Management of choanal atresia in cases of craniofacial malformation.

OBJECTIVE: To report the method and results of endonasal endoscopic approach in congenital choanal atresia in cases of craniofacial malformation. PATIENTS: The pathology of unilateral and bilateral choanal atresia and the treatment results in seven children treated between 1999 and 2006 are presented. 5 infants suffering from bilateral atresia also had severe malformations (Charge syndrome [2 patients], trisomy 18, microcephalus, central cranioschisis, cleft lip and anophthalmia 9). The surgical intervention was carried out immediately after birth. 2 patients with unilateral atresia were treated in their second and sixth year of life respectively. CT scan was the diagnostic procedure of choice. Intraoperative endoscopy showed both membraneous and osseous atresia. SURGICAL PROCEDURE: Trocars of different sizes were used to open the atresia plate, while the osseous parts were removed with diamond drills. Silicone tubes were inserted transnasally and remained in place for several months to improve the infants'ability to breath and drink. RESULTS: In all cases of bilateral atresia the tubes either had to be changed repeatedly or replaced with larger tubes due to dislocation and head growth. The septum was perforated in one case. No further stenoses were detected following the removal of the tubes (after 3-6 months). CONCLUSION: The transnasal access is particularly suited to newborns and infants because it induces a minor surgical trauma and carries a low risk of bleeding if endoscopes are used. The results show that the risk of restenoses can be minimised with sufficient fixating and in-patient care.

[Salivary duct carcinoma of the sublingual gland--a case report]. / Das Speichelgangkarzinom der Glandula Sublingualis.

RATIONALE: Pleomorphic adenoma is the most common neoplasm of major and minor salivary gland origin, followed by infectious reasons and sialolithiasis. Less common are adenocarcinomas presented. The salivary duct carcinoma represents a rare variant of the group of adenocarcinomas originated from the salivