Chronic rhinosinusitis with nasal polyposis and biological agents: the ARIA-ITALY Survey.

Summary: Background. Chronic rhinosinusitis (CRS) is an inflammatory disease that affects the nasal mucosa and the paranasal sinuses. CRS can be associated by nasal polyposis (CRSwNP phenotype) in up to 30% of patients and it is frequently associated with bronchial asthma. CRSwNP shows predominantly an underlying activation of type 2 inflammatory pathways with the involvement of eosinophils, IgE, interleukin (IL)-4, IL-5 and IL-13. Biological drugs that target these inflammatory cytokines are currently a therapeutic option recognized by guidelines for the treatment of uncontrolled form of the disease. Methods. As part of the activity of the "ARIA-Italy" working group, a panel of 255 Italian Ear, Nose and Throat (ENT) specialists, pneumologists and immuno-allergologists actively participated in this national survey and answered a series of questions geared toward understanding the main criteria for patient characterization and therapeutic decision, highlighting multidisciplinarity, and the implementation of the management of CRSwNP patients, as a part of the precision medicine concept and the appropriate use of the biologicals. Results. Two hundred and fifty-five experts and specialists participated in the survey. Conclusions. The results of this survey obtained from an extensive number of active specialists throughout Italy allow some important concluding remarks to be drawn. The main points of agreement were that multidisciplinary care teams provide many benefits but that, once the team is established, meetings and communication between members must be coordinated. Finally, the dissemination of national disease registries and the continuous updating of guidelines and position papers related to CRSwNP and comorbidities should be encouraged.

Facial ridge management in canal wall down tympanoplasty for middle-ear cholesteatoma.

OBJECTIVE: To estimate whether leaving a high facial ridge during canal wall down tympanoplasty increases the risk of residual cholesteatoma. METHODS: In this retrospective case review, 321 patients treated with primary canal wall down tympanoplasty for middle-ear cholesteatoma were divided into a completely lowered facial ridge group and a non-completely lowered facial ridge group. Factors affecting facial ridge management, residual disease rate and disease-free survival were analysed. RESULTS: Residual disease rates were 10.8 per cent in the non-completely lowered facial ridge group and 16.6 per cent in the completely lowered facial ridge group (p = 0.15). Localisation at sinus tympani, mesotympanum or supratubal recess, pre-operative extracranial complications, and destroyed ossicular chain or fixed platina were associated with a completely lowered facial ridge. Residual disease rates and disease-free survival did not significantly differ between the groups. CONCLUSION: Facial ridge can be managed according to cholesteatoma extension. The facial ridge can be maintained high if the cholesteatoma does not involve sinus tympani, mesotympanum or supratubal recess, without increasing the risk of residual disease.

Post-operative outcomes of different surgical approaches to oropharyngeal squamous cell cancer: a case-matched study.

OBJECTIVE: To compare the post-operative outcomes of transoral laser microsurgery, lateral pharyngotomy and transmandibular surgery in oropharyngeal cancer management. METHODS: Records of 162 patients treated with transmandibular surgery, transoral laser microsurgery or lateral pharyngotomy were reviewed. The transoral laser microsurgery cohort was matched with the lateral pharyngotomy and transmandibular surgery cohorts for tumour stage, tumour subsite and human papilloma virus status, and the intra- and post-operative outcomes were compared. RESULTS: Duration of surgery and hospital stay were significantly longer for transmandibular surgery. Tracheostomy and nasogastric feeding tube rates were similar, but time to decannulation and to oral feeding were longer in the transmandibular surgery group. Transmandibular surgery more frequently required flap reconstruction and had a greater complication rate. Negative margins were fewer in the lateral pharyngotomy group than in the transoral laser microsurgery and transmandibular surgery groups. CONCLUSION: In comparison with transmandibular surgery, transoral laser microsurgery and lateral pharyngotomy were associated with fewer complications and faster functional recovery. Lateral pharyngotomy had a higher rate of positive margins than transoral laser microsurgery, with a consequently greater need for adjuvant therapy. Many patients are nonetheless unsuitable for transoral surgery. All these factors should be considered when deciding on oropharyngeal cancer surgical treatment.

