- Email: [email protected]
Urinary obstruction caused by factitious urethral stones: An amazing manifestation of Munchausen syndrome by proxy
Urinary Obstruction Caused by Factitious Urethral Stones: An Amazing Manifestation of Munchausen Syndrome by Proxy By Mehmet
Emin !$enocak,
Aydln Ttirken, and Nebii Btiyakpamukqu Ankara, Turkey
@The diagnosis of Munchausen syndrome by proxy is frequently difficult to document and often delayed. A unique presentation of a case of Munchausen syndrome by proxy involving insertion of factitious stones in the lower urinary tract is reported. Copyright 0 1995by W.B. Saunders Company INDEX urinary
WORDS: foreign
Munchausen syndrome by proxy, bodies, urinary obstruction.
child
abuse,
ONTEMPORARY societies recognize child C abuse as a serious medical and social problem. Although Dr Kempe was not the first* to recognize child abuse or write about it, he was the first to focus our attention on the problem as a distinct and far more common entity than previously reported.2 It is estimated that the incidence of physical abuse is 6 per 1,000 live births, with a prevalence of 380 cases per million per year. 3 Estimates of abuse vary greatly because not all cases are reported. Several manifestations of child abuse have been documented, including burns; trauma to the central nervous system, soft tissues, skeleton, and viscera; ingestion; near-drowning; sexual abuse; starvation; aspiration; and neglect.4 Child abuse has been a relatively uncommon problem in our country. In an attempt to collect data on the actual incidence of this problem in our hospital, we found only 1 to 2 cases in 800,000 in a lo-year period. Munchausen syndrome by proxy is a form of child abuse in which parents fabricate or induce illness in their children. This syndrome was initially described in 1951 by Asher. It has become increasingly recognized since that time. Different varieties of Munchausen syndrome presentations have been reported, including cardiac arrest,6 recurrent sepsis,7 factitious bleeding:-lo recurrent vomiting, diarrhea,nJ2 factitious cystic fibrosis,13 pseudo-obstruction,14 prolonged coagulopathy,15 autoerythrocyte sensitization syndrome, 16and hematuria.17-20 The purpose of our report is to describe an unusual example of Munchausen syndrome by proxy (involving the urinary tract) and to discuss this form of chiId abuse, with a review of the literature. From the Depatiment of Pediattic Surgery, Hacettepe lJnivers&v, Medical Faculv, Ankara, Turkey. Address reprint requests to Mehmet Emin Jenocak, MD, Kehribar Sok. No. 9146, 06700 cankaya, Ankara, Turkey. Copyright 0 1995 by U? B. Saunders Company 0022-3468/9513012-0028$03.00/O 1732
CASE REPORT A 2-year-old boy was referred because of voiding disability of 2 days’ duration. According to the history provided by his father, the patient had had a large number of stones removed over the previous 6 months, by several physicians. The father showed the previously removed stones. Upon physical examination in the emergency room, the genitalia were normal except for a very dilated external urethral meatus (Fig 1). Two masses resembling intraluminal stones were palpated in the distal (close to the meatus) and proximal (nearly urogenital diaphragm) urethra, respectively. The other physical examination findings were normal. A pelvic radiograph showed two radiopaque stones (Fig 2). One stone was easily removed by a clamp through the wide open external meatus. A urethral catheter was installed and the bladder emptied. Retrograde urethrography showed the dilated urethra and a false tract with a stone at the end. Excretoty urography was completely normal, without any evidence of calculi in the upper urinary system. During cystoscopy, an edematous, irregular mucosa and trabeculated bladder wall were observed. During withdrawal of the cystoscope, another orifice was observed at the junction of prostatomembranous and bulbous urethra. The endoscope was introduced into the second orifice because the passage of this tract gradually narrowed. It could not be advanced, and examination was terminated. This false passage had no obvious mucosa. No stones were observed in the normal urethra, The second stone was located at the end of the acquired false passage. The stone was removed through an overlying skin incision, and the connection between the stone bed and the urethra was shown with a catheter. A Penrose drain was placed into the false site where the stone had been, and the incision was closed. A Foley catheter was inserted. The post-operative course was uneventful. The Penrose drain and Foley catheter were removed on the fifth and fifteenth postoperative day, respectively. A postoperative urethrogram showed normal findings. The false passage was obliterated and had healed spontaneously after removal of the stone. The second stone had the appearance of a pebble. The stones brought by the father were analyzed and reported as silicious pebbles, which are found in nature. Introduction of the stones by someone as a form of child abuse was suspected. A detailed investigation showed that the patient’s father was a bigamist and had illegally taken a second young woman as a fellow-wife (“kuma” in Turkish) because his first wife was infertile. The patient was born by the fellow-wife. The first wife remained in the same house, a common practice in some rural areas of Turkey. The first wife vented her anger at her husband, his fellow-wife, and their son by inserting pebbles into the boy’s urethra. After her actions were exposed, the woman was immediately removed from the home and separated from the family. On follow-up control 1% years later, the patient was in good health, with no evidence of stones in the lower and upper urinary tracts. DISCUSSION
According to Asher, Munchausen syndrome by proxy is a “common syndrome which most doctors have seen but about which little has been written.“5 JournalofPediatric
Grgery,
Voi 30, No 12 (December),
1995: pp 1732-1734
URINARY
OBSTRUCTION
BY FACTITIOUS
Fig I. Photograph shows abnormally stone was palpated in the distal urethra.
