Injuries of the vulva and vagina in childhood

Injuries of the vulva and vagina are relatively rare in children. Over a seven-year period, we treated 45 girls. The most common etiologic factor in our study group was trauma. While 28 had only vulvar lesions, the rest had injuries of both the vulva and vagina. Thirty-two children were treated surgically for only vulvar and vaginal injuries. However, additional organ treatment was mandatory for 13 children.     

Vulvar disorders in the prepubertal female

Inspection of the vulva should be a routine part of well child care. Detection of poor perineal hygiene permits the establishment of good hygiene practices, which may prevent development of vulvovaginitis. Condylomata acuminata, molluscum contagiosum, herpetic vulvitis, and vulvovaginitis secondary to Neisseria gonorrhoeae, Gardnerella vaginalis, Chlamydia trachomatis, and Trichomonas vaginalis arouse suspicion of child sexual abuse, which must be addressed. Atopic dermatitis, psoriasis, lichen planus, and lichen sclerosis et atrophicus are often chronic and early diagnosis with appropriate treatment and follow-up contributes to the acceptance of the disease by parent and child. Early detection of the rare neoplasms of the vulva is essential to improved survival.     

A girl with labium majus swelling

Asymmetric vulva swelling during prepubertal period is related to a variety of inflammatory and neoplastic lesions. An 11-year-old girl with childhood asymmetric labium majus enlargement is presented.     

小阴唇粘连病因调查

目的 通过对小阴唇粘连患儿的病因调查,为寻找预防措施提供依据.方法 2010年3～12月重庆医科大学附属儿童医院共诊治小阴唇粘连患儿100例,为实验组,同期在儿保科就诊未患本病的同龄女婴100例为对照组,收集并分析两组临床资料,包括尿布使用、会阴护理、体重、尿常规检查,检测实验组雌激素水平.结果 实验组100例,就诊...     

Yolk sac tumor of the vulva: a case report with recurrence after long-term follow-up

A yolk sac tumor (YST) of the vulva is extremely rare and highly malignant with recurrence frequently occurring within a year. This report presents the 13th known case of vulvar YST, with recurrence occurring after the longest known follow-up period so far reported in the literature.     

Holocarboxylase synthetase deficiency induced by HLCS gene mutations: a rare disease study北大核心CSCD

男性患儿,16月龄,因发现头面部红斑15个月,外阴红斑10个月,加重5 d就诊。患儿新生儿期即出现口周、眼周红斑,婴儿期出现颈部、腋下、外阴三角区等腔口和皱褶部位的红斑、丘疹,可见脱屑和糜烂。血气分析提示代谢性酸中毒,血遗传代谢病氨基酸和酰基肉碱谱分析、尿液有机酸分析结果均提示多种羧化酶缺乏症,基因检测结果提示HLCS基因存在c.1522C>T(p.R508W)纯合突变。最终该患儿诊断为全羧化酶合成酶缺乏症,口服生物素治疗取得良好的临床疗效。该文总结了1例全羧化酶合成酶缺乏症患儿的临床资料,对其病因、诊断、治疗进行归纳总结,为临床医生诊断该类罕见疾病提供思路。     

Asymptomatic shedding of herpes simplex virus from the cervix and lesion site during pregnancy. Correlation of antepartum shedding with shedding at delivery

Asymptomatic shedding of herpes simplex virus (HSV) was detected in 0.83% of 955 cultures obtained from pregnant women with culture-proved recurrent genital HSV infections during pregnancy; seven (2.3%) of 299 women had asymptomatic shedding. In addition, one (2.3%) of 42 pregnant women with recurrent genital infection in the past but no attacks during pregnancy shed HSV when asymptomatic. Shedding occurred more frequently from the usual lesion site than from the cervix. The virus was not isolated at delivery from women with asymptomatic antepartum shedding. When an active lesion was present, concomitant shedding of HSV from the cervix occurred in seven (3.6%) of 193 pregnant women with vulvar lesions and in one (2.1%) of 47 women with lesions remote from the vulva. Ninety-two percent of the latter lesions were caused by type 2 HSV. In women who have had recurrent genital HSV infections in the past, asymptomatic shedding occurs in those with active attacks during pregnancy as well as in those asymptomatic throughout pregnancy; however, asymptomatic shedding during the antepartum period does not predict asymptomatic shedding at delivery.     

AN UNUSUAL CASE OF CROHN'S DISEASE IN A WEST INDIAN CHILD

ABSTRACT. Although perianal ulceration is common in Crohn's disease, extensive perineal ulceration particularly in children is rare. The case history is presented of a child who developed progressive hypertrophy of the vulva and ulceration of the groins at the age of 11 years but diagnosis of Crohn's disease was delayed for 21 months. Treatment with steroids and azathioprine has induced a successful remission but difficulty has been experienced in reducing the steroid dosage.     

Familial esophageal leiomyomatosis associated with Alport's syndrome in a 9-year-old boy

The authors describe a family in which the mother and one son are affected by oesophageal leiomyomatosis and nephritis with haematuria. The mother also presents hypertrophy of vulva and clitoris, and her son has perceptive deafness and congenital cataract. In the medical literature only 15 cases of oesophageal leiomyomatosis in children and adolescents could be found. The association with Alport's syndrome was first described by Torres and Guarner in 1983.     

Management of perineal canal anomaly

A 6-year-old girl presented due to passage of stool through her vulva since birth. Examination revealed a fistulous tract between vestibule of the vagina and an otherwise normally formed anal canal. The tract was successfully excised through an anterior sagittal approach with a defunctioning sigmoid colostomy, which was closed 12 weeks later. The embryology, morbid anatomy, and treatment of this rare congenital anomaly are discussed along with a review of the literature.     

