Spontaneous
Rupture of a Noninfected
Urachal
Cyst
By Victor Valda and Michael J. Conn Paterson, New Jersey
DISCUSSION
projections onto the anterior surface of the cloaca, the future bladder. It must be remembered that in the early embryo the bladder extends to the umbilicus. As embryonic growth continues, the bladder descends into the true pelvis. The apical portion of this early bladder narrows as the descent occurs into the fibromuscular strand known as the urachus. By the fifth month the small caliber tube is present. In the adult, this tube lies between the peritoneum and the transversalis fascia, extending from the bladder to the umbilicus. Here it lies adjacent to the umbilical ligaments, which are the remnants of the umbilical artery and veins. Normally, the lumen of the urachus in the human is obliterated or cohapsed. When present the lumen is usually lined with cuboidal or transitional epithelium.4 This fibrous cord or tube is usually 3 to 5 cm long.‘%s The pathology associated with the urachus is generally divided into four categories. The first is a patent urachus in which a communication between the bladder and the umbilicus exists. The next category pertains to the umbilical sinus, in which the urachus opens into the umbilicus. Here, drainage from the umbilicus will often be present. The third category is the vesicourachal diverticulum, in which the urachus has a wide patent opening into the bladder. Urinary compIaints are often cited with this type. The last category is the urachal cyst, in which the urachus encompasses a cystlike structure within its length. This last disease state, the urachal cyst, becomes prominent when infection occurs. Rupture of the cyst then becomes possible, and symptomatology can arise. Complaints and findings include lower abdominal pain, fever, voiding difficulties, midline hypogastric tenderness, urinary tract infections, and palpable lower abdominal mass. Diagnostic tests can be helpful in delineating this disease process. Recently, it has been shown that sonography is especially useful if an infected urachal cyst is suspected.’ Hopefully, the cystic mass would then be outlined for identification. The treatment for an infected urachal cyst is surgical excision. Simple drainage of the infected cyst results in reaccumulation in approximately 30% of
The urachus is formed early in the embryo. Controversy on its derivation exists.3 Most authors feel that the most superior aspect of the urachus is derived from the allantois, whereas the remainder of it is from the bladder dome. The allantois is an extraembryonic cavity that is located within the body stalk, with
From the Department of Pediatric Surgery, St Joseph’s Hospital and Medical Center. Paterson, NJ. Address reprint requests to Victor Valda, MD, 142 Gifford Ave, Jersey City, NJ 07304. Copyright o I991 by W-B. Saunders Company 0022-3468/9112606-0026$03.00/O
0 Spontaneous rupture of a noninfected, asymptomatic urachal cyst has not been previously reported. Thus, the following case report is of interest. Copyright o 1991 by W.B. Saunders Company INDEX WORDS:
Urachal cyst.
D
ISEASES OF THE urachus are rare. It has been reported that patency of the urachus in adult autopsy specimens is about 2%.’ In children, most difficulties arise related to urachal cysts that become infected and then rupture into the peritoneum. Literature reviews have pointed out a few such cases.2.3 CASE REPORT A 9-year-old Hispanic boy presented to the emergency room complaining of lower abdominal pain of a few hours duration. He described the pain as sharp and without radiation. The pain first appeared after urination earlier that evening. He denied dysuria or other urinary complaints. There was no history of trauma and the pain was not associated with nausea or vomiting. The boy and his mother did not report any previous like symptomatology or fever at home. He was otherwise healthy with a previous medical history that was significant only for a tonsillectomy at age 4. On physical examination, positive findings included a diffusely tender lower abdomen with guarding present. Bowel sounds were hypoactive. Rectal examination was nontender, heme negative. His temperature was 99.2”F with a white blood cell count of 9,900 (no bands present). Microscopic urinalysis showed no red blood cells and 0 to 1 white blood cells. The patient was admitted to the hospital for observation. During the following day his pain further localized to the suprapubic and right lower abdominal areas. He developed mild abdominal rebound in addition to the guarding. He was then brought to the operating room with a preoperative diagnosis of acute appendicitis. Upon entering the peritoneal cavity a large amount of clear fluid was encountered. The appendix was normal in appearance. Exploration showed a large cystic structure in the midline below the umbilicus, attached to the underside of the anterior abdominal wall. The diagnosis of a ruptured urachal cyst was entertained. Close examination demonstrated a small irregular perforation near the upper pole of this cystic structure. In addition, a tiny communication was noted inferiorly with the bladder. The cystic structure was resected in its entirety. along with a small area of adjacent bladder dome, and the abdomen was closed after vigorous irrigation. Pathology examination showed urachal cyst (Fig 1). Cultures of the peritoneal fluid and of the urine subsequently returned as no growth. The boy had an unremarkable postoperative course.
Joufffa/ofPediafric Surgery, Vol26, No 6 (June), 1991: pp 747-748
747
748
Fig 1. Gross photograph surface).