1733
STONES
wide
external
meatus.
A
However, an increasing number of cases are being documented.21 Munchausen syndrome by proxy generally affects children under 6 years of age. The most common presentations of Munchausen syndrome by proxy have been summarized by Rosenberg.22 Signs and symptoms are usually recurrent or persistent in nature, and symptoms occur only when the parent (or perpetrator) is alone with the child.4J4,21 Foreign bodies in the urinary tract are extremely rare, especially in children.23 There are three possible routes of entry, (1) external violence, (2) migration from the gastrointestinal tract, and (3) retrograde migration via the urethra. The urinary tract is rarely involved in cases of Munchausen syndrome by proxy.19 We have identified only a few reports describing factitious hematuria,17-20 and only three patients (one child)19 had passed genuine urinary stones.18J9 However, no stone or foreign bodies were detected in their urinary tracts during further evaluation of these cases. To our knowledge, the present case is the first documenting the presence of factitious stones in the urinary tract. In more than 95% of all cases of Munchausen by proxy, the mother is the perpetrator. The typical mother is intelligent and knowledgeable about medicine, and frequently has a background in nursing or child care.6,9J1,21 In most cases, the parents are married, and there may be a history of an unstable family relationship and/or parental psychiatric disturbance. In the present case, the father had illegally married another woman (the patient’s mother) without divorcing his first wife. The perpetrator (first wife) had neither a medical background, nor was she intelligent. Her relationship with her husband was also emotionallv distant. In most cases. the father is usually uninvolved,9T21 and when the true cause of the
child’s illness is discovered, he is usually surprised, as he was in our case. The diagnosis of induced or factitious illness is very difficult. This commonly results in acquisition of numerous and occasionally highly invasive tests. Significant morbidity and mortality may be associated with the syndrome, occasionally resulting in death. In one series, death occurred in more than 10% of cases.lO Early recognition or suspicion of this syndrome can reduce the number of unnecessary diagnostic procedures performed and may be lifesaving. When faced with a patient with enigmatic signs and symptoms and a family with classic personality traits, Munchausen syndrome by proxy should be considered. The warning signs of this syndrome have been emphasized by Meadow2r and could alert the physician to the possibility of factitious disease. Disappearance of symptoms after removal of the suspected perpetrator remains the key to diagnosis. Alternatively, symptoms may reappear when the perpetrator
Fig 2.
Plain radiogram
shows
the factitious
stones.
1734
returns. In spite of that, the diagnosis may go unrecognized for years. The prognosis for Munchausen syndrome by proxy remains poor. Prevention can be difficult and cannot
SENOCAK,
TtiRKEN,
AND
EWYUKPAMUK(X
be instituted until the diagnosis is determined. Once the syndrome is identified, however, family intervention and long-term management and support by social service organizations are imperative.
