Systematic Literature Review of Pediatric Male and Female Genital Hair Thread Tourniquet Syndrome

Abstract

The aim of this study was to describe genital hair thread tourniquet syndrome (HTTS) and its treatment by pediatric and adolescent health care providers through a systematic literature review. Methods:We performed a systematic literature review on pediatric male and female genital HTTS. Studies were included if they involved genital HTTS in males or females 21 years and younger and were published in English. The main outcomes were body parts involved, symptoms, treatment, anesthetic type, providers involved in diagnosis and management, and implications of delayed or missed diagnosis.Results: There were 38 female cases from 33 publications and 147 male cases from 47 publications . The average age among females and males was 9.1 and 5.1 years, respectively. Among cases involving female patients, 93% of them were premenarchal; patients were circumcised in 90% of reviewed cases of male HTTS. The most commonly involved body parts were clitoris and labia minora in females, and penis and urethra in males. Males most commonly presented with edema and urinary symptoms, whereas females most commonly presented with edema and pain. General anesthesia was used for tourniquet excision in most cases. Male and female genital HTTS were mostly managed by urologists and emergency medicine physicians, respectively. Conclusion:This systematic literature review of more than 150 cases of male and female genital HTTS describes evaluation and management of genital HTTS spanning 7 decades. The main treatment of genital HTTS remains prompt diagnosis and removal of the tourniquet, as well as education on prevention strategies. Delayed diagnosis due to lack of recognition of the HTTS can lead to serious sequelae. Development of national guidelines regarding best practices in management of genital HTTS disseminated to all providers taking care of pediatric and adolescent patients will lead to improved patient care.

The First Report of Isolated Clitoral Hood Hair-Thread Tourniquet Syndrome: A Study of Six Patients

“…Table 3 summarizes the details of some of the studies on HTTS including our study of the first six cases of isolated clitoral hood strangulation and other studies of HTTS involving other body parts. The symptoms of HTTS included pain, edema, erythema, abnormal gait, pruritus, discharge, and dysuria [1]. In our experience and those of others [1], HTTS management varied according to the presentation; ranging from simple removal of the strangulating hair with local care in an early presentation to excision of the necrotic lost tissue with the residual stump defect closure if broad in late presentation with necrosis of the strangulated tissue.…”

“…The symptoms of HTTS included pain, edema, erythema, abnormal gait, pruritus, discharge, and dysuria [1]. In our experience and those of others [1], HTTS management varied according to the presentation; ranging from simple removal of the strangulating hair with local care in an early presentation to excision of the necrotic lost tissue with the residual stump defect closure if broad in late presentation with necrosis of the strangulated tissue. However, in the presence of predisposing structural abnormalities such as labial hypertrophy or excessively redundant hood, previous reports on labiaplasty of injured or disfigured labia minora due to recurrent HTTS in hypertrophic labia minora [17,25] suggest that the correction of the abnormality should be included in the treatment.…”

“…The most commonly involved parts were labia minora (45.4%), the clitoris (42.4%), labia majora (9.1%), and mons pubis (3%) [21][22][23]. Adjei et al (2022) in a systematic literature review [1] reported HTTS cases involving pediatric male and female genitalia spanning almost 70 years. There were 38 female cases from 33 publications (1973-2020) and 147 male cases from 47 publications .…”

“…Hair-thread tourniquet syndrome (HTTS) is a potentially serious condition in which hair or fabric threads get wrapped around an appendage of various body parts (fingers, toes, uvula, tongue, clitoris, labia minor or major, and penis), thereby causing edema, ischemia, and, in severe cases, autoamputation [1]. The first known medical case of HTTS was published in The Lancet in 1832, and the syndrome was termed HTTS in a 1988 pediatric study of six children who presented with the disease [2,3].…”

Acute preputial strangulation secondary to hair tourniquet syndrome

“…Most frequent reported symptoms are edema (31%), urinary symptoms (17%), and in only 9% pain. Average age of debut is 5.1 years (5 months to 12 years) 1 …”