ILLUSTRATIVE CASE
Hair-Thread Tourniquet Syndrome of the Hypertrophic Clitoris in a 6-Year-Old Girl Mesut Parlak, MD* and Ali Erdal Karakaya, MD† Abstract: Hair-thread tourniquet syndrome is defined as the ischemic strangulation of hair resulting in edema and severe pain and may cause amputation of organs. Strangulation of the external genitalia (clitoris, labia minora) has rarely been described in girls. Here, we present a case of hypertrophic clitoris injury secondary to hair strangulation in a 6-year-old girl. Key Words: hair-thread tourniquet syndrome, hypertrophic clitoris (Pediatr Emer Care 2015;31: 363–364)
H
air-thread tourniquet syndrome (HTTS) is defined as severe strangulation by hair mainly observed in infants and children. Hair leads to ischemic strangulation, edema, as well as severe pain and may cause amputation of organs. Hair-thread tourniquet syndrome typically involves the digits and genitalia. Strangulation of the external genitalia (clitoris, labia minora) has rarely been described in girls. Poor genital hygiene, infantile masturbation, and child abuse have been reported as a possible cause of the strangulation.1–3 We present the first case of hypertrophic clitoris injury secondary to hair strangulation in a 6-year-old girl, which can be another reason of HTTS.
CASE A 6-year-old girl was presented to the pediatric surgery department with an acute painful swelling of the clitoris. The symptoms had been present for 24 hours. There was no dysuria, insect bite, trauma, operation, and masturbation history. Physical examination revealed a swollen, edematous, erythematous, and tender clitoris, which was encircled by a long black hair. The edematous lesion was measured approximately 0.5 0.5 cm in size (Fig. 1). She was examined again under anesthesia because of anxiety. A long single hair was wrapped around the clitoris, causing local congestion and edema. Using a fine-toothed forceps, the hair was removed around the base of the edematous lesion. The lesion had been resolved, revealing only a small local edema within a few hours after the removal (Fig. 2). The clitoris had a normal appearance on the following visits, but on the last examination, the clitoral length was still 2 cm and hypertrophic (>95%; 1.58 cm) for healthy kids her age.4 Her serum 17-hydroxyprogesterone level was 0.83 ng/mL5 and her electrolytes were normal.
FIGURE 1. Clitoral hair-thread tourniquet syndrome visualized.
mainly in digits or the penis in the younger age groups. However, the HTTS of the external genitalia (clitoris, labia minora and majora) is rare and seen at ages ranging from 4 to 14 years.2,3 The mechanism of the tourniquet syndrome is not always demonstrated. The adhesion of hairs is increased on wet skin and has mechanic effects that can be stretched easily. Poor genital hygiene, infantile masturbation, child abuse, and adhesion of hair threads after baths have also been associated with HTTS.1,3,6,7 Our patient had a hypertrophic clitoris, and there was no history of masturbation that could have resulted in clitoral pseudohypertrophy. In addition, the normal serum hormonal values eliminated the diagnosis of congenital adrenal hyperplasia. Finally, it has been decided that clitoral hypertrophy can be an anatomic variation and it has caused risk for HTTS of the penis and digits. Only a few cases of clitoral HTTS have been reported in the literature. The cases generally presented to clinics with vaginalperineal pain, swelling in the vulvar region or clitoris for several
DISCUSSION The first HTTS was described in 1612 in the literature, and the first qualified case was published in Lancet in 1832.1 Approximately, 100 cases of HTTS have been reported, and it occurs From the Departments of *Pediatric Endocrinology, and †Pediatric Surgery, Necip Fazil State Hospital, Kahramanmaras, Turkey. Disclosure: The authors declare no conflict of interest. Reprints: Mesut Parlak, MD, Department of Pediatric Endocrinology, Necip Fazil State Hospital, Kahramanmaras, Turkey (e‐mail: [email protected]). Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 0749-5161
FIGURE 2. Hypertrophic clitoris after release of tourniquet.
Pediatric Emergency Care • Volume 31, Number 5, May 2015
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
www.pec-online.com
363
Pediatric Emergency Care • Volume 31, Number 5, May 2015
Parlak and Karakaya
hours, and dysuria. Urinary tract infection, insect bite, dermatitis, and trauma should be considered as a differential diagnosis. The early diagnosis and treatment are important, but skin reepithelialization can mask hair and causes a delay in diagnosis. In the literature, an interesting case of autoamputation of the clitoris has been reported. The pathophysiology of hair tourniquet injury is constriction and obstruction of distal venous and lymphatic drainage, and this may result in local edema and inflammation. If not removed, interruption of the arterial perfusion leads to ischemic injury, tissue necrosis, and autoamputation.1,3,8–12
CONCLUSIONS Our case was unique in a way that the anatomic variation of hypertrophic clitoris may be described as a risk factor, which is not previously reported in the literature. REFERENCES 1. Alverson B. A genital hair tourniquet in a 9-year-old girl. Pediatr Emerg Care. 2007;23:169–170.
