Description

• Characterized by the tight wrapping of an appendage such as fingers, toes, or even genitalia with hair or hairlike materials (i.e., synthetic fibers, hair ties, elastic bands), causing the appendage to have interrupted blood flow and resulting ischemia and necrosis
• Appendage strangulation is an emergency and requires rapid diagnosis and treatment. Although typically found in infants, any individual with compromised mentation/communication can be at risk. Digits and the penis are the most common locations, but other potentially affected areas include the scrotum, clitoris, labia minora and majora, and the uvula.
• Extremely rare, although possible involvement of a limb/strangulation at the level of the neck
• Synonym(s): hair-thread tourniquet syndrome, toe tourniquet, finger tourniquet; penile hair tourniquet syndrome; hair coil strangulation

Epidemiology

Incidence
Incidence is unknown. Many cases likely not reported by caregivers and treated at home. Of the reported cases:

• Most common location is the penis (44%), followed by toes (40%), and fingers (9%). The most commonly affected toe is the 3rd toe (32%). About 1/3 of cases will have multiple digits involved.
• The offending agent for both toe and penile strangulation is predominantly human hairs. Finger cases usually involve synthetic fibers.
• Finger cases usually occur in the first few months of life. Toe strangulation is more common in older infants. Penile cases have an average age of 2 years. Clitoral and labial cases are found in older children.

Prevalence
Prevalence is not known. Many cases are likely not reported by caregivers.

Etiology and Pathophysiology

• Circumferential strangulation of an appendage initially leads to distal blockage of the lymphatic and venous drainage. This fluid congestion leads to tissue edema that causes further vascular blockage and arterial occlusion.
• Prolonged interruption of arterial flow will lead to tissue damage and death. This process can take hours to days, depending on multiple factors; if not interrupted, can result in amputation
• Hair tourniquet syndrome likely occurs as an infant’s fingers/toes come in contact with a hair or similar fiber in the enclosed covering of mittens/socks or a similar situation. As the infant moves digits back and forth, the hair is progressively wound around the digit.
• Wet hair especially may constrict as it dries, leading to additional strangulation.

General Prevention

• Parents should consider laundering their child’s clothing inside out to prevent accumulation of human hairs/fibrous materials that contribute to strangulation. This is especially true for socks, mittens, and similar clothing items with blind ends.
• Parents who notice an increased amount of hair loss after giving birth (i.e., telogen effluvium) should be mindful to remove loose hairs.
• It has also been suggested that being circumcised can increase a child’s risk for penile strangulation because an uncircumcised glans is more difficult to entrap. Also postulated is that the increased size of adult appendages accounts for the rare number of cases of hair tourniquets in older children and adults with cognitive impairment.

Commonly Associated Conditions

• Although hair tourniquet syndrome is rarely reported in older children/adults, any individual with compromised mentation/communication abilities may be at risk.
• If a child presents multiple times with this complaint, medical providers should consider nonaccidental child abuse (e.g., fabricated illness by a caregiver) as a cause.

History

• At presentation, caregivers may have already recognized the strangulation of the appendage and are seeking medical attention because they are unable to remove the offending material or are concerned about the appearance of the appendage.
• However, some caregivers may bring a child in secondary to the inability to console the child or reports of persistent referred pain (e.g., complaints of abdominal pain secondary to a penile tourniquet).
• With concerns about potential abuse (i.e., a nonaccidental trauma), the medical provider should elicit and document as many details about the events surrounding the injury as possible. This can include a list of recent caregivers, any history of abuse in the home, if the child has ever had prior hair tourniquets, or if older children have had a similar episode (1)[C].

Physical Exam

• Most patients will present with normal vital signs, in mild to moderate distress, and varying levels of consolability.
• Patients are generally noted to have an edematous, discolored digit/genitalia, and the edema can begin at any length of the appendage. The area of strangulation may even be confused for a circumferential laceration. With significant edema, it may be very difficult to appreciate the presence of the offending material. Fair-colored hair is especially difficult to visualize.
• Examination of the distal portion of the appendage may show a normal to delayed capillary refill. It may be tender to palpation and passive movement. In early identification, the appendage may be faint to dark red or purple in color and still warm to the touch. Edema is usually present, and providers may also note blistering of tissue. More advanced cases may reveal a pale or dusky appendage that is cool to the touch.
• Usually, only a single digit is affected, but there can be multiple digits or even bilateral involvement. The physical exam should be thorough to rule out involvement of other appendages and the genitalia.
• The presence of multiple, organized knots may suggest a nonaccidental origin. Disordered knots, however, are a common finding in nonabuse cases (1)[C].

Differential Diagnosis

• Amniotic bands may have a similar appearance, but these are usually identified at birth.
• Ainhum (constriction of 5th toe of unknown etiology) also known as “dactylolysis spontanea,” typically occurs in dark-skinned persons and is extremely rare.
• Health care workers and child protection workers are shown to misidentify a high proportion of hair tourniquet cases as nonaccidental trauma. Generally insufficient evidence exists to prove/disprove child abuse. Even in older children, many cases are due to exploratory behaviors (e.g., a child placing a sibling’s hair ties on his own penis). Some cultures may even nonmaliciously tie strings around a boy’s penis in attempts to improve bed wetting behaviors.

