Surgical treatment of malignant biliary papillomatosis invading adjacent organs: A case report

BACKGROUND Biliary papillomatosis(BP) is a rare disease characterized by multiple papillary adenomas in the intrahepatic and extrahepatic biliary tree and has a high risk of malignant transformation. Early diagnosis and treatment for BP are challenges, as it spreads along the biliary tract. A radical resection with an adequate resection margin is advocated in patients with malignant BP.CASE SUMMARY We report a case of BP with malignant transformation in a 52-year-old female patient who presented with emaciation, severe malnutrition, and a T-tube for biliary drainage that was placed for almost 2 years at the time she visited us. The enhanced magnetic resonance imaging of the upper abdomen revealed a neoplasm with a diameter of 15 cm located in the gallbladder area surrounding the common bile duct where a drainage tube was placed. It was the first case with malignant BP involving multiple organs and the abdominal wall and receiving a radical resection. Plastic surgeons helped close the incision by transferring the rectus muscle and external oblique muscle flap. A diagnosis of papillary carcinoma with diffuse malignant papillomatosis of the biliary duct was given.The postoperative course was uneventful and she was asymptomatic and in good physical condition at 35 mo postoperatively with appropriate chemotherapy.CONCLUSION Aggressive surgical treatment with appropriate chemotherapy is advocated for patients with malignant BP even if multiple organ invasion is present.

Impediments to clinical diagnosis and management of juvenile-onset recurrent respiratory papillomatosis in Ilorin, Nigeria

Background: To report the impediments to the diagnosis and management of children with JuvenileOnset Recurrent Respiratory Papillomatosis as seen in a teaching hospital in West Africa. Methods: Retrospectively analysed study of participants requiring surgical intervention for histologically confirmed juvenile onset recurrent respiratory papillomatosis managed at the Department of Otorhinolaryngology, University of Ilorin Teaching Hospital, Ilorin over a 10-year period (2002-2011) using prospectively collated database. Results: Juvenile-Onset Recurrent Respiratory Papillomatosis constituted 8.8% of 307 laryngeal pathologies seen during the study period. 18 (66.7%) were males and 9 (33.3%) females with a sex ratio (M:F) of 2:1. Age ranged from 1 year 4 months to 12 years with a mean age of 6.1 years (SD = 2.78), with the 6 - 10 age group constituting the largest categories in 51.9% of patients seen. Patients from low socioeconomic class constituted slightly over half of the patients studied (51.9%). 55.5% of the patients presented late (>13 months). All patients presented with hoarseness, stridor and difficulty in breathing. Misdiagnosis made by the referring clinician included Asthma, Laryngo-tracheo-bronchitis, Foreign body aspiration and Laryngomalacia. Involvement of the glottis occurred in 70.4% of cases seen, while bilateral involvement was present in 91.3% of cases operated. Recurrence was seen in 63.0% of the patients and the best attendance at follow-up clinic was during the first three months post surgical extirpation. Emergency tracheostomy was done to relieve airway obstruction in 17 (63.0%) patients. Conclusion: Impediments to diagnosis and management of JORRP are multifactorial. Misdiagnosis, late presentation and poor follow-up clinic attendance of patients with JORRP is still a common occurrence among the physicians. Recommendations on how to improve management and outcome of JORRP were made.

Coblation: An Alternative to CO2 Laser and Microdebrider for Laryngeal Papillomatosis

Objective: To assess the efficacy of coblation in treating laryngeal papillomatosis and its effect on post operative voice outcome. Study Design: This is a retrospective study. Setting: Tertiary referral centre. Subjects and Methods: All the patients diagnosed with laryngeal papillomatosis between January 2013 to December 2016 were included in this study. Preoperative assessment was done with rigid and flexible laryngoscopy. PRAAT software was used for voice analysis. All patients underwent coblation assisted microlaryngeal surgery. Post operatively patients were followed up at 1 week, 15 days, 1 month, 3 months and 6 months with rigid laryngoscopy. Voice analysis was repeated after 3 months to assess the improvement in voice. Results: Coblation was found to be effective in treating laryngeal papillomatosis. In our series of seven patients, three patients underwent repeat procedure with coblation for recurrence. But longterm follow up these patients did not reveal any recurrence of laryngeal papillomatosis. Voice analysis revealed a significant improvement in measures of perturbation and maximum phonation time. Conclusion: Coblation is a promising alternative to the conventional methods for the treatment of laryngeal papillomas as it can achieve satisfactory disease clearance with good voice quality.