VALDA AND CONN
of urechal cyst (operative specimen, cut
the cases.’ In addition, because adenocarcinoma can arise in the urachal remnant, total excision of the urachus with removal of the adjacent bladder dome area is of extreme importance.’ Previous studies have pointed out the rarity of
urachal disease. Agatstein and Stabile reviewed the recent English language literature and found only 11 reported cases of intraperitoneal rupture of infected urachal cysts.’ No reports on noninfected urachal cyst rupture were noted. In this specific case report, no evidence of infection was found. Also, no history of trauma was present. The intraperitoneal fluid and the cyst fluid were clear, and all cultures taken were subsequently negative. The urinalysis and white blood cell count were unremarkable, and the patient was not febrile. Symptomatology in this patient most likely began after the cyst ruptured into the peritoneal cavity. It is unclear as to why this urachal cyst ruptured when an infection was not present. Most likely it was due to the enlarging cystic structure, secondary to increasing fluid sequestration. Urachal cysts, as previously reported, are usually asymptomatic until they become infected. Infection is not needed for an urachal cyst to rupture. Therefore, significant urachal disease does not have to be associated with the symptomatology and sequelae of infection. There is morbidity present in the cases of urachal cysts that are not yet infected.
REFERENCES 1. 3effs RD, Lepor H: Management of the exstrophy-epispadias complex and urachal anomalies, in Walsh PC, Gittes RF, Perlmutter AD, et al (eds): Cambell’s Urology (ed 5). Philadelphia, PA, Saunders, 1986, pp 1915-1919 2. Agatstein EH, Stabile B: Peritonitis due to intraperitoneal perforation of infected urachal cysts. Arch Surg 119:1269-1273, 1984 3. Blichert-Toft M, Nielsen 0: Diseases of the urachus simulating intra-abdominal disorders. Am J Surg 122123-127, 1971 4. Colodny AH, Lebowitz RL: Abnormalities of the bladder and
prostrate, in Ravitch MM, Welch KJ, Benson CD, et al (eds): Pediatric Surgery (ed 3). Chicago, IL, Year Book, 1979, pp 1306-1308 5. Blichert-Toft M, Koch F, Nielsen 0: Anatomic variants of the urachus related to clinical appearance and surgical treatment of urachal lesions. Surg Gynecol Obstet 137~51-54,1973 6. Spatero R, Davis R, McLochalan M, et al: Urachal abnormalities in the adult. Radiology 149:659-663,1983 7. Thomford N, Knight P, Nusbaum J: Urachal abnormalities in the adult. Am Surg 37:405-407,197l
Rupture of a Noninfected
Urachal
Cyst
By Victor Valda and Michael J. Conn Paterson, New Jersey
DISCUSSION
projections onto the anterior surface of the cloaca, the future bladder. It must be remembered that in the early embryo the bladder extends to the umbilicus. As embryonic growth continues, the bladder descends into the true pelvis. The apical portion of this early bladder narrows as the descent occurs into the fibromuscular strand known as the urachus. By the fifth month the small caliber tube is present. In the adult, this tube lies between the peritoneum and the transversalis fascia, extending from the bladder to the umbilicus. Here it lies adjacent to the umbilical ligaments, which are the remnants of the umbilical artery and veins. Normally, the lumen of the urachus in the human is obliterated or cohapsed. When present the lumen is usually lined with cuboidal or transitional epithelium.4 This fibrous cord or tube is usually 3 to 5 cm long.‘%s The pathology associated with the urachus is generally divided into four categories. The first is a patent urachus in which a communication between the bladder and the umbilicus exists. The next category pertains to the umbilical sinus, in which the urachus opens into the umbilicus. Here, drainage from the umbilicus will often be present. The third category is the vesicourachal diverticulum, in which the urachus has a wide patent opening into the bladder. Urinary compIaints are often cited with this type. The last category is the urachal cyst, in which the urachus encompasses a cystlike structure within its length. This last disease state, the urachal cyst, becomes prominent when infection occurs. Rupture of the cyst then becomes possible, and symptomatology can arise. Complaints and findings include lower abdominal pain, fever, voiding difficulties, midline hypogastric tenderness, urinary tract infections, and palpable lower abdominal mass. Diagnostic tests can be helpful in delineating this disease process. Recently, it has been shown that sonography is especially useful if an infected urachal cyst is suspected.’ Hopefully, the cystic mass would then be outlined for identification. The treatment for an infected urachal cyst is surgical excision. Simple drainage of the infected cyst results in reaccumulation in approximately 30% of
The urachus is formed early in the embryo. Controversy on its derivation exists.3 Most authors feel that the most superior aspect of the urachus is derived from the allantois, whereas the remainder of it is from the bladder dome. The allantois is an extraembryonic cavity that is located within the body stalk, with
From the Department of Pediatric Surgery, St Joseph’s Hospital and Medical Center. Paterson, NJ. Address reprint requests to Victor Valda, MD, 142 Gifford Ave, Jersey City, NJ 07304. Copyright o I991 by W-B. Saunders Company 0022-3468/9112606-0026$03.00/O
0 Spontaneous rupture of a noninfected, asymptomatic urachal cyst has not been previously reported. Thus, the following case report is of interest. Copyright o 1991 by W.B. Saunders Company INDEX WORDS:
Urachal cyst.