Therapeutic strategies in the treatment of Menière's disease: the Italian experience.

PURPOSE: Ménière's disease (MD) is an inner ear disorder of unknown etiology, whose pathological substrate is the endolymphatic hydrops. Different treatments have been proposed; however, evidence of their effectiveness is lacking. The aim of this study was to evaluate by a questionnaire which medical and surgical treatments are used in Italy for the treatment of MD and to compare them with those proposed in other countries. METHODS: An electronic questionnaire of 40 questions was formulated and sent to Italian otolaryngologist (ENT) divided into two groups: Group 1 ("generalists" 60.8%) and Group 2 ("neurotologist- NO" 39.2%). RESULTS: One hundred and twenty five ENT replied. Treatment of the acute phase, apart from symptomatics, was based on diuretics that are prescribed by 83.5% of respondents, steroids, prescribed by 66.7%, and vasodilators, prescribed by 22%. In the intercritical phase, 87.2% of respondents recommended low-salt diet, 78.4% of respondents prescribed betahistine, and 52.8% diuretics. Statistical analysis did not show correlation neither with the declared specialization nor with the number of patients treated. In case of failure of medical treatment, IT gentamicin was suggested by 48.8% of the respondents and IT steroids by 40.8%. Statistical analysis showed that generalists prefer IT steroids and NO IT gentamicin (p 0.019). In case of failure of both medical treatment and IT treatment, vestibular neurectomy was indicated by 58.4% of the respondents, 6.4% indicated endolymphatic sac surgery, and 2.4% surgical labyrinthectomy. CONCLUSION: In Italy, the treatment of MD stand on a gradual approach that starts from the dietary-behavioral changes and a pharmacological therapy based on betahistine. In refractory cases, IT treatment initially with steroids and, therefore, with gentamicin allows the control in vertigo in the majority of cases. In case of failure of IT treatment, VNS is the surgery of choice.

Primary squamous cell carcinoma of the external auditory canal: surgical treatment and long-term outcomes.

This study was conducted on patients with squamous cell carcinoma of the external auditory canal and temporal bone treated with surgery alone or surgery plus postoperative radiotherapy. It was designed as a retrospective investigation with complete long-term follow-up covering the years from 1983 to 2008. The setting was a tertiary referral centre. Forty-one consecutive cases underwent surgery involving en bloc lateral or subtotal temporal bone resection, parotidectomy and neck dissection plus radiotherapy in advanced cases. The Pittsburgh staging system was adopted. No cases were lost to follow-up, which ranged from 2 to 220 months, while for survivors ranged from 60 to 220 months and included clinical examinations and imaging. Outcome was expressed as NED (no evidence of disease), DOC (dead of other causes), DOD (dead of disease), AWD (alive with disease), disease-free survival (DFS) and disease-specific survival (DSS). Results were expressed with raw data and Kaplan Meyer curves. Patients with T1 and T2 disease had a DFS of 67% and a DSS of 92%. For T3 and T4 cases, the DFS was 41% and DSS was 48%. All treatment failures were due to local recurrences. The cases classified as T4 because the lesion extended from the cartilage canal to the periauricular soft tissues, or from the anterior wall to the parotid space, had a better outcome than the other T4 cases: this different prognosis suggests the need to stage tumours differently. Nodal disease coincided with a worse outcome due to local recurrence.

Spontaneous ICA rupture: a severe late complication after giant nasopharyngeal angiofibroma resection.