Successful Treatment of Intra-Arterial Peplomycin Infusion for Recurrent Oral Florid Papillomatosis

A 56-year-old woman had noticed the erosion of oral mucosa and tongue 8 years ago. The mucosal lesions had been initially diagnosed as oral lichen planus and resistant to various treatments with prednisolone, etretinate and mizoribine and so on. One year ago, rapidly growing verrucous lesion occurred on her upper lip. Although we administered intralesional radiation therapy, the tumor recurred and new whitish lesions on the buccal mucosa and hard palate occurred 9 months after treatment. We confirmed an anatomical blood supply to the tumors by a fluorescent real-time imaging system and subsequently administered the intra-arterial infusion of peplomycin through retrograde catheters from bilateral superficial temporal arteries under the final diagnosis as oral florid papillomatosis (OFP). The tumors were dramatically shrunk and did not recur 16 months after treatment. OFP is known as clinically multiple whitish and verrucous lesions over the oral cavity and lip and a subtype of SCC with high differentiation. We suppose that an intra-arterial infusion therapy of peplomycin should be considered as the curative treatment for OFP.

Prurigo Pigmentosa and Confluent and Reticulated Papillomatosis a Spectrum of One Disease: A New Case Report

Background: Prurigo Pigmentosa (PP) is a rare inflammatory dermatitis first discovered in 1971. Characterized by a sudden eruption of pruritic reticulated, pink-brown papules coalescing into plaques distributed symmetrically over shoulders, neck, chest, and back. Various triggers have been identified, including the ketogenic diet. Clinicopathological presentation looks similar to confluent and reticulated papillomatosis (CARP) which is a rare dermatosis of unknown etiology characterized by hyperkeratotic pigmented papules & peripheral reticulation involving seborrheic areas. Aim: To document a new case presentation of PP caused by a low-carbohydrate restricted diet and discuss the comparison with CARP clinically, pathologically, and treatment modalities. Case report: A 15-year-old childhood male developed PP 3 weeks after self-initiating a low carbohydrate-restricted ketogenic diet for weight management. Clinically and histopathologically the lesion looks similar to CARP, treated successfully with re-introduction of high carbohydrates in his food, a short course of systemic steroids in combination with oral doxycycline capsules for the one-month duration. Conclusion: PP & CARP have been considered a spectrum of one disease, and PP is a pruritic variant from CARP caused by a low carbohydrate-restricted diet.

A review of the risk factors associated with juvenile-onset recurrent respiratory papillomatosis:genetic,immune and clinical aspects

Background Juvenile-onset recurrent respiratory papillomatosis(JoRRP)is one of the most common benign lesions of hyperplastic respiratory epithelial tissue in children and is predominantly caused by human papillomaviruses(HPVs)6 and 11.The clinical course of the disease is variable,and some patients even develop a malignancy.The purpose of this review was to summarize the related factors affecting the disease course in patients with JoRRP.Data sources We used databases,including PubMed and Google Scholar,to search for publications on factors associated with the genetic,immune,and clinical aspects of JoRRP.The most relevant articles to the scope of this review were chosen for analysis.Results Mother-to-child transmission is the most important mode of disease transmission;other factors,such as immune condition or genetic susceptibility,may be important determinants of JoRRP occurrence.Genetically,the presence of DRBI*0301 and HFV 6/11 E6/E7 is associated with a more severe disease.Immunewise,patients have an enhanced T helper 2-like response.In addition,regulatory T cells are enriched in tumors and may become one of the effective prognostic indicators.For clinical characteristics,patients infected with HPV-11 have more aggressive disease.However,compared with HPV type,age at first onset is a more important factor related to the aggressiveness of JoRRP.Furthermore,socioeconomic status may also affect the course.Conclusions Genetic,immune,and some clinical factors have been noted to play an important role in the course of JoRRP.Exploring definite influencing factors will be an important direction of research in the future.