Diagnostic Tests & Interpretation

Initial Tests (lab, imaging)

• Generally no labs are used in diagnosis of this syndrome. Diagnosis is made by careful examination and a high index of suspicion.
• Imaging is generally not indicated for diagnosis/treatment. However, in cases where the diagnosis is missed, the injured tissue may reepithelialize over a strangulating hair/fiber. In rare instances, the offending material may then further penetrate through tissue approaching bony structures. Plain film x-rays may demonstrate soft tissue edema, periosteal reactions, or even bony erosion (2).

Medication

First Line
Over-the-counter depilatory creams (e.g., Nair) have increasingly become the first-line choice of treatment in cases when offending material cannot simply be unwound. A 30-minute application, followed by rinsing the skin, is generally sufficient (3)[C]:

• Depilatory creams may not be effective on synthetic fibers.

Second Line
Wound-associated cellulitis is rare but should be treated with appropriate antibiotic coverage (3)[C].

Issues For Referral

• If the offending material cannot be removed with the above methods, surgical consult may be indicated and removal done under general anesthesia (3).
• After removal, if the appendage does not demonstrate appropriate reperfusion or necrosis is present, prompt surgical consult is warranted. Poor cosmetic outcomes at any point of healing may warrant referral to a plastic surgeon (3).
• If the penis is involved in any of the above scenarios, a urologist should be consulted (4).

Additional Therapies

• Once the offending material has been removed and good perfusion assured, patients can usually be discharged with an antibiotic ointment (e.g., Polysporin) for topical application (3).
• If the surface of the skin was compromised, tetanus prophylaxis (as age appropriate) should be considered (3).

Surgery/Other Procedures

• In some instances, not all fibers can be visualized and removed with unwinding and depilatory creams alone. This is especially true if the surface skin has reepithelialized over the offending material. In these instances, a surgical incision is recommended (4)[C].
• The appendage should be thoroughly cleaned with a disinfectant solution. 1% lidocaine, without epinephrine, should be injected with a 25-gauge needle on each side of the digit for a digital block. A no. 11 or no. 15 surgical blade should then be used to create a longitudinal incision over the area of strangulation on the dorsal side of the digit. Some authors recommend that if an incision is required, then cutting to the depth of the bone (in digits) is ideal to ensure all fibers have been dissected (2,3,5)[C].

Admission, Inpatient, and Nursing Considerations

• Most children do not require inpatient monitoring. If necrosis/amputation is present or if after treatment the affected appendage does not show adequate perfusion, then admission for further monitoring and treatment is recommended.
• Most patients are stable at the time of presentation.
• If an infant presents with a history of poor oral intake and infrequent voiding secondary to the discomfort of the hair tourniquet or has signs/symptoms of dehydration on physical exam, then consider IV fluid resuscitation. Severe dehydration is treated with a 20 mL/kg bolus of normal saline, with repeat boluses as needed followed by routine maintenance fluids. Mild to moderate dehydration can usually be managed with oral rehydration.
• After reperfusion has been assured, nursing should be ordered to check the neurovascular status of the affected areas at frequent, regular intervals (e.g., q2h). Nursing should report any adverse changes in perfusion (e.g., delayed capillary refill), coloration (e.g., increasing paleness/cyanosis), or decreasing sensation.
• Once the patient is able to demonstrate adequate, sustained perfusion of the affected area, has adequate pain control with oral medications, and is taking adequate oral intake to maintain hydration, the patient can be safely discharged with close follow-up with primary care provider.

Follow-up Recommendations

Regular follow-up with primary care provider until the injury site is fully healed

Patient Education

If the injury site demonstrates increasing pain, decreasing sensation, poor perfusion, or discoloration, the patient should seek prompt medical attention. Medical providers should review prevention strategies with families (discussed earlier).

Prognosis

Prognosis is typically very good in cases when the diagnosis is made early.

Complications

• Amputation of the appendage, cellulitis, scarring, gangrene, poor cosmetic outcome, flexion deformity, tissue loss, permanent neurovascular damage, loss of function
• Penile complications can also include urethral strictures, urethral transection, and urethral fistula formation.

ICD-10

• S30.842A External constriction of penis, initial encounter
• S60.349A External constriction of unspecified thumb, initial encounter
• S60.449A External constriction of unspecified finger, initial encounter
• S90.444A External constriction, right lesser toe(s), initial encounter
• S90.445A External constriction, left lesser toe(s), initial encounter
• S90.446A External constriction, unspecified lesser toe(s), initial encounter

ICD-9

• 911.8 Other and unspecified superficial injury of trunk, without mention of infection
• 915.8 Other and unspecified superficial injury of fingers without mention of infection
• 917.8 Other and unspecified superficial injury of foot and toes, without mention of infection

SNOMED

• 236757005 Strangulation of penis (disorder)
• 249248002 Constriction in shaft of penis (finding)
• 274193006 Superficial injury of finger (disorder)
• 274200008 superficial injury of toe (disorder)
• 283043006 Superficial injury of little toe (disorder)