Pai syndrome: a report of a case and review of the literature.

Pai syndrome is a rare congenital disorder first described in 1987. The main clinical features of the syndrome include median cleft of the upper lip, intra-cranial lipoma, and cutaneous polyps. Only four cases have been described previously. This is the fifth who is a twin of Arabian descent to be reported. Full description of the clinico-pathological features and a review of the relevant medical literature is presented. To the best of our knowledge, this is the first case of Pai syndrome in a twin in the English literature.

Pharyngeal tuberculosis.

PURPOSE: To increase awareness of tuberculosis (TB) as an important differential diagnosis of lesions in the pharynx and discuss its presentation. PATIENTS AND METHODS: The study included nine patients (2 males and 7 females); each with a diagnosis of primary pharyngeal tuberculosis (PTB). Of these, 3 had nasopharyngeal TB, 5 had tonsillar TB, and 1 had hypopharyngeal TB. The diagnostic criteria were either positive culture, positive smear, or histopathologic features of caseating granuloma consistent with TB in the biopsy specimen and a response to treatment. RESULTS: All patients had primary infection. The main presenting symptom in all nasopharyngeal TB was neck mass, whereas tonsillar TB patients presented with sore throats or discomfort. Dysphagia was the presenting symptom in hypopharyngeal TB. Six patients (3 with nasopharyngeal TB and 3 with tonsillar TB) had cervical adenopathy. The smear for acid-fast bacillus was positive in 4 patients (44.4%); culture was positive in 2 patients (22.2%). Histopathologic features of caseating granuloma, consistent with TB, were positive in all patients who received antituberculous medications. CONCLUSION: Otolaryngologists should consider pharyngeal TB as one of the differential diagnosis of lesions of the pharynx especially in those countries where TB is endemic.

Mycobacterial infection of the head and neck: presentation and diagnosis.

OBJECTIVE: To increase the awareness of the different presentations of head and neck mycobacteriosis, especially in apparently immunocompetent patients, and discuss its diagnostic difficulties. STUDY DESIGN: A retrospective analysis from an otolaryngology service in a university hospital. METHODS: Retrospective analysis of head and neck mycobacterial infections diagnosed at King Abdul Aziz University Hospital, Riyadh, Saudi Arabia, between 1983 and 1997. RESULTS: The study group consisted of 75 apparently immunocompetent patients (41 female and 34 male patients). The mean age at presentation was 32.6 years. Mycobacterial cervical lymphadenitis constituted 62 cases (82.7%); pharyngeal tuberculosis, 8 cases (10.7%); and sinonasal disease, 5 cases (6.7%). The presenting complaints were neck mass in 86.3%, nasal obstruction in 5.3%, sore throat or discomfort in 5.3%, and external nasal lesion in 4% of patients. There was a history of previous tuberculosis in 10 patients (13.3%), and 5 patients (6.7%) had history of contact with infected individuals. Constitutional symptoms were recorded in 24 cases (32%). The diagnosis was established on the basis of positive smear in 26.5% of patients and on the basis of positive culture in 33.3% of patients, and caseating granuloma consistent with tuberculosis was found in all patients. CONCLUSION: The present study is a report of a series of cases of mycobacterial infection of the head and neck in non-immunocompromised patients. It presents diagnostic and therapeutic problems. Awareness of the different presentations, as well as development of modern, efficient diagnostic methods, is required.

Association of rhinoscleroma with rhinosporidiosis.

Rhinoscleroma caused by the bacillus Klebsiella rhinoscleromatis and rhinosporidiosis caused by the fungus Rhinosporidium seebri are rare, specific nasal infections, both of which have a certain geographical distribution. To the best of our knowledge no association between them has been reported in the international literature. We have documented such an association in two male Indian patients aged-32 and 27 years, respectively-both presenting with unilateral blood-stained discharge and nasal blockage. They showed strawberry-like polypoidal masses, and histological examination confirmed the diagnosis. Klebsiella rhinoscleromatis was cultured twice in the first case. The patients were treated with complete excision and a long course of septrin, for which Klebsiella rhinoscleromatis is sensitive. The purpose of this paper is to report the first association of these two granulomatous infections, to show the impact of immigration on the differential diagnosis, and to review the relevant literature.

