Genitourin Med 1997;73:63-65 63 Recurrent respiratory papillomatosis with pulmonary cystic disease in a child, following

Case Report maternal genital Genitourin Med: first published as 10.1136/sti.73.1.63 on 1 February 1997. Downloaded from

R T Sadikot, A C Andrew, J D Wilson, A G Arnold

Human papillomavirus (HPV) infection of the genital tract is associated with the development of genital warts. A causal link between maternal HPV infection and development of laryngeal papillomatosis in the offspring has been proposed. We report a case of pulmonary cystic disease, a rare but serious of laryngeal papillomatosis in a child, following maternal genital warts. (Genitourin Med 1997;73:63-65) Keywords: genital warts; pulmonary cystic disease; child

Introduction ENT department. A diagnosis of laryngeal Laryngeal papillomata are benign tumours, papillomatosis was made on and most commonly occurring in infants and chil- histologically the proved to be dren. Although histologically benign, they can benign. The severity of symptoms warranted a become a serious clinical problem because of tracheostomy and subsequently she remained their location, resistance to treatment, recur- under the care of ENT surgeons, being treated rences, and, rarely, due to their tendency to with endoscopic laser therapy for recurrent involve the lower .' It is now papillomata. Her mother gave a history of hav- well established that respiratory papillomata ing genital warts during pregnancy and deliv- are caused by HPV types 6 and 11.2 3 ery. Medical Chest Unit, Genital warts occur in sexually active men Physical examination of the girl revealed a Castle Hill Hospital, and women, and are also caused by HPV types tracheostomy tube with poor local hygiene. North Humberside R T Sadikot 6 and 11. A causal link between maternal Chest examination was unremarkable. A chest A G Arnold condylomata and respiratory papillomatosis in radiograph showed multiple cysts with fluid Department of the offspring has been proposed, and sporadic levels. Routine blood tests were normal. Pathology, Leeds case reports have appeared describing this Serum electrophoresis, alpha, antitrypsin lev- General Infirmary, phenomenon.4 We report the case of a young els, immunology studies (including T and B http://sti.bmj.com/ Leeds A C Andrew girl, with laryngeal papillomata and distal cell functions) were all normal. Sputum exam- Department of GU involvements, whose mother had genital warts ination was negative for acid fast bacilli. Medicine, Leeds at delivery. Spirometry revealed a moderately severe com- General Infirmary, bined obstructive and restrictive defect with Leeds 2.3/3 and J D Wilson FEV,/FVC of 1-1/2.2 (predicted 4), arterial blood gases showed hypoxaemia with on September 28, 2021 by guest. Protected copyright. Address correspondence to: Case report R T Sadikot, University of A 15 year old girl presented to the Chest Unit P02 of 9 5 and pCO2 of 4.2. CT of the chest Arkansas for Medical a of sided chest confirmed extensive lesions in both Sciences, Department of with history right pleuritic cystic Medicine, 4301 West pain, productive cough and haemoptysis. , most pronounced in the apices (fig). Markham, Little Rock, Following a normal vaginal delivery at term, at It was concluded that the cysts were sec- Arkansas 72205-7199, USA. one had with to the and that Accepted for publication the age of year she presented dry ondary laryngeal papillomas, 1 1 September 1996 cough, hoarseness of voice and to the her presenting symptoms were related to infec-

CT scans ofthe chest. 64 Sadikot, Andrew, Wilson, Arnold

tion within these cysts. With antibiotic treat- of 20.1 Various theories have been proposed to ment her symptoms abated, and the fluid levels explain the association, for example, contigu- within the cysts resolved. ous extension of the papillomata, diffuse viral A repeat of CT of the chest, a year later, contamination, multicentric origin of papillo- showed significant deterioration of the lung mata, and aerial dissemination. FragmentsGenitourin Med: first published as 10.1136/sti.73.1.63 on 1 February 1997. Downloaded from cysts. Following this episode, her tra- can become detached during endoscopic cheostomy tube was blocked with new papillo- resection and following tracheostomy, leading mata requiring several endoscopic resections. to distal spread.' Lung involvement can result In situ hybridisation of these papillomata con- in thin walled cysts, nodules, fibrosis and firmed HPV1 1. bronchiectasis. The mechanism of formation of lung cysts remains controversial. It may be due to obstruction of peripheral airways by the Discussion producing an area of emphysema Laryngeal papillomata, the most common by a check-valve mechanism, or it may result benign tumours of the , were first from necrosis and excavation of solid nodular described in the seventeenth century.5 The lesion. The distribution and extent of lung term juvenile laryngeal papillomatosis pre- lesions is best evaluated by CT. vailed in the 1960s; however, it is now clear As with other forms of HPV infection there that they can spread distally to involve the tra- is no treatment that will eradicate the . chea in 2%, and lungs in 1% of cases.' The Numerous forms of medical and surgical disease is notorious for its recurrence and treatment have been employed with varying hence is now termed "recurrent respiratory success. Medical forms have included use of papillomatosis'. Papillomata are relatively fre- made from the patients papilloma, quent in children, but can occur at any age. radiotherapy, ultrasound, injection of hor- The highest incidence is before the age of 5 mones, steroids, vidarabine, topical years.6 podophyllin, systemically administered cal- There is a strong evidence of viral aetiology. cium and magnesium, tetracyclines, topical or Batsakis postulated an activation of a persis- intralesional treatment with 5 fluorouracil, tent viral infection, coupled with an unknown systemic bleomycin and human leucocyte promoter as the aetiological basis for laryngeal .'0 The mainstay of palliation is papillomata.2 As yet no whole virus has been endoscopic resection. A single removal is isolated from laryngeal papillomata but exami- occasionally curative but recurrence com- nation of DNA extracted from speci- monly requires further intervention. In recent mens has identified human papilloma virus years microsurgical techniques and the use of types 6 and 11 as the aetiological agent.' carbon dioxide laser treatment has been advo- The link between maternal condylomata cated.9 Tracheostomy is avoided if at all possi- and respiratory papillomata in the offspring ble. The variety of treatments employed in the

