

excision of bladder diverticulum

3 Fig 1. (A) This voiding cystourethrogram in a l-year-old child demonstrates an extrinsic defect on the dome of the bladder (B) due to compression from the urachal cyst. (B1 The same study also showed bilateral vesicoureteral reflux as well as a basal bladder diverticulum (arrows).

Right ureteral stenosis; right

Right ureteral reimplant

hydronephrosis 4

Right ureteral reflux

Observation

5

Bilateral inguinal hernia

Repair

Abbreviation: VU, vesicoureteral.

MANAGEMENT

OF URACHAL CYSTS

1053

patients. Of the remaining two cultures, one grew Escherichia coli, and the other had no growth. On the basis of past history or present studies in the five patients who could be evaluated, four were found to have significant genitourinary abnormalities (see Table 1). Three required surgery for correction of their problems, while one was managed expectantly with observation. The fifth child had bilateral inguinal hernias that had been repaired previously but had no other specific genitourinary pathology. DISCUSSION

The urachus forms early in embryonic life as a tubular connection between the dome of the developing bladder and the allantoic stalk. At the time of birth, the bladder remains an abdominal organ, but as infancy progresses, it is drawn into the pelvis. The urachus usually has regressed into a thin cordlike structure connecting, with some variations, to the umbilicus above and bladder below. Some patency of the urachus is not uncommon and has been reported in as many as 1 in 1,000 autopsies.5 Furthermore, in as many as 50% of bladder dissections, microscopic communications exist between the urachus and the bladder.6 Symptomatic patency, however, seems to be rare, with fewer than 500 cases having been reported in the literature.4 It is still unclear why so few become symptomatic while the rest remain silent. Once a persistent urachal remnant becomes symptomatic, a diagnosis of infected urachal cyst may be straightforward. A lower midline mass and/or lower midline abdominal pain with erythema near or drainage from the umbilicus are highly suggestive. If the cause of symptoms is unclear, cystography or abdominal ultrasound examination can be helpful and even diagnostic. More frequently, the diagnosis is less obvious, and an infected cyst can even present as an acute abdomen. In such cases, the correct diagnosis may only be made intraoperatively. The management of infected urachal cysts has remained controversial. In the past, patients were managed by incision and drainage alone,’ and the cysts often recurred.4 However, the unacceptably high complication and mortality rates in the preantibiotic era made complete resection an unattainable goal. Initial incision and drainage followed by delayed resection of

the cyst became the standard treatment for infected cysts. 8z9More recently, radical excision of the cyst, including a cuff of bladder, has been recommended for complete removal of the urachus as well as the cyst. In this way, according to one review,4 recurrence of infection, as well as the possible development of carcinoma in the urachus, can be prevented. A two-stage procedure has other disadvantages, as it entails two hospitalizations and two operative procedures, with an increased chance of complications. The addition of antibiotics has had a profound impact on the surgeon’s ability to resect infected tissue in other areas with lowered morbidity. This appears to be substantiated by our experience in the management of infected urachal cysts. With adjuvant antibiotic therapy, primary resection of infected cysts was safely and successfully completed in five children, Although radical resection of the urachus was performed in two children, one child developed prolonged urinary drainage. With the potential for such complications, and the acceptable results of resection of the urachus alone without a complete cuff of bladder, radical resction may not be necessary. Therefore, on the basis of these results, initial resection of both the infected cyst and the remaining urachus with adequate antibiotic coverage (oxacillin, gentamycin, and clindamycin) can and should be considered the preferred method of management when possible. In cases of severe sepsis, where the patient is too ill to tolerate a more extensive procedure, incision and drainage with deiayed resection should still be considered the procedures of choice.” Of importance in the evaluation and management of any child with an infected urachal cyst should be consideration of the potential for genitourinary anomalies. The frequent association of such abnormalities with patent urachus has been well known in the past,” but only one prior review has noted this association with cystic urachal disease.” In our patients, four of the five whose urinary tracts were evaluated were found to have significant pathology; in three patients, problems were severe enough to require surgical correction. Clearly, these findings strongly suggest that a thorough radiologic screening be obtained for any child with symptomatic urachal disease. An intravenous pyelogram and voiding cystourethrogram should be more than adequate to detect any of the possible genitourinary anomalies that may be present.

REFERENCES 1. Beg RC: The urachus and umbilical fistulae. Surg Gynecol Obstet 45: 165-I 78, 1927 2. Bandler, CC;, Milbert AH, Alley JL: Urachal calculus with consideration of associated pathology of the urachus in adult life. NY State J Med 42:2203-2209, 1942

3. Bourne nant? J Ural

CW,

May JE: Urachal 1977

remnants:

Benign

or malig-

1I8:743-747.

4. Blichert-Toft acquired variants.

M, Nielsen OV: Congenital patent urachus Acta Chir Stand 137:807-814, 1971

and

NEWMAN

1054

5. Campbell MF: Urology, vol 2 (ed 2). Philadelphia, Saunders, 1963, pp 1709-1710 6. Hammond G, Yglesias L, Davis JE: The urachus: Its anatomy and associated fasciae. Anat Ret 80:271-287, 1941 7. Tauber J, Bloom B: Infected urachal cysts. J Urol66:692-696, 1951 8. Kantor HI: Cysts of the urachus: Report of two cases. Ann Surg 109:277-285, 1939

ET AL

9. Brodie N: Infected urachal cysts. Am J Surg 69:243-248, 1945 10. MacMillan RW, Schullinger JN, Santulli TV: Pyourachus: An unusual surgical problem. J Pediatr Surg 8:387-389, 1973 11. Hinman F: Surgical disorders of the bladder and umbilicus of urachal origin. Surg Gynecol Obstet 113:605-614, 1961 12. Rich RH, Hardy BE, Filler RM: Surgery for the anomalies of the urachus. J Pediatr Surg 18:37&372, 1983

Discussion J. N. Schullinger

(New York): We also have seen six

such patients at the Babies’ Hospital in New York and I think the thing that has impressed us the most has been the unusual severity and seriousness of the infections. Only two of our cases were able to undergo primary excision. The other four had perforated into the free peritoneal cavity with resulting peritonitis and were too sick to undergo a primary excision. Of these four, one child was moribund when we first saw him and went on and died; so there is a death. Another child is brain damaged from the severity of the shock and sepsis. I think that these cases point out how potentially serious and grave this condition can be. We also had a high incidence of staphylococcal infection. Four of our six cases grew out Staph aureus. Curiously enough, we did not see any other associated urinary abnormalities. Our last patient was a 4-year-old boy who presented with a midline lower abdominal wall abscess and this was drained only. Three months later, the child had a normal ultrasound. He was lost to follow-up. Two years later his cyst recurred and perfo-

rated into the right lower quadrant. He was drained and later went on to uneventful excision. I think that we would certainly agree with Dr Newman and his colleagues that infected urachal cysts have all the potential for very serious complicating problems. We would also agree that simple drainage is not enough; it is not definitive. However, primary excision may not always be advisable either, especially in the very sick child with perforation and peritonitis. B. M. Newman (closing): I would like to thank Dr Schullinger for his comments and I would like to close with several comments of my own. I think that in children who are clearly moribund or septic, a primary cyst excision is not an appropriate type of therapy, but rather drainage is what is necessary and appropriate. With regard to the comment about urologic abnormalities, I think that in the literature very few authors have actually evaluated their patients for urologic abnormalities, and the several papers that have done this have demonstrated a much higher incidence of associated anomalies.