Risk factors of juvenile onset recurrent respiratory papillomatosis in the lower respiratory tract

Background Juvenile onset recurrent respiratory papillomatosis （JORRP） is a relatively rare disease. It affects the larynx in most cases. Because reports on JORRP to the lower respiratory tract （LRT） are few, we investigated clinical features of patients with a history of JORRP to analyze the risk factors of this disease. Methods Data from 208 JORRP patients admitted to Beijing Tongren Hospital from January 2008 to December 2010 were reviewed. Gender, age at onset of symptoms, age at first surgery, duration of symptoms before the first surgical procedure, the number of surgical procedures, mean interval between surgical interventions, and the number of tracheotomies in patients with and without LRT spread of JORRP were analyzed. The data from patients with and without tracheotomy were recorded and compared. Two cases of primary tracheal papillomatosis were reviewed. Results Papillomas extension down to the LRT was observed in 46 children （22.1%）. Patients with LRT papillomatosis had a shorter time from the onset of the symptoms until the first surgery, required significantly more surgical procedures, and had a shorter mean surgical interval. Tracheotomy was performed in 13/162 （8.0%） children with laryngeal papillomatosis vs. 36/46 （78.3%） children with LRT papillomatosis. After tracheotomy, 36/49 （73.5%） children developed LRT papillomatosis and 10/157 （6.4%） children who did not have a tracheotomy developed LRT papillomatosis. Patients with tracheotomy required a significantly higher number of surgical procedures. The younger the patient had a tracheotomy, the longer the duration of cannulation was. Conclusions JORRP patients with LRT spread are prone to develop more aggressive disease. Tracheotomy resulted in a significant increase of LRT involvement.

Intraductal papillary neoplasm of the bile duct

Intraductal papillary neoplasm of the bile duct(IPNB)is a variant of bile duct carcinoma that is characterized by intraductal growth and better outcomes compared with common cholangiocarcinoma.IPNBs are mainly found in patients from Far Eastern areas,where hepatolithiasis and clonorchiasis are endemic.According to the immunohistochemical profiles of the mucin core proteins,IPNBs are classified into four types:pancreaticobiliary,intestinal,gastric,and oncocytic.Approximately 40%-80%of IPNBs contain a component of invasive carcinoma or tubular or mucinous adenocarcinoma,suggesting that IPNB is a disease with high potential for malignancy.It is difficult to make an accurate preoperative diagnosis because of IPNB’s low incidence and the lack of specificity in its clinical manifestation.The most common abnormal preoperative imaging findings of IPNB are intraductal masses and the involvement of bile duct dilation.Simultaneous proximal and distal bile duct dilation can be detected in some cases,which has diagnostic significance.Cholangiography and cholangioscopy are needed to confirm the pathology and demonstrate the extent of the lesions.However,pathologic diagnosis by biopsy cannot reflect the actual stage in many cases because different foci may be of different stages and because mixed pathologic findings may exist in the same lesion.Surgical resection is the major treatment.Systematic cholangioscopy with staged biopsies and frozen sections is recommended during resection to ensure that no minor tumors are left and that curative resection is achieved.Staging,histologic subtype,curative resection and lymph node metastasis are factors affecting long-term survival.

Adenoma of the Nipple, Mimicking Paget’s Disease of the Breast: Report of a Case

Nipple adenoma is a rare benign condition that simulates malignancy. A 37-year-old woman presented with unilateral bloody nipple discharge for 1-year duration followed by severe nipple erosion. As biopsy revealed nipple adenoma and therefore, complete local excision was done. The final histopathology showed florid papillomatosis which was adequately excised. Nipple adenoma although rare entity this should be included in the differential diagnosis of any nipple erosion such as carcinoma and Paget’s disease of the breast specially when associated with bloody discharge in premenopausal women.

Clinical Analysis of Hoarseness in Children as Seen in Otorhinolaryngology Department of a Tertiary Health Institution in North-West, Nigeria

Normal phonation is produced in the larynx by the blast of pulmonic air stream that vibrates the vocal cords. Hoarseness reflects changes in the structure or function of the vocal cords. Objective: This is to describe the clinical profile of hoarseness in children and aetiological factors in a tertiary health institution. Methodology: This is a retrospective eight-year (2006-2013) study of clinical records of patients with hoarseness as their main symptom seen in the ENT department of the Usmanu Danfodiyo University Teaching Hospital Sokoto. Results: Fifty-two children with hoarseness were studied. Thirty three (63.5%) were males, while 19 (36.5) were female with a male: female ratio of 1.7:1. The age range was 1 - 13 years with a mean age of 5.1 years. Age group 4 - 7 years (38.5%) were observed to be more affected with duration of hoarseness ranging from 1 day to 5 years. RRP 23 (44.2%) was the most common aetiology of hoarseness followed by foreign body 15 (28.8%) and Acute Laryngeal infections 9 (17.3%). Tracheostomy was done in 29 (55.8%) of patients. Two patients died post operatively due to improper tracheostomy tube care in the ward. Conclusion: With delayed presentation and its attendant grave sequelae, it has become imperative that persistent hoarseness in children lasting more than three weeks should be endoscopically evaluated by an otorhinolaryngologist.