Trends and Outcomes of Hospitalizations Due to Hemolytic Uremic Syndrome: A National Perspective.

BACKGROUND: Hemolytic uremic syndrome (HUS) is a rare but challenging disease with varying degrees of mortality and prognosis. We aim to evaluate the trends and outcomes of hospitalizations due to HUS by utilizing a large population-based dataset. METHODS: We derived a study cohort from the Nationwide Inpatient Sample (NIS) for the years 2007-2018. Our primary outcomes were in-hospital mortality, discharge disposition, and predictors of poor outcomes. We then utilized the Cochran Armitage trend test and multivariable survey logistic regression models to analyze the trends, outcomes, and predictors. RESULTS: A total of 8043 hospitalizations ranging from age zero to above 65 years of age occurred due to HUS from 2007-2018. The number of hospitalizations with HUS increased steadily from 528 in 2007 to 800 in 2013, but afterwards, we noticed a steady decline to 620 in 2018. Additionally, trends of in-hospital mortality slowly increased over the study period but we noticed a decline in the rate of discharge to skilled nursing facilities (SNFs). Furthermore, in multivariable regression analysis, predictors of increased mortality in hospitalized HUS patients were advanced age (95%CI: 1.221-1.686; p-value <0.0001) and requirement for dialysis (95%CI: 1.141-4.167; p-value: <0.0001). Advanced age >65 years (OR: 2.599, 95%CI: 1.406-4.803; p-value: 0.0023), as well as comorbidities such as diabetes mellitus and pulmonary circulatory diseases, which are under vascular events (OR: 1.467, 95%CI:1.075-2.000; p-value: 0.0156), were shown to have a higher rate of discharge to SNFs. Moreover, patients needing intravenous immunoglobulin (IVIG) and plasmapheresis had high odds of discharge to SNFs ((OR: 1.99, 95%CI: 1.307-3.03; p-value: 0.0013) and (OR: 5.509, 95%CI: 2.807- 10.809; p-value <0.0001), respectively), as well as smaller hospital bed size and hospital type (OR: 1.849, 95%CI: 1.142-2.993; p-value: 0.012). CONCLUSION: In this national representative study, we observed a total decrease in hospitalizations as well as discharge to SNFs; however we saw an increase in inpatient mortality. We also identified multiple predictors significantly associated with increased mortality, some of which are potentially modifiable and can be points of interest for future studies.

Clinical parameters predicting development of pulmonary malignancies in patients treated for head and neck squamous cell carcinoma.

BACKGROUND: As the locoregional control rates in head and neck squamous cell carcinoma (HNSCC) have increased, these patients may suffer distant metastasis in a higher proportion of cases. Clinicopathological characteristics allowing prediction of high-risk profile would allow adapting posttreatment surveillance to individual risk. METHODS: A retrospective review of all patients with HNSCC treated at the Jewish General Hospital, McGill University, Montreal, Quebec, Canada, between 1999 and 2008 was conducted for this study. RESULTS: The study included 428 patients with a mean follow-up of 65 months (±SEM 1.7). Eighty patients (18.6%) developed pulmonary malignancy during follow-up. In multivariate Cox-regression analysis, locoregional failure and current smoking were associated with higher risk of pulmonary malignancy (p < .001 and p = .008, respectively). CONCLUSION: Locoregional failure and smoking persistence are predictors of pulmonary malignancy in patients with HNSCC. © 2015 Wiley Periodicals, Inc. Head Neck 38: E1277-E1280, 2016.

Computerized tomography based tumor-thickness measurement is useful to predict postoperative pathological tumor thickness in oral tongue squamous cell carcinoma.

BACKGROUND: Tumor thickness has been shown in oral tongue squamous cell carcinoma (OTSCC) to be a predictor of cervical metastasis. The postoperative histological measurement is certainly the most accurate, but it would be of clinical interest to gain this information prior to treatment planning. This retrospective study aimed to compare the tumor thickness measurement between preoperative, CT scan, and surgical specimens . METHODS: We retrospectively included 116 OTSCC patients between 2001 and 2013. Thickness was measured on computer tomography imaging and again surgical specimens. RESULTS: The median age was 66 years. 62.8 % of patients were smokers with a mean of 31.4 pack-years. Positive nodal disease was reported in 41.2 %. Mean follow-up time was 33.1 months. The correlation between CT scan-based tumor thickness and surgical specimens based thickness was significant (Spearman rho = 0.755, P < 0.001). CONCLUSION: Tumor thickness assessed by CT scan may provide an accurate estimation of true thickness and can be used in treatment planning.