Juvenile nasopharyngeal angiofibroma (JNA) is a benign vascular tumor occurring in young males. Surgery for giant JNA is a complex procedure, with a high risk of major complications. We report a rare case of massive epistaxis in a 15-year-old boy resulting from spontaneous rupture of the intracavernous tract of the internal carotid artery 20 days after resection of a giant JNA by midface degloving. The event was managed by an emergency arteriography with coils selectively deployed to occlude the vessel and to stop hemorrhage. This treatment has been shown to be effective in producing immediate hemostasis and stable long-term occlusion.

Petrous apex arachnoid cyst: a case report and review of the literature.

Cholesterol granuloma and cholesteatoma are the two most common destructive lesions of the petrous apex. Arachnoid cyst is much less common. These three expansile lesions are often indistinguishable on clinical grounds. Accurate pre-operative radiological diagnosis on computed tomography scan and magnetic resonance imaging is important in order to plan the appropriate treatment. Pre-operative radiological differential diagnosis between primary cholesteatoma of petrous apex and a intrapetrous arachnoid cyst remains a significant problem. The following aspects need evaluation for recognition of intrapetrous arachnoid cysts: 1) an awareness of their existence, 2) homogeneous signal on T1 and T2 weighted images, closely resembling cerebro-spinal fluid signal, 3) special heavily weighted T2 images on magnetic resonance imaging: fluid-attenuated inversion recovery imaging, 4) careful correlation of clinical-radiological data. Symptomatic arachnoid cysts are best treated with conservative drainage surgery through middle cranial fossa. A case of a petrous apex arachnoid cyst is reported which has been radiologically mistaken for a primary cholesteatoma and operated through an infratemporal fossa approach type B. The patient (40-year-old female) came to our attention with right trigeminal pain which had been present for one year and dizziness. Neurotologist and skull-base surgeons should include arachnoid cyst as a rare possibility in the evaluation and treatment of petrous apex cystic lesions.

Prolonged hyperinsulinemic hypoglycemia in a small for date preterm.

Hyperinsulinism is an important cause of hypoglycemia in early infancy. Many forms of hyperinsulinemic hypoglycemia are described: transient, prolonged, persistent. Transient forms are well recognized in infants of diabetic mother; prolonged forms are responsible for the hypoglycemia in small-for-date (SGA) infants and asphyxiated newborns. Persistent hyperinsulinemic hypoglycemia occurs in a group of congenital disorders associated with an abnormality of beta-cell regulation throughout the pancreas. Accurate diagnosis and treatment are essential in all various forms of hyperinsulinism also because newborns are at high risk of permanent brain damage. We report a case of prolonged hyperinsulinemic hypoglycemia in a SGA preterm, immediately treated with a high dose of glucose and glucocorticoid and then with diazoxide. Hypoglycemia was continued until 2 months of age when it resolved spontaneously and completely.

Paralytic ileus in a mechanically ventilated preterm infant treated with fentanyl.

The administration of fentanyl for sedation of ventilated newborns can induce several side-effects such as hypertension, respiratory muscle rigidity and, as shown in this report, decreased gastrointestinal motility. We report a case of paralytic ileus in a ventilated preterm infant who was given fentanyl in the first 24 hours of life. To our knowledge, the association of paralytic ileus with fentanyl has not been reported previously in full-term or preterm infants. This study indicates that early recognition is required to shorten the delay in diagnosis.

Anterior approaches in juvenile nasopharyngeal angiofibromas with intracranial extension.