4. Akbiyik F, Kutlu AO. External genital proportions in prepubertal girls: a morphometric reference for female genitoplasty. J Urol. 2010;184: 1476–1481. 5. Lashansky G, Saenger P, Fishman K, et al. Normative data for adrenal steroidogenesis in a healthy pediatric population: age- and sex-related changes after adrenocorticotropin stimulation. J Clin Endocrinol Metab. 1991;73:674–686. 6. Bannier MA, Miedema CJ. Hair tourniquet syndrome. Eur J Pediatr. 2013;172:277. 7. Sivathasan N, Vijayarajan L. Hair-thread tourniquet syndrome: a case report and literature review. Case Rep Med. 2012;2012:171368. 8. Press S, Schachner L, Paul P. Clitoris tourniquet syndrome. Pediatrics. 1980;66:781–782. 9. Rich MA, Keating MA. Hair tourniquet syndrome of the clitoris. J Urol. 1999;162:190–191. 10. Sylwestrzak MS, Fischer BF, Fischer H. Recurrent clitoral tourniquet syndrome. Pediatrics. 2000;105:866–867.
2. Golshevsky J, Chuen J, Tung PH. Hair-thread tourniquet syndrome. J Paediatr Child Health. 2005;41:154–155.
11. Kuo JH, Smith LM, Berkowitz CD. A hair tourniquet resulting in strangulation and amputation of the clitoris. Obstet Gynecol. 2002;99:939–941.
3. Serour F, Gorenstein A, Dan M. Tourniquet syndrome of the clitoris in a 4-year-old girl. J Emerg Med. 2007;33:283–284.
12. Osterhoudt KC, Beleniski F. Images in emergency medicine. The hair tourniquet syndrome. Ann Emerg Med. 2005;45:458, 463.
364
www.pec-online.com
© 2015 Wolters Kluwer Health, Inc. All rights reserved.
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Hair-Thread Tourniquet Syndrome of the Hypertrophic Clitoris in a 6-Year-Old Girl Mesut Parlak, MD* and Ali Erdal Karakaya, MD† Abstract: Hair-thread tourniquet syndrome is defined as the ischemic strangulation of hair resulting in edema and severe pain and may cause amputation of organs. Strangulation of the external genitalia (clitoris, labia minora) has rarely been described in girls. Here, we present a case of hypertrophic clitoris injury secondary to hair strangulation in a 6-year-old girl. Key Words: hair-thread tourniquet syndrome, hypertrophic clitoris (Pediatr Emer Care 2015;31: 363–364)
H
air-thread tourniquet syndrome (HTTS) is defined as severe strangulation by hair mainly observed in infants and children. Hair leads to ischemic strangulation, edema, as well as severe pain and may cause amputation of organs. Hair-thread tourniquet syndrome typically involves the digits and genitalia. Strangulation of the external genitalia (clitoris, labia minora) has rarely been described in girls. Poor genital hygiene, infantile masturbation, and child abuse have been reported as a possible cause of the strangulation.1–3 We present the first case of hypertrophic clitoris injury secondary to hair strangulation in a 6-year-old girl, which can be another reason of HTTS.
CASE A 6-year-old girl was presented to the pediatric surgery department with an acute painful swelling of the clitoris. The symptoms had been present for 24 hours. There was no dysuria, insect bite, trauma, operation, and masturbation history. Physical examination revealed a swollen, edematous, erythematous, and tender clitoris, which was encircled by a long black hair. The edematous lesion was measured approximately 0.5 0.5 cm in size (Fig. 1). She was examined again under anesthesia because of anxiety. A long single hair was wrapped around the clitoris, causing local congestion and edema. Using a fine-toothed forceps, the hair was removed around the base of the edematous lesion. The lesion had been resolved, revealing only a small local edema within a few hours after the removal (Fig. 2). The clitoris had a normal appearance on the following visits, but on the last examination, the clitoral length was still 2 cm and hypertrophic (>95%; 1.58 cm) for healthy kids her age.4 Her serum 17-hydroxyprogesterone level was 0.83 ng/mL5 and her electrolytes were normal.