REFERENCES 1. Silverman F: Unrecognized trauma in infants, the battered 13. Orenstein DM, Wasserman AL Munchausen syndrome by child syndrome, and the syndrome of Ambroise Tardieu. Radiolproxy simulating cystic fibrosis. Pediatrics 78621-624, 1986 ogy 104:337-353,1972 14. Sullivan CA, Francis GL, Bain MW, et al: Munchausen 2. Kempe CH, Silverman FN, Steele BF, et al: The batteredsyndrome by proxy: 1990. A portent for problems? Clin Pediatr child syndrome. JAMA 181:17-24,1962 30:112-116,199l 3. Heins M: The “battered child” revisited. JAMA 2.51:329515. Babcock J, Hartman K, Pedersen A, et al: Rodenticide 3300,1984 induced coagulopathy in a young child. A case of Munchausen 4. Reece RM: Unusual manifestations of child abuse. Pediatr syndrome by proxy. Am J Pediatr Hematol Oncol 15:126-130, 1993 Clin North Am 37:905-921,199O 16. Clark GD, Key JD, Rutherford P, et al: Munchausen’s 5. Asher R: Munchamen’s syndrome. Lancet 1:339-341,195l syndrome by proxy (child abuse) presenting as apparent autoeryth6. Rosen CL, Frost JD, Bricker T, et al: Two siblings with rocyte sensitization syndrome: An unusual presentation of Polle recurrent cardiorespiratory arrest: Munchausen syndrome by proxy syndrome. Pediatrics 74:1100-1102,1984 or child abuse? Pediatrics 71:715-720, 1983 17. Tunbridge WIIG: Munchausen syndrome. N Engl J Med 7. Liston TE, Levine PL, Anderson C: Polymicrobial bacteremia 280:1130-1131, 1969 due to Polle syndrome. The child abuse variant of Munchausen by proxy. Pediatrics 72:211-213,1983 18. Atkinson RL, Earl1 JM: Munchausen syndrome with renal stones. JAMA 230:89,1974 8. Malatac JJ, Wiener ES, Gartner JC Jr, et al: Munchausen syndrome by proxy: A new complication of central venous catheter19. Sneed RC, Bell RF The dauphin of Munchausen: Factitious ization. Pediatrics 75:523-525,1985 passage of renal stones in a child. Pediatrics 58:127-130,1976 9. Kravitz RM, Wilmott RW: Munchausen syndrome by proxy 20. Meadow R: Munchausen syndrome by proxy: The hinterpresenting as factitious apnea. Clin Pediatr 29:587-592, 1990 land of child abuse. Lancet 2~343-345,1977 10. Yomtovian R, Swanger R: Munchausen syndrome by proxy 21. Meadow R: Munchausen syndrome by proxy. Arch Dis Child documented by discrepant blood typing. Am J Clin Path01 95:23257~92-981982 233,199l 22. Rosenberg DA Web of deceit: A literature review of 11. Ackerman NB Jr, Strobe1 CT: Polle syndrome: Chronic Munchausen syndrome by proxy. Child Abuse Negl 11:547-563, diarrhea in Munchausen’s child. Gastroenterology 81:1140-1142, 1987 1981 23. Chowdhury SD, Higgins PM: An intrarenal foreign body. Br 12. Sutphen JL, Saulsbury FT Intentional ipecac poisoning J Urol44:133-1351972 Munchausen syndrome by proxy. Pediatrics 82:453-456,1988
Emin !$enocak,
Aydln Ttirken, and Nebii Btiyakpamukqu Ankara, Turkey
@The diagnosis of Munchausen syndrome by proxy is frequently difficult to document and often delayed. A unique presentation of a case of Munchausen syndrome by proxy involving insertion of factitious stones in the lower urinary tract is reported. Copyright 0 1995by W.B. Saunders Company INDEX urinary
WORDS: foreign
Munchausen syndrome by proxy, bodies, urinary obstruction.
child
abuse,
ONTEMPORARY societies recognize child C abuse as a serious medical and social problem. Although Dr Kempe was not the first* to recognize child abuse or write about it, he was the first to focus our attention on the problem as a distinct and far more common entity than previously reported.2 It is estimated that the incidence of physical abuse is 6 per 1,000 live births, with a prevalence of 380 cases per million per year. 3 Estimates of abuse vary greatly because not all cases are reported. Several manifestations of child abuse have been documented, including burns; trauma to the central nervous system, soft tissues, skeleton, and viscera; ingestion; near-drowning; sexual abuse; starvation; aspiration; and neglect.4 Child abuse has been a relatively uncommon problem in our country. In an attempt to collect data on the actual incidence of this problem in our hospital, we found only 1 to 2 cases in 800,000 in a lo-year period. Munchausen syndrome by proxy is a form of child abuse in which parents fabricate or induce illness in their children. This syndrome was initially described in 1951 by Asher. It has become increasingly recognized since that time. Different varieties of Munchausen syndrome presentations have been reported, including cardiac arrest,6 recurrent sepsis,7 factitious bleeding:-lo recurrent vomiting, diarrhea,nJ2 factitious cystic fibrosis,13 pseudo-obstruction,14 prolonged coagulopathy,15 autoerythrocyte sensitization syndrome, 16and hematuria.17-20 The purpose of our report is to describe an unusual example of Munchausen syndrome by proxy (involving the urinary tract) and to discuss this form of chiId abuse, with a review of the literature. From the Depatiment of Pediattic Surgery, Hacettepe lJnivers&v, Medical Faculv, Ankara, Turkey. Address reprint requests to Mehmet Emin Jenocak, MD, Kehribar Sok. No. 9146, 06700 cankaya, Ankara, Turkey. Copyright 0 1995 by U? B. Saunders Company 0022-3468/9513012-0028$03.00/O 1732