Duplication of the rectum: report of four cases and review of the literature

Rectal duplications are rare anomalies. Recently, we observed four cases of rectal duplication, each presenting with different clinical features including chronic constipation, a prolapsing rectal “polyp,” a “growth” from the vulva, and acute retention of urine. The variety of clinical presentations may lead to delay in diagnosis and multiple operations. Accepted: 28 October 1997     

Lichen sclerosus in the differential diagnosis of suspected child abuse cases

Lichen sclerosus in young females can present as traumatic injuries on the vulva which can mimic sexual abuse. The case of an unconscious 6-year-old girl presenting in a reported inflicted drug overdose and with a clinical picture of ecchymosis and abrasions of the anogenital area is presented and discussed. The following case underscores the need for clinicians evaluating children for suspected abuse, to consider alternate conditions and causes that may not be related to sexual abuse.     

Successful management of a completely duplicated lower urinary system

We present a young girl with an exceedingly rare case of a duplicated bladder, urethra, vulva, vagina, cervix, uterus, appendix, colon and anus, as well as a lipomyleomeningocele. This complex of anomalies has been referred to in the literature as caudal duplication syndrome. This particular case was complicated with urinary and fecal incontinence, vesicoureteral reflux, and recurrent rectovaginal fistulas. We report the successful surgical management of her condition with resulting urinary and fecal continence and resolution of reflux and fistulas.     

Cutaneous amebiasis

An infant with cutaneous amebiasis of the vulva and amebic liver abscess is described. Epidemiologic investigations and serologic studies were crucial in establishing the diagnosis. The vulvar amebic ulcers responded dramatically to metronidazole therapy. Cutaneous amebiasis is a rare complication of Entamoeba histolytica infection which should be considered in the differential diagnosis of perineovulvar or penile ulcers. Cutaneous amebiasis may also occur on the abdominal wall surrounding a draining hepatic abscess, colostomy site, or laparotomy incision.     

Four-flap technique for secondary cosmetic correction of external genitalia in adrenogenital syndrome

Patients with adrenogenital syndrome (AGS) treated with staged operations or by Crawford or Fortunoff vaginoplasty often present with flat or absent labia minora and absent phallic skin; the cosmetic appearance is decidedly unsatisfactory. In order to improve the cosmetic appearance, construction of the labia minora is necessary. Five patients with AGS and unsatisfactory cosmetic postoperative appearance of the vulva were successfully treated with a four-flap labioplasty procedure. The surgical technique and results are reported and discussed. Correspondence to: G. Belloli     

Vulvar carcinoma in a 12-year-old girl with vertically acquired human immunodeficiency virus infection

We report the first case of a girl with vertically acquired human immunodeficiency virus (HIV) infection, who developed invasive squamous cell carcinoma of the vulva at 12 years of age. Lesions resembling bowenoid papulosis covered the perianal area as well. She underwent a nonmutilating surgical excision of the infiltrating lesion. More than 3 years later, her clinical condition is excellent, although dysplastic, noninfiltrating multifocal lesions persist. This case highlights the need to perform careful periodic genital examinations in all HIV-infected children and adolescents born to HIV-positive mothers.     

Papillomavirus-induced genital warts in a girl – management by surgery and immunomodulating therapy

A 4-year-old girl with condylomata acuminata of the vulva and papular warts of the surrounding skin is presented. The lesions were removed by surgery. Histologic investigation showed koilocytosis of the squamous epithelium and in-situ hybridization revealed human papilloma virus type 6 infection. There were no signs of sexual abuse or sexual transmission of the virus. After ablation, an interferon-containing ointment was applied. In order to prevent recurrence, a low-molecular-weight immunomodulating leucocyte fraction was given for more than 1 year, during which time no relapse was observed. Accepted: 15 March 1997     

22月龄女孩外阴反复皮疹半年余

患儿,女,22个月,因发现外阴部反复皮疹半年余就诊。病理组织活检诊断为朗格汉斯组织细胞增生症（LCH）,评估患儿全身情况,无其他系统受累表现,确诊为LCH（皮肤型）。对于仅发生于外阴部的皮疹,临床表现无特异性时,排除传染性软疣、性传播疾病、皮脂腺异位症等疾病后,应考虑LCH可能。     

A case of multiple deformities: upper harelip, tubercles of the auricle, anus in vulva]

The case presented of a newborn with multiple deformities: preauricular tubercles, left auricular roof deformity, labicachisis of the right upper lip, dermoid-type swelling on the left eye, deep labial fold at the left commissure and "forked" anus in vulva. Though the combination does not exactly match any syndrome, it has various points in common with Goldenhar's syndrome, of which it might be a phenotype variant. The single cause of the malformations might be an early (microvascular) disturbance of the primary segments in growth areas surrounding the cephalic and caudal segments, first of the medullary plate and subsequently of the primary medullary canal.     

Congenital H-type anorectal fistula: two case reports

The congenital H-type fistula between the anorectum and the urogenital tract without anal atresia is an extremely rare entity in the spectrum of anorectal anomalies. We report about two young girls, who had a congenital fistula between the left labium/vulva and the anus (=H-type fistula). After the management of the local infection and the abscess, the two patients underwent an anterior sagittal anorectovaginoplasty (ASARVP). Up to now there was no relapse of the fistula or the abscess in both cases. Thus ASARVP can be used as a simple method for congenital H-type fistulas with excellent functional and anatomical results.