Laryngotracheal rhinosporidiosis.

Rhinosporidiosis is a chronic granulomatous disease caused by Rhinosporidium seeberi. It usually affects mucous membranes of the nose, nasopharynx, and ocular conjunctiva. Cutaneous, laryngeal, tracheal, genital, and bony dissemination is rare. Laryngotracheal involvement poses many diagnostic and therapeutic challenges. A 45-year-old South Indian man presented with complaints of a mass in both nostrils for 2 years, associated with progressive hoarseness of voice and difficulty in breathing for 6 months. Rhinosporidial lesions were seen bilaterally in the nasal cavity. Telescopic and fiberoptic laryngoscopic examinations showed reddish, strawberry-like masses with whitish spots on their surface involving the larynx and trachea. Computed tomography of the head and neck revealed soft-tissue mass lesions involving the bilateral nasal cavities and nasopharynx, extending to the oropharynx and involving the larynx and trachea. A preliminary tracheostomy was performed, followed by direct laryngoscopic excision of the laryngeal lesions and rigid-bronchoscopy-guided excision of the tracheal lesions. The patient was prescribed dapsone and advised to take it for 2 years. At 2 years of follow-up, there was no recurrence.

Embryogenic cervico-thyro-piriform tract.

Branchial cleft fistulae are rare congenital anomalies that arise from the abnormal persistence of branchial remnants. Branchial arch anomalies are rare. They usually present as a lateral neck mass or abscess in the form of acute suppurative thyroiditis. A complete fistula of the third arch is extremely rare. We describe such a case in a 13-year-old girl who presented with a small opening in the left lower neck, from which a mucoid discharge had been present since birth. The fistula was accompanied by recurrent neck swelling. Computed tomography with contrast injection into the external skin opening revealed a continuous tract that extended to the base of the piriform sinus. Total excision of the tract up to the piriform sinus with a left hemithyroidectomy was performed. At follow-up 28 months postoperatively, the patient exhibited no evidence of recurrence.

Giant tracheocele with multiple congenital anomalies.

Tracheocele--an outpouching of tracheal mucous membrane--is an uncommon entity. It can occur as a congenital or acquired form. The congenital entity remains mostly dormant until adulthood, and then it typically presents as a herniation with multiple air-filled sacs. The acquired form develops as the result of blunt trauma, recurrent pulmonary infection, intubation, instrumentation, or surgery, and it typically presents as a single paratracheal cavity. We present an extremely rare case of a tracheocele associated with multiple congenital anomalies involving the face, limbs, and heart.

Primary extranodal T-cell non-Hodgkin lymphoma of the tongue.

The most common sites of extranodal non-Hodgkin lymphoma (NHL) are the gastrointestinal tract and the head and neck region. Head and neck involvement accounts for 8 to 13% of all extranodal lymphomas. Primary NHLs of the oral cavity, especially in the tongue, are extremely rare, which makes it difficult to understand their biologic behavior. Extranodal NHLs of the tongue usually present as a nodular lesion of the B-cell type. The T-cell variant of tongue NHL with an ulcerated exophytic presentation is extremely rare. We report such a case in a 37-year-old woman.

Development of hemangioma in a tongue harboring long-standing angiokeratoma circumscriptum.

Angiokeratoma is a very rare vascular lesion of the papillary dermis. It is characterized by vascular ectasia with overlying epidermal hyperkeratosis. The systemic form of angiokeratoma is associated with Fabry disease and fucosidosis. There are several localized forms. Tongue involvement is uncommon. Hemangiomas are tumors made up of capillaries; they grow by active endothelial proliferation as opposed to expansion of vascular spaces in vascular malformations. Lingual hemangiomas are usually indolent, but they can cause cosmetic deformities, recurrent hemorrhage, and functional problems with speaking, mastication, and deglutition. We report a case of angiokeratoma of the tongue with an underlying hemangioma in a 30-year-old woman. Angiokeratomas have been reported to develop over arteriovenous malformations and in the area of lymphangioma circumscriptum following repeated local trauma. To the best of our knowledge, the development of a lingual hemangioma in a patient with long-standing angiokeratomatous lesions has not been previously reported in the literature.