Although surgery is regarded as the mainstay of treatment for juvenile nasopharyngeal angiofibromas (JNAs), ancillary treatment modalities such as radiotherapy and on rare occasions chemotherapy are still recommended by many for intracranial extension with apparent radiologic involvement of the cavernous sinus and internal carotid artery. Further, most authors undertaking surgical excision of this subgroup of patients would recommend a lateral or combined frontal and lateral approach for its removal. In a series of 49 cases of JNA, 14 were found during surgery to have intracranial extradural extension; the anterior approach was used for their removal. Although in these cases, on radiography the cavernous sinus often looked to be invaded and the internal carotid artery was displaced superolaterally, there was no difficulty in establishing a plane of dissection. Total removal was achieved in 11 of the 14 cases with a single-stage procedure. Of the 3 cases with residual tumor, only 1 occurred intracranially. Removal was achieved by a subtemporal approach in this case. For the extracranial residual tumors 1 required a midface degloving and the other, with a 1-cm residual tumor in the nasopharynx, has been treated conservatively for 6 years with no evidence of growth. No deaths or significant complications have occurred, and radiotherapy has not been required. We conclude that JNAs are tumors with a predilection for spread but that rarely invade dura, acting instead to displace it. We believe that surgery is the method of choice for treating these lesions and that an anterior surgical approach with microsurgical techniques should be used in the first instance. In the last 2 cases we preferred a midface degloving technique to avoid facial scarring and because this approach allows a widening of the surgical field if needed by the performance of bilateral maxillary free bone flaps. On the rare occasion that a lateral approach, with its attendant permanent conductive hearing loss, is found to be necessary for total tumor removal, this can be done as a staged procedure. This may be necessary when the tumor has spread lateral to the horizontal internal carotid artery.

A modified retrosigmoid approach for direct exposure of the fundus of the internal auditory canal for hearing preservation in acoustic neuroma surgery.

OBJECTIVE: This is a clinical report on a modified retrosigmoid approach with direct exposure of the fundus of the internal auditory canal for hearing preservation in acoustic neuroma surgery. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center of an ear, nose, and throat department in a public hospital. PATIENTS: One hundred fifty consecutive procedures were reviewed, including 61 males and 89 females with an age range of 13 to 69 years and a mean age of 47 years. There were 15 patients with tumor occupying solely the internal auditory canal and 135 patients with extension into the cerebellopontine angle with an extrameatal diameter of up to 52 mm and a mean of 11.5 mm. INTERVENTION: The retrosigmoid approach included a wide craniotomy, a perimeatal petrous bone removal up to the blue line of the labyrinth, and a direct exposure of the fundus at the orifices of the facial and cochlear nerves. The quadrant of the superior vestibular nerve remained unexposed. MAIN OUTCOME MEASURES: Hearing was measured according to the American Academy of Otolaryngology-Head and Neck Surgery criteria for reporting results of hearing preservation and by comparison with the preoperative level. Facial nerve function was measured using the House-Brackmann grading. The radicality of tumor removal was investigated with mid- to long-term magnetic resonance imaging (MRI). RESULTS: Measurable hearing was preserved in 45.3%, and in 32.4% of these cases, it was within 15 dB/15% discrimination. Grade 1 or 2 facial function was preserved in 85.3%. MRI follow-up revealed a 3.3% tumor residual or regrowth in the complete series. No residual tumor was found at the 3-year MRI in the last series of patients operated on with direct control of the fundus. CONCLUSIONS: This modified retrosigmoid approach permits the direct exposure of the facial and cochlear quadrants of the fundus. This allows tumor dissection under direct visual control. Removing the tumor from the vestibular quadrant of the fundus is done blindly in a minority of cases and carries a minimal risk of residual tumor. This technique requires only conventional equipment and skills of neurotology.

[Hepatic and pancreatic disease in patients with acquired immunodeficiency syndrome (AIDS)]. / Patologie epatiche e pancreatiche in pazienti con sindromi da immunodeficienza acquisita (AIDS).

