Klingsor Syndrome: An Observation in a Patient with Schizophrenia ()
1. Introduction
Traumatic amputations of the external genitalia have various etiologies. It may be an animal bite, a post-circumcision accident, self-mutilation or hetero-mutilation by aggression [1] [2]. Genital self-mutilation is a rare urological emergency, most commonly seen in psychotic patients. This pathology causes urinary and sexual complications and significant psycho-social impact [3].
Advances in microsurgery have improved the results of reimplantation of the amputated penis. The repair must concern the urethra as well as the vasculonervous structures and the corpus cavenosa. However, when the surgeon does not have microsurgical equipment, vascular repair may only concern the superficial (larger) dorsal vein to avoid postoperative congestion [4]. The success of the treatment depends on the time between the occurrence of the trauma and access to care, this conventional time is set at 6 hours, but reimplantation should always be attempted within 12 hours or even 24 hours of the trauma [5]. Mohammed in South Africa had successfully re-implanted a penile autoamputation 8 hours after the trauma [6].
We report a case of genital self-mutilation in a 35-year-old man, non-compliant schizophrenic received after 04 days of the trauma.
2. Case Presentation
A 35-year-old patient, a mason by profession, married and father of four children, was referred from a local hospital for self-mutilation of the external genitalia, which occurred following a psychotic attack. Before this referral, the patient had received surgical debridement, uretrovesical catheterization and transfusion. He is said to be addicted to alcohol and recently diagnosed with schizophrenia without compliance with treatment. He had a history of cranioencephalic trauma which was caused by a fall from a height of 5 meters 5 years ago.
The penile amputation stump was kept in a bag (Figure 1(a) and Figure 1(b)).
His general condition was preserved. He was hemodynamically stable. The examination noted an almost complete amputation of the penis at 3/4 of the root of the penis with absence of the testicles. The scrotal flap covered the stump of the penis with stitches allowing the uretrovesical probe to exteriorize (Figure 2).
(a) (b)
Figure 1. Aspect of penile amputation.
Figure 2. Remaining penis stump sutured with scrotal flap, bladder catheter in place.
Given the delay, penile reimplantation was not carried out. He benefited from dressing with good local progress; psychiatric treatment began upon his admission using neuroleptics: Risperidone, Loxapine and Tegretol. The urological therapeutic plan, after the stability of his mental state was a definitive perineal urethrostomy. The patient was lost to follow-up after leaving the hospital against medical advice.
3. Discussion
Klingsor syndrome refers to genital self-mutilation in man following delusional religious beliefs. Later, Schweitzer proposed expanding the term to include all psychotic disorders, drug and alcohol abuse [5]. This is how genital self-mutilation has been classified into 3 etiological subgroups: schizophrenic patients, trans sexual people and people with religious and cultural delusions, or into two categories according to other authors: genital self-mutilation without psychosis and Klingsor syndrome with psychosis [6] [7]. Rare cases of heteromutilation, often by aggression, are also reported.
Schizophrenia found in our patient is the risk factor frequently reported in the literature [8].
The incidence of these lesions is poorly known due to lack of reporting. Most often, only clinical cases are reported [9] [10]. Diabaté [1] in Senegal (2020), reported 8 cases of traumatic amputation of the external genitalia, including one case of self-mutilation. The trauma may involve the bursae, their contents and the penis. The pathological lesions vary greatly, ranging from simple skin laceration (blade, strangling hair) to removal of the testicles and penis (partial or complete) [6] [11], as was the case we report where there was complete amputation of the testicles and penis.
Our patient was 35 years old, the same age as the patient reported by Omar in Morocco [12] but it varies according to other authors; this is the case of Amal in Oman and Cardeno in the USA who reported cases whose ages were 52 and 60 years respectively [8] [9].
Klingsor syndrome is a serious situation. Apart from the noted hemorrhagic emergency and operating difficulties, there is a psychosocial impact, sexual and urinary complications (stenosis, urethral fistula) which make this pathology complex [1] [13]. Treatment must be multidisciplinary involving the urologist, psychiatrist, psychologist and emergency physician [14].
It is recommended to supervise reconstructive surgery through psychiatric care in order to prevent possible recurrence; it would be useless to carry out a reimplantation of the penis if the patient is not psychiatrically stable.
On the surgical level, advances in microsurgery have made it possible to improve the results of reimplantation of the amputated penis [11]. However, when the surgeon does not have microsurgical equipment, vascular repair may only involve the superficial (larger) dorsal vein to avoid postoperative congestion.
The success of this surgery depends on the good preservation of the stump and especially on the speed of the operation, the conventional time limit of which is set at 6 hours, but reimplantation must always be attempted within 12 hours or even 24 hours of the trauma. The amputated stump must be, after washing and disinfection, preserved in cold, sterile physiological serum then placed in a jar containing ice. It can also be wrapped in a sterile compress soaked in physiological saline [1] [15]. Our patient was received on the fourth day after trauma with the non-viable amputated stump making the attempt at reimplantation impossible.
The result after reimplantation is generally acceptable with a satisfactory aesthetic appearance of the penis and a good erection, although loss of sensitivity of the organ is sometimes reported [16].
When the time limit for reimplantation has passed and in the event of necrosis of the amputated stump, definitive perineal urethrostomy remains the last resort.
4. Conclusion
Klingsor syndrome is a rare phenomenon, most often observed in schizophrenia patients. Treatment remains complex, but it must be early and multidisciplinary.