FIGURE 1. Clitoral hair-thread tourniquet syndrome visualized.
mainly in digits or the penis in the younger age groups. However, the HTTS of the external genitalia (clitoris, labia minora and majora) is rare and seen at ages ranging from 4 to 14 years.2,3 The mechanism of the tourniquet syndrome is not always demonstrated. The adhesion of hairs is increased on wet skin and has mechanic effects that can be stretched easily. Poor genital hygiene, infantile masturbation, child abuse, and adhesion of hair threads after baths have also been associated with HTTS.1,3,6,7 Our patient had a hypertrophic clitoris, and there was no history of masturbation that could have resulted in clitoral pseudohypertrophy. In addition, the normal serum hormonal values eliminated the diagnosis of congenital adrenal hyperplasia. Finally, it has been decided that clitoral hypertrophy can be an anatomic variation and it has caused risk for HTTS of the penis and digits. Only a few cases of clitoral HTTS have been reported in the literature. The cases generally presented to clinics with vaginalperineal pain, swelling in the vulvar region or clitoris for several
DISCUSSION The first HTTS was described in 1612 in the literature, and the first qualified case was published in Lancet in 1832.1 Approximately, 100 cases of HTTS have been reported, and it occurs From the Departments of *Pediatric Endocrinology, and †Pediatric Surgery, Necip Fazil State Hospital, Kahramanmaras, Turkey. Disclosure: The authors declare no conflict of interest. Reprints: Mesut Parlak, MD, Department of Pediatric Endocrinology, Necip Fazil State Hospital, Kahramanmaras, Turkey (e‐mail: [email protected]). Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 0749-5161
FIGURE 2. Hypertrophic clitoris after release of tourniquet.
Pediatric Emergency Care • Volume 31, Number 5, May 2015
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
www.pec-online.com
363
Pediatric Emergency Care • Volume 31, Number 5, May 2015
Parlak and Karakaya
hours, and dysuria. Urinary tract infection, insect bite, dermatitis, and trauma should be considered as a differential diagnosis. The early diagnosis and treatment are important, but skin reepithelialization can mask hair and causes a delay in diagnosis. In the literature, an interesting case of autoamputation of the clitoris has been reported. The pathophysiology of hair tourniquet injury is constriction and obstruction of distal venous and lymphatic drainage, and this may result in local edema and inflammation. If not removed, interruption of the arterial perfusion leads to ischemic injury, tissue necrosis, and autoamputation.1,3,8–12
CONCLUSIONS Our case was unique in a way that the anatomic variation of hypertrophic clitoris may be described as a risk factor, which is not previously reported in the literature. REFERENCES 1. Alverson B. A genital hair tourniquet in a 9-year-old girl. Pediatr Emerg Care. 2007;23:169–170.
4. Akbiyik F, Kutlu AO. External genital proportions in prepubertal girls: a morphometric reference for female genitoplasty. J Urol. 2010;184: 1476–1481. 5. Lashansky G, Saenger P, Fishman K, et al. Normative data for adrenal steroidogenesis in a healthy pediatric population: age- and sex-related changes after adrenocorticotropin stimulation. J Clin Endocrinol Metab. 1991;73:674–686. 6. Bannier MA, Miedema CJ. Hair tourniquet syndrome. Eur J Pediatr. 2013;172:277. 7. Sivathasan N, Vijayarajan L. Hair-thread tourniquet syndrome: a case report and literature review. Case Rep Med. 2012;2012:171368. 8. Press S, Schachner L, Paul P. Clitoris tourniquet syndrome. Pediatrics. 1980;66:781–782. 9. Rich MA, Keating MA. Hair tourniquet syndrome of the clitoris. J Urol. 1999;162:190–191. 10. Sylwestrzak MS, Fischer BF, Fischer H. Recurrent clitoral tourniquet syndrome. Pediatrics. 2000;105:866–867.
2. Golshevsky J, Chuen J, Tung PH. Hair-thread tourniquet syndrome. J Paediatr Child Health. 2005;41:154–155.
11. Kuo JH, Smith LM, Berkowitz CD. A hair tourniquet resulting in strangulation and amputation of the clitoris. Obstet Gynecol. 2002;99:939–941.
3. Serour F, Gorenstein A, Dan M. Tourniquet syndrome of the clitoris in a 4-year-old girl. J Emerg Med. 2007;33:283–284.
12. Osterhoudt KC, Beleniski F. Images in emergency medicine. The hair tourniquet syndrome. Ann Emerg Med. 2005;45:458, 463.
364
www.pec-online.com
© 2015 Wolters Kluwer Health, Inc. All rights reserved.
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