AIDS is frequently expressed through gastrointestinal o abdominal symptoms. In addition, patients with AIDS or ARC frequently have hepatic and biliary symptoms, while pancreatic alterations are found in 4-30% of patients hospitalised for AIDS. Since AIDS patients are immunodepressed, they are subject to opportunistic infection often multifocal and the pathological processes can be present simultaneously. About 2/3 of patients have enlarged liver, steatosis, splenomegaly, lymphoadenopathy, cholecystic and biliary tract abnormalities, alterations of liver function tests, and abdominal discomfort in the upper right quadrant. Jaundice is rare and hepatic failure is not common. Hepatic biopsy is often necessary to establish the diagnosis. The hepatic localisation of an opportunistic pathogenic agent is generally a sign of systemic dissemination which is expressed as granulomatous hepatitis (atypical mycobacteria, frequently mycobacterium avium, or M. tuberculosis representing the reactivation of latent diseases), peliosis hepatis, infection from CMV, HSV, EBV, Pneumocystis carinii, and mycotic infections. Coinfections with the hepatic virus (HBV, HDV, HCV) are also often present. Pharmacological damage may also be present (mainly caused by antibiotic therapies). Neoplasia are rare (hepatic Kaposi's sarcoma associated with cutaneous and gastrointestinal manifestations, or generally metastatic lymphoma). Damage of the biliary tract usually develops after other manifestations of the illness; the most frequent pictures are cholestatic syndromes and cholangitis, while cholecystitis and jaundice are rare. Pancreatic lesions are generally asymptomatic. They are diagnosed during autopsy and are caused principally by opportunistic agents.

[Urinary tract ultrasonography in newborns with late oligohydramnios]. / Indagine ecografica dell'apparato urinario in neonati con oligoidramnios tardivo.

BACKGROUND AND AIM: The aim of this study was to evaluate the value of neonatal renal ultrasonography in the diagnosis of urinary tract malformation pathologies in newborns from pregnancies complicated by late oligohydramnios. METHODS: During the period January 1994-October 1997, 119 newborns from pregnancies complicated by oligohydramnios occurring in the third trimester of pregnancy underwent ultrasonography of the urinary tract at our Centre. All newborns had previously undergone prenatal ultrasonography. RESULTS: Ultrasonography revealed calicopyelic dilatation < 10 mm in 7 newborns (5.8%), 5 of whom had already been reported at the prenatal ultrasonography. One new-born (0.8%) also showed the presence of an ectopic kidney that had not been diagnosed at the prenatal scan. By the next follow-up all the calico-pyelic dilatations had become normal. CONCLUSIONS: Neonatal ultrasonography did not show any alterations to the urinary tract that might be responsible for the reduction of amniotic liquid. We therefore feel that is useful to perform neonatal ultrasonography in those situations in which the onset of oligohydramnios occurred in the second trimester of pregnancy and in cases in which no scan was performed during the prenatal period.

Renal blood flow velocity in preterm and term neonates during the fourth day of life: changes in relation to gestational age and birth weight.

Measurements of renal artery blood flow velocity have been previously reported during prenatal life and during the first month of life in neonates. Since renal flow velocity increases during the first 3 days of postnatal age we determined, during the fourth day of life, the changes of renal blood flow velocity in relation to gestational age and birth weight in 109 healthy preterm and term infants with gestational age between 26 and 42 weeks and birth weight between 750 and 3,730 g. Renal arteries were insonated about 3 mm from the abdominal aorta, by placement of a microconvex 7-MHz transducer below the costal margin in the dorsolateral area of the right or left flank. Mean flow velocity significantly increased with advancing of gestational age and birth weight. This study provides normative data for renal artery blood flow velocity in healthy preterm and term infants during the fourth day of life.

Acardiac twins. Two case reports.

The acardius syndrome is a rare complication of monozygotic twin pregnancies, occurring in 35,000 births. Development results from arterio-arterial and veno-venous anatomoses leading to predominance of one of the twins. The acardiac twin is grossly abnormal and the outcome is invariably fatal. The normal twin provides circulation for itself as well as the acardiac and the perinatal mortality is about 50-70%, as the result of congestive heart failure. Conservative therapy may be important for congestive cardiac failure in the normal twin. Surgical therapy is direct to maintain the pump fetus by interrupting the vascular communication between the twins. We present two cases of acardiac twin prenatally diagnosed by ultrasound and successfully managed by conservative therapy.