CASE REPORT A 2-year-old boy was referred because of voiding disability of 2 days’ duration. According to the history provided by his father, the patient had had a large number of stones removed over the previous 6 months, by several physicians. The father showed the previously removed stones. Upon physical examination in the emergency room, the genitalia were normal except for a very dilated external urethral meatus (Fig 1). Two masses resembling intraluminal stones were palpated in the distal (close to the meatus) and proximal (nearly urogenital diaphragm) urethra, respectively. The other physical examination findings were normal. A pelvic radiograph showed two radiopaque stones (Fig 2). One stone was easily removed by a clamp through the wide open external meatus. A urethral catheter was installed and the bladder emptied. Retrograde urethrography showed the dilated urethra and a false tract with a stone at the end. Excretoty urography was completely normal, without any evidence of calculi in the upper urinary system. During cystoscopy, an edematous, irregular mucosa and trabeculated bladder wall were observed. During withdrawal of the cystoscope, another orifice was observed at the junction of prostatomembranous and bulbous urethra. The endoscope was introduced into the second orifice because the passage of this tract gradually narrowed. It could not be advanced, and examination was terminated. This false passage had no obvious mucosa. No stones were observed in the normal urethra, The second stone was located at the end of the acquired false passage. The stone was removed through an overlying skin incision, and the connection between the stone bed and the urethra was shown with a catheter. A Penrose drain was placed into the false site where the stone had been, and the incision was closed. A Foley catheter was inserted. The post-operative course was uneventful. The Penrose drain and Foley catheter were removed on the fifth and fifteenth postoperative day, respectively. A postoperative urethrogram showed normal findings. The false passage was obliterated and had healed spontaneously after removal of the stone. The second stone had the appearance of a pebble. The stones brought by the father were analyzed and reported as silicious pebbles, which are found in nature. Introduction of the stones by someone as a form of child abuse was suspected. A detailed investigation showed that the patient’s father was a bigamist and had illegally taken a second young woman as a fellow-wife (“kuma” in Turkish) because his first wife was infertile. The patient was born by the fellow-wife. The first wife remained in the same house, a common practice in some rural areas of Turkey. The first wife vented her anger at her husband, his fellow-wife, and their son by inserting pebbles into the boy’s urethra. After her actions were exposed, the woman was immediately removed from the home and separated from the family. On follow-up control 1% years later, the patient was in good health, with no evidence of stones in the lower and upper urinary tracts. DISCUSSION
According to Asher, Munchausen syndrome by proxy is a “common syndrome which most doctors have seen but about which little has been written.“5 JournalofPediatric
Grgery,
Voi 30, No 12 (December),
1995: pp 1732-1734
URINARY
OBSTRUCTION
BY FACTITIOUS
Fig I. Photograph shows abnormally stone was palpated in the distal urethra.
1733
STONES
wide
external
meatus.
A
However, an increasing number of cases are being documented.21 Munchausen syndrome by proxy generally affects children under 6 years of age. The most common presentations of Munchausen syndrome by proxy have been summarized by Rosenberg.22 Signs and symptoms are usually recurrent or persistent in nature, and symptoms occur only when the parent (or perpetrator) is alone with the child.4J4,21 Foreign bodies in the urinary tract are extremely rare, especially in children.23 There are three possible routes of entry, (1) external violence, (2) migration from the gastrointestinal tract, and (3) retrograde migration via the urethra. The urinary tract is rarely involved in cases of Munchausen syndrome by proxy.19 We have identified only a few reports describing factitious hematuria,17-20 and only three patients (one child)19 had passed genuine urinary stones.18J9 However, no stone or foreign bodies were detected in their urinary tracts during further evaluation of these cases. To our knowledge, the present case is the first documenting the presence of factitious stones in the urinary tract. In more than 95% of all cases of Munchausen by proxy, the mother is the perpetrator. The typical mother is intelligent and knowledgeable about medicine, and frequently has a background in nursing or child care.6,9J1,21 In most cases, the parents are married, and there may be a history of an unstable family relationship and/or parental psychiatric disturbance. In the present case, the father had illegally married another woman (the patient’s mother) without divorcing his first wife. The perpetrator (first wife) had neither a medical background, nor was she intelligent. Her relationship with her husband was also emotionallv distant. In most cases. the father is usually uninvolved,9T21 and when the true cause of the
child’s illness is discovered, he is usually surprised, as he was in our case. The diagnosis of induced or factitious illness is very difficult. This commonly results in acquisition of numerous and occasionally highly invasive tests. Significant morbidity and mortality may be associated with the syndrome, occasionally resulting in death. In one series, death occurred in more than 10% of cases.lO Early recognition or suspicion of this syndrome can reduce the number of unnecessary diagnostic procedures performed and may be lifesaving. When faced with a patient with enigmatic signs and symptoms and a family with classic personality traits, Munchausen syndrome by proxy should be considered. The warning signs of this syndrome have been emphasized by Meadow2r and could alert the physician to the possibility of factitious disease. Disappearance of symptoms after removal of the suspected perpetrator remains the key to diagnosis. Alternatively, symptoms may reappear when the perpetrator
Fig 2.