Primary frontal sinus carcinoma with extradural anterior cranial fossa involvement.

Paranasal sinus carcinoma is rare, with an estimated annual incidence of less than 1 per 100,000 population. Primary frontal sinus involvement is extremely rare, accounting for only 0.3% of all paranasal sinus malignancies. A frontal sinus cancer may be mistaken for a mucocele, pyocele, or osteomyelitis. We report the case of a 48-year-old woman with a carcinoma that originated in the frontal sinus. Computed tomography and magnetic resonance imaging demonstrated bony destruction with intracranial extension but no involvement of the bulbus. The patient underwent a frontal craniotomy, tumor excision, and postoperative radiotherapy. One year later, she remained symptom-free.

Microbiological profile with antibiotic sensitivity pattern of cholesteatomatous chronic suppurative otitis media among children.

INTRODUCTION: Chronic suppurative otitis media (CSOM) is the most common cause of childhood hearing impairment in the developing countries and atticoantral type is associated with increased incidence of intracranial and extracranial complications. This study was undertaken to define the microbiology of atticoantral type of chronic otitis media and the antibiotic sensitivity pattern, thereby reducing the potential risks of complications. MATERIALS AND METHODS: A retrospective study was done in the Department of Otolaryngology, JIPMER, Puducherry from the year August 2003 to October 2009 using the medical record department database to retrieve the patient details. During this study period, 223 children with atticoantral type CSOM consisting of 126 males and 97 females with an age range of 1-14 years were assessed. Patients with persistent otorrhea for more than 3 months with atticoantral type of chronic otitis media were selected. The exudates were collected under sterile conditions and inoculated onto culture media; bacterial growth and antibiotic sensitivity pattern were studied. RESULTS: Nine species of micro organisms were isolated from the middle ear aspirate, Pseudomonas aeruginosa being the most predominant isolate constituting about 32% (72 discharging ears) of the total isolates followed by Proteus mirabilis (20% of isolates) and Staphylococcus aureus (19% of isolates). Gram negative organisms accounted 58% of total isolates and gram positive organisms constituted 22% isolates. Candida albicans and methicillin resistant S. aureus were identified in 4% and 2% of isolates, respectively. 100% of Pseudomonas isolates showed susceptibility to ceftazidime and a high sensitivity (92% of isolates) to ciprofloxacin and 88% isolates were sensitive to amikacin. 100% of P. mirabilis isolated from inoculates showed sensitivity to ceftazidime and ciprofloxacin. It also showed 87-97% sensitivity to ceftriaxone, amikacin and ampicillin. All (100%) of the Staphylococcus isolates were sensitive to vancomycin and 84-86% were sensitive to ciprofloxacin and erythromycin. In general, gram negative organisms showed increased sensitivity to ceftazidime, ciprofloxacin and amikacin, while gram positive organisms to vancomycin, erythromycin and ciprofoxacin. CONCLUSION: Continuous and periodic evaluation of microbiological pattern and antibiotic sensitivity of cholesteatomatous CSOM is necessary to decrease the potential risks of complications by early institution of appropriate systemic and topical antibiotic alongside mastoid exploration. We believe that our data may contribute to an effective medical management of chronic suppurative otitis media with cholesteatoma. Since the most common organisms in our clinical set up being P. aeruginosa, P. mirabilis and S. aureus, which showed a percentage susceptibility of 100% to ceftazidime and vancomycin, thus making it an empirical antibiotic combination therapy of choice in the recent times.

Recurrent neck infection with branchial arch fistula in children.

OBJECTIVE: Acute suppurative neck infections associated with third or fourth branchial arch fistulas are frequently recurrent. Third and fourth branchial arch anomalies are much less common than those of second arch and usually present with left thyroid lobe inflammation. The authors present their experience with 15 cases of pyriform sinus fistulae (PSF) of third branchial arch origin and 3 cases of fourth arch origin, all of which presented as recurrent neck infection mainly on the left side. METHODS: A retrospective review of 18 cases of third and fourth arch fistulae treated at JIPMER from 2005 to 2010. This study includes 18 patients with PSF diagnosed by the existence of fistulous tract radiologically and intraoperatively with pathological correlation. Neck exploration with excision of tract and left hemithyroidectomy was performed in all cases. RESULTS: The patients consisted of 7 males and 11 females, and the ages ranged from 3 to 15 years. All of them presented with recurrent episodes of neck infection. Investigations performed include computed tomography (CT) fistulography, barium swallow and ultrasound which were useful in delineating pyriform sinus fistulous tract preoperatively. All cases were on the left side and the fistula was identified by barium swallow in 14 cases (80%), while intraoperative and pathologic confirmation of the tract was possible in all cases (100%). Neck exploration with an emphasis on complete exposure of the recurrent laryngeal nerve and exposure of the pyriform sinus opening to facilitate complete fistulous tract excision with left hemithyroidectomy was successful in all patients. A follow up period of 1-3 years showed no recurrence. CONCLUSION: Recurrent neck infection in a child should alert the physician to the possibility of an underlying pyriform sinus fistula of branchial origin and CT fistulography should be performed after the resolution of the neck infection to delineate the tract anatomically.