[The study of the modification of the natural resonance of the external ear following tympanoplasty]. / Studio delle modificazioni della risonanza naturale del C.U.EST. a seguito di interventi di timpanoplastica.

The shape, position and amplitude of the natural resonance curve of external ear is related to sensation evoked in a subject by sound coming from the external environment. The involution of this curve varies from individual and is the sum of the effects provoked on the incident sound by the head, auricle and the conformation of canal (EEE = External Ear Effect). Natural amplification supplied by the system allows the individual to distinguish natural sound, whose being natural make them pleasant to hear. Any interventions that modifies the anatomy of the auricle and/or the canal activity alternates the REUR (Real Ear Unaided Response) to some degree. Restoration of basic anatomical conditions are choice of interventions which displace the curve only slightly allows a physiological sensation of sound to be maintained. Surgeon often neglect this little studied aspect preferring to evaluate this work in terms of quantity thus rather ignoring the quality of sound achieved.

[Influence of the early mother-infant contact in the delivery room on short or long term breast feeding]. / Influenza del contatto precoce madre-figlio in sala parto sull'allattamento al seno a breve e lungo termine.

Two groups of mothers and their children were followed up in order to assess the influence of early mother-child contact on breast-feeding; a first control was carried out at discharge from the nursery and a follow-up 6 months later. All the mothers were in good health and pregnancy was physiological with uncomplicated eutocic delivery; none of the babies presented pathologies during the period of their stay in the nursery and in the subsequent 6 months. Group A comprised 126 mothers who had had contact with their babies in the delivery room for at least one hour; group B comprised 109 mothers whose first contact with their baby was only 3-6 hours after birth. Significant differences between the 2 groups were observed as early as the first control which took place at discharge from the nursery: 69.1% of the babies in group A against 51.3% in group B had taken mother's milk exclusively (p < 0.01). Equally significant differences emerged from the subsequent control at 6 months: 70 babies out of 116 (60.4%) were breast fed exclusively or partially, against 42 out of 101 (42.4%) in group B (p < 0.01). Furthermore, important data emerged from the duration of breast-feeding, whether this was exclusive or otherwise, in the first 6 months of life: group A differed significantly from group B (p < 0.002). Our figures suggest a positive influence of early mother-child contact on breast-feeding and on its duration. Notwithstanding the fact that there is no unanimous confirmation in the literature, it is considered that such behaviour will bring benefits to both mother and child.

[The sub-occipital approach in functional surgery of acoustic neuroma]. / La via sub-occipitale nella chirurgia funzionale del neurinoma dell'viii nervo cranico.

In this study the Authors analyze 380 acoustic neuroma removals carried out from 1972 to 1992 focusing their attention on 90 attempts to save hearing by employing a suboccipital approach. In this series the facial nerve was preserved in 99% of the cases with completely normal function in 78%. The cochlear nerve was anatomically preserved in 96% of the subjects. According to the Shelton-Brackmann classification applied to evaluate hearing results, good hearing (Class A = PTA < or = 30 dB; SDS > or = 70%) was obtained in 12% of the cases, serviceable hearing (Class B = PTA < or = 50 dB; SDS > or = 50%) in 13%, measurable hearing (Class C = any measurable hearing) in 19% and anacusis (Class D) in 56% of the patients. CSF leak occurred in 6.6% of the cases, meningitis in 2.2%, paresis or paralysis of the ninth and tenth cranial nerves in 3% and ataxia in 2%. In acoustic neuroma surgery, hearing preservation is a new but complicated topic. In fact, some operative steps--such as the separation of tumor from nerves and arteries, tumor mass reduction, exposure of the end of the IAC--certainly influence surgical results, but are a matter of uncontrollable variance even within series from the same surgeon and render hearing preservation an innovative idea still awaiting, however, a controllable procedure. The ethical feasibility of hearing preservation is confirmed by our results in which hearing preservation attempts using a suboccipital approach have the same morbidity that the translabyrinthine route would have in the same patient.