D
ISEASES OF THE urachus are rare. It has been reported that patency of the urachus in adult autopsy specimens is about 2%.’ In children, most difficulties arise related to urachal cysts that become infected and then rupture into the peritoneum. Literature reviews have pointed out a few such cases.2.3 CASE REPORT A 9-year-old Hispanic boy presented to the emergency room complaining of lower abdominal pain of a few hours duration. He described the pain as sharp and without radiation. The pain first appeared after urination earlier that evening. He denied dysuria or other urinary complaints. There was no history of trauma and the pain was not associated with nausea or vomiting. The boy and his mother did not report any previous like symptomatology or fever at home. He was otherwise healthy with a previous medical history that was significant only for a tonsillectomy at age 4. On physical examination, positive findings included a diffusely tender lower abdomen with guarding present. Bowel sounds were hypoactive. Rectal examination was nontender, heme negative. His temperature was 99.2”F with a white blood cell count of 9,900 (no bands present). Microscopic urinalysis showed no red blood cells and 0 to 1 white blood cells. The patient was admitted to the hospital for observation. During the following day his pain further localized to the suprapubic and right lower abdominal areas. He developed mild abdominal rebound in addition to the guarding. He was then brought to the operating room with a preoperative diagnosis of acute appendicitis. Upon entering the peritoneal cavity a large amount of clear fluid was encountered. The appendix was normal in appearance. Exploration showed a large cystic structure in the midline below the umbilicus, attached to the underside of the anterior abdominal wall. The diagnosis of a ruptured urachal cyst was entertained. Close examination demonstrated a small irregular perforation near the upper pole of this cystic structure. In addition, a tiny communication was noted inferiorly with the bladder. The cystic structure was resected in its entirety. along with a small area of adjacent bladder dome, and the abdomen was closed after vigorous irrigation. Pathology examination showed urachal cyst (Fig 1). Cultures of the peritoneal fluid and of the urine subsequently returned as no growth. The boy had an unremarkable postoperative course.
Joufffa/ofPediafric Surgery, Vol26, No 6 (June), 1991: pp 747-748
747
748
Fig 1. Gross photograph surface).
VALDA AND CONN
of urechal cyst (operative specimen, cut
the cases.’ In addition, because adenocarcinoma can arise in the urachal remnant, total excision of the urachus with removal of the adjacent bladder dome area is of extreme importance.’ Previous studies have pointed out the rarity of
urachal disease. Agatstein and Stabile reviewed the recent English language literature and found only 11 reported cases of intraperitoneal rupture of infected urachal cysts.’ No reports on noninfected urachal cyst rupture were noted. In this specific case report, no evidence of infection was found. Also, no history of trauma was present. The intraperitoneal fluid and the cyst fluid were clear, and all cultures taken were subsequently negative. The urinalysis and white blood cell count were unremarkable, and the patient was not febrile. Symptomatology in this patient most likely began after the cyst ruptured into the peritoneal cavity. It is unclear as to why this urachal cyst ruptured when an infection was not present. Most likely it was due to the enlarging cystic structure, secondary to increasing fluid sequestration. Urachal cysts, as previously reported, are usually asymptomatic until they become infected. Infection is not needed for an urachal cyst to rupture. Therefore, significant urachal disease does not have to be associated with the symptomatology and sequelae of infection. There is morbidity present in the cases of urachal cysts that are not yet infected.
REFERENCES 1. 3effs RD, Lepor H: Management of the exstrophy-epispadias complex and urachal anomalies, in Walsh PC, Gittes RF, Perlmutter AD, et al (eds): Cambell’s Urology (ed 5). Philadelphia, PA, Saunders, 1986, pp 1915-1919 2. Agatstein EH, Stabile B: Peritonitis due to intraperitoneal perforation of infected urachal cysts. Arch Surg 119:1269-1273, 1984 3. Blichert-Toft M, Nielsen 0: Diseases of the urachus simulating intra-abdominal disorders. Am J Surg 122123-127, 1971 4. Colodny AH, Lebowitz RL: Abnormalities of the bladder and
prostrate, in Ravitch MM, Welch KJ, Benson CD, et al (eds): Pediatric Surgery (ed 3). Chicago, IL, Year Book, 1979, pp 1306-1308 5. Blichert-Toft M, Koch F, Nielsen 0: Anatomic variants of the urachus related to clinical appearance and surgical treatment of urachal lesions. Surg Gynecol Obstet 137~51-54,1973 6. Spatero R, Davis R, McLochalan M, et al: Urachal abnormalities in the adult. Radiology 149:659-663,1983 7. Thomford N, Knight P, Nusbaum J: Urachal abnormalities in the adult. Am Surg 37:405-407,197l