Longstanding malformation of right sided pinna in an elderly man.

The pinna is the second most common site for external ear vascular malformation in the head and neck. These malformations are relatively uncommon in adults and can pose difficult therapeutic challenges. We hereby present a case of a 69-year-old man with a congenital lesion in the right pinna consistent with an arteriovenous malformation. The lesion was complicated by ulceration and bleeding for 6 months prior to presentation. Resection of pinna was carried out, and satisfactory functional and esthetic results were obtained. There was no recurrence at 22 months of regular follow up.

Giant congenital auricular arteriovenous malformation.

Arteriovenous malformation (AVM) is an uncommon vascular anomaly usually present in intracranial location, however may be present in other sites of the body, mostly in the head and neck region. The lesion may be present since birth or caused by trauma, but may become obvious during puberty or pregnancy. The diagnosis can be confirmed by selective angiography. Treatment usually includes super selective embolization followed by surgical excision within 48 h. We hereby present a rare case of a grotesquely disfiguring congenital auricular arteriovenous malformation in a 77-year-old elderly man, which has recently been complicated by ulceration, infection and hemorrhage in the past 6 months. The pinna showed a 15 cm x 8 cm pulsatile, erythematous, ulcerative hemorrhagic swelling which on angiography revealed tortuous superficial temporal and occipital arteries supplying the lesion. Complete auricular excision with split skin grafting was carried out. The option of preoperative embolization was not considered due to his renal dysfunction and the high dye load required for embolizing the fast flow arteriovenous malformation.

Cervical infection secondary to pyriform sinus fistula of branchial origin.

Complete third branchial arch anomalies are rare and have been described only in case reports, affecting mainly children and typically presenting as a cervical inflammatory process. Anomalies of the third and fourth branchial apparatus, though rare, usually present as sinuses/incomplete fistulas of pyriform sinus or recurrent suppurative thyroiditis. A 6-year-old girl presented with a small opening on the left side of her anterior neck, which had been present since birth and was associated with recurrent infection. She had no history of incision and drainage of swelling. Computed tomography with contrast injection into the cervical opening revealed a fistulous tract extending from the cervical neck skin to the pyriform fossa. Complete excision of the fistulous tract and left hemithyroidectomy were performed. There was no recurrence at 22 months of follow-up.

True thyroglossal fistula.

Thyroglossal duct anomalies are the most common malformations in the neck and constitute 70% of all the congenital cervical masses. They are more common in the pediatric population under 5 years of age, and 60% of lesions are diagnosed before the age of 20. They represent remnants from the embryological migration of thyroid tissue from foramen caecum to the thyroid fossa. The thyroglossal duct cyst may rupture spontaneously and present as a draining sinus, which has been erroneously called a thyroglossal fistula, although communication with foramen caecum is extremely rare. We hereby present a case of true thyroglossal fistula in a 21-year-old male with a fistulous communication between the neck skin and foramen caecum.

Hypohidrotic ectodermal dysplasia with atrophic rhinitis and nasal myiasis.

Hypohidrotic/anhidrotic ectodermal dysplasia is a rare inherited disorder characterized by hypohidrosis/anhidrosis, hypotrichosis, dysodontia and heat intolerance. Most common mode of transmission is X-linked recessive, showing complete expression in males, and only partial manifestations in the female carrier heterozygotes. Features like atrophic rhinitis, nasal and aural myiasis, syndactyly, cleft lip and/or palate, mental retardation and immunodeficiency are uncommonly seen in this syndrome. We hereby report a case of hypohidrotic ectodermal dysplasia with unusual features of atrophic rhinitis and nasal myiasis.