was proposed many years before the causal management demonstrate that there is nohttp://sti.bmj.com/ HPVs were shown to be identical. In 1956, known cure, and attempts to prevent recur- Hajek reported a case of laryngeal papillomata rence have been largely unsuccessful. in an infant born to a mother who had a his- Cystic pulmonary lesions in themselves are tory of extensive condylomata in late preg- rarely symptomatic and require no treatment nancy and suggested that the disease was unless infected. Death is usually associated transmitted from the mother to the offspring with complications of a surgical procedure or during birth.4 A history of maternal condylo- respiratory failure, due to disease progression on September 28, 2021 by guest. Protected copyright. mata in pregnancy, or at the time of delivery, and loss of pulmonary parenchyma. If thera- was elicited from 29% to 60% of mothers, of peutic HPV prove successful in the juvenile onset respiratory papillomatosis treatment of genital warts, they may offer a patients.7 In contrast, such a history was further therapeutic option for recurrent respi- obtained in less than 5% of obstetric patients.8 9 ratory papillomatosis. This may also raise the The infection is usually acquired by the baby possibility of lung transplantation in patients perinatally but rare instances of children with with parenchymal lung damage, as in the case respiratory papillomata from caesarean deliv- described. ery, have been reported. HPV infection of the genital tract is com- Uncomplicated papillomata usually have a mon. USA national figures have estimated benign course and can regress spontaneously, 85 200 new cases of genital warts in particularly if confined to the larynx. Their 1993-1994. The best estimate of the risk of potential to compromise laryngeal function papillomatosis is 1 in 400 pregnancies of and to cause airway obstruction sometimes women with genital warts. Only 1% of these makes them a serious threat to life. Malignant will develop lung involvement. In light of this transformation is exceedingly rare and is usu- the risk of pulmonary papillomatosis is very ally associated with previous radiation ther- rare, however as it is a serious condition with a apy. reduced life expectancy, it is important that There are relatively few reports of involve- doctors treating women with genital warts are ment of the lower respiratory tract. The aver- aware of its existance. age time interval between the presentation The risk ofvertical spread to infant mucosal with laryngeal papillomata, and the later pre- sites, and the seriousness of laryngeal to sentation with cystic lung damage, is 12 pulmonary transmission at a later stage, as in years.5 Patients with this complication have a the case we have described, needs to be poor prognosis, few surviving beyond the age emphasised. Recurrent respiratory papillomatosis with pulmonary cystic disease in a child, following maternal genital warts 65

We are most thankful to M Samuels for secretarial assistance. matosis. Laryngol Otol 1988;102:1022-8. 7 Quick CA, Watts SL, Kyrzek RA, Saras AJ. Relationship 1 Kramer SS, Wehnut WD, Stocker JT, Kashima H. between condylomata and laryngeal papillomata: Clinical Pulmonary manifestations of juvenile laryngotracheal and molecular virological evidence. Ann Otol Rhinol papillomatosis. AYR 1985;144:687-94. Laryngol 1980;89:467-71. 2 Batsakis JG, Raymond AK, Rice DH. The pathology of 8 Mounts P and Shah KV. Respiratory papillomatosis: etio- head and neck tumours: Papillomas of the upper aero- logical relation to genital trait papilloma virus. Prog Med Genitourin Med: first published as 10.1136/sti.73.1.63 on 1 February 1997. Downloaded from digestive traits. Head and Neck Surg, 1983;5:332-4. Virol 1984;29:90-114. 3 Mounts P, Shah KV, Kashima H. Viral aetiology of juve- 9 Shah K, Kashima H, Polk BF, Shah F, Abbey H and nile and adult onset squamous papilloma of the larynx. Abramson A. Rarity of Caesarean delivery in cases of Proc Nall Acad Sci USA 1982;79:5425-9. juvenile onset respiratory papillomatosis. Obstet Gynaecol 4 Hajek E. Contributions to the aetiology of laryngeal papil- 1986;68:795-9. loma in children. Laryngol Otol 1956;70: 166-72. 10 Healey GB, Gelber RD, Trowbridge AL, Grundfast KM, 5 Cohn AM, Kos JT, Taber LH, Adams E. Recurring laryn- Ruben RJ and Price KN. Treatment of recurrent respira- geal papilloma. AmJ Otolaryngol 1981;2:129-32. tory papillomatosis with human leucocyte interferon. N 6 Benjamin B and Parsons D. Recurrent respiratory papillo- EnglJtMed 1988;319:401-7. http://sti.bmj.com/ on September 28, 2021 by guest. Protected copyright.