Lobular capillary haemangioma (pyogenic granuloma) of the nose.

OBJECTIVE: To present a number of cases with lobular capillary haemangioma (pyogenic granuloma) of the nose; and to discuss the clinical and histopathological diagnosis of this disorder. METHODS: A clinicopathological study of patients diagnosed with nasal lobular capillary haemangioma who were treated at King Abdel Aziz University Hospital, Riyadh from 1986 to 1995. RESULTS: The study group consisted of 12 patients (four males and eight females ranging in age from 17 to 65 years; mean 30.1 years). Clinically, most patients presented with epistaxis and a rapidly growing unilateral haemorrhagic mass. Most lesions were located on the septal mucosa. The clinical impression was misleading in some cases. The histological diagnosis was based on the lobular arrangement of capillaries. One case was initially misdiagnosed as angiofibroma. Excisional biopsy was the mainstay of treatment. One instance of recurrence was recorded. CONCLUSION: This uncommon lesion should be considered in the differential diagnosis of a rapidly growing haemorrhagic lesion within the nasal fossa. Clinical and histological diagnostic pitfalls occur frequently.

Mastoid abscess: underlying disease and management.

Despite a reduction in the incidence, subperiosteal mastoid abscesses (MAs) are still seen today with significant potentials for morbidity and even mortality. I present a series of 19 patients, seen over a 5-year period, with particular emphasis on the underlying disease and management. Ten abscesses (52.6%) succeeded acute suppurative otitis media (AOM); and nine (47.4%) resulted from chronic suppurative otitis media (CSOM). Eighty percent of those with AOM were younger than 10 years, whereas 77.8% of those with CSOM were older than 10 years. Fifty percent of the MAs that resulted from AOM had cortical mastoidectomy. The other five patients were treated with a less invasive method: antibiotics and incision and drainage without mastoid surgery. In eight MA cases caused by CSOM, the management of the abscess as well as the underlying disease was performed in one stage. Two-stage treatment occurred in one case. The study supports the recently recognized increasing role of CSOM in the etiology of MA, especially in adolescents and young adults. The controversy over the management of this disorder is discussed.

Surgery for postintubation tracheal and tracheosubglottic stenotic lesions.

Postintubation tracheal stenosis is a recognized problem. Although its incidence has recently decreases, it is still a difficult complication to treat. We have reviewed our experience with 10 patients with tracheal stenosis over the last five years between 1990 and 1995. There were seven male and three female patients with an average age of 14.2 +/- 4 years (range 6 to 48 years). Resection and reconstruction with primary anastomosis was performed in seven patients, while conservative treatment with dilatation was performed in two patients. One patient refused surgery. Operations performed included resection of tracheocricoid segment with tracheothyroid anastomosis (N=3) and tracheal resection with end-to-end anastomosis (N=4). The resected airway segment ranged from 3 cm to 6 cm. In view of the intense inflammatory and fibrotic process in and around the stenotic segment, the practice of tracheostomy for the relief of postintubation acute tracheal obstruction should not be taken lightly, as it adds not only to the severity of the inflammatory process, but also increases the length of the tracheal segment to be resected. Postoperatively, all patients were extubated; this was accomplished by the end of surgery in six patients, while the seventh patient was extubated three weeks later. There was no mortality in this series. When normal functional activity and airway patency were taken as two parameters to judge the outcome of surgery, results were good in six (86%) patients and satisfactory in one. These results support the validity of one-stage reconstruction approach as one alternative for the treatment of postintubation tracheal and tracheosubglottic stenotic lesions.