Plain radiogram
shows
the factitious
stones.
1734
returns. In spite of that, the diagnosis may go unrecognized for years. The prognosis for Munchausen syndrome by proxy remains poor. Prevention can be difficult and cannot
SENOCAK,
TtiRKEN,
AND
EWYUKPAMUK(X
be instituted until the diagnosis is determined. Once the syndrome is identified, however, family intervention and long-term management and support by social service organizations are imperative.
REFERENCES 1. Silverman F: Unrecognized trauma in infants, the battered 13. Orenstein DM, Wasserman AL Munchausen syndrome by child syndrome, and the syndrome of Ambroise Tardieu. Radiolproxy simulating cystic fibrosis. Pediatrics 78621-624, 1986 ogy 104:337-353,1972 14. Sullivan CA, Francis GL, Bain MW, et al: Munchausen 2. Kempe CH, Silverman FN, Steele BF, et al: The batteredsyndrome by proxy: 1990. A portent for problems? Clin Pediatr child syndrome. JAMA 181:17-24,1962 30:112-116,199l 3. Heins M: The “battered child” revisited. JAMA 2.51:329515. Babcock J, Hartman K, Pedersen A, et al: Rodenticide 3300,1984 induced coagulopathy in a young child. A case of Munchausen 4. Reece RM: Unusual manifestations of child abuse. Pediatr syndrome by proxy. Am J Pediatr Hematol Oncol 15:126-130, 1993 Clin North Am 37:905-921,199O 16. Clark GD, Key JD, Rutherford P, et al: Munchausen’s 5. Asher R: Munchamen’s syndrome. Lancet 1:339-341,195l syndrome by proxy (child abuse) presenting as apparent autoeryth6. Rosen CL, Frost JD, Bricker T, et al: Two siblings with rocyte sensitization syndrome: An unusual presentation of Polle recurrent cardiorespiratory arrest: Munchausen syndrome by proxy syndrome. Pediatrics 74:1100-1102,1984 or child abuse? Pediatrics 71:715-720, 1983 17. Tunbridge WIIG: Munchausen syndrome. N Engl J Med 7. Liston TE, Levine PL, Anderson C: Polymicrobial bacteremia 280:1130-1131, 1969 due to Polle syndrome. The child abuse variant of Munchausen by proxy. Pediatrics 72:211-213,1983 18. Atkinson RL, Earl1 JM: Munchausen syndrome with renal stones. JAMA 230:89,1974 8. Malatac JJ, Wiener ES, Gartner JC Jr, et al: Munchausen syndrome by proxy: A new complication of central venous catheter19. Sneed RC, Bell RF The dauphin of Munchausen: Factitious ization. Pediatrics 75:523-525,1985 passage of renal stones in a child. Pediatrics 58:127-130,1976 9. Kravitz RM, Wilmott RW: Munchausen syndrome by proxy 20. Meadow R: Munchausen syndrome by proxy: The hinterpresenting as factitious apnea. Clin Pediatr 29:587-592, 1990 land of child abuse. Lancet 2~343-345,1977 10. Yomtovian R, Swanger R: Munchausen syndrome by proxy 21. Meadow R: Munchausen syndrome by proxy. Arch Dis Child documented by discrepant blood typing. Am J Clin Path01 95:23257~92-981982 233,199l 22. Rosenberg DA Web of deceit: A literature review of 11. Ackerman NB Jr, Strobe1 CT: Polle syndrome: Chronic Munchausen syndrome by proxy. Child Abuse Negl 11:547-563, diarrhea in Munchausen’s child. Gastroenterology 81:1140-1142, 1987 1981 23. Chowdhury SD, Higgins PM: An intrarenal foreign body. Br 12. Sutphen JL, Saulsbury FT Intentional ipecac poisoning J Urol44:133-1351972 Munchausen syndrome by proxy. Pediatrics 82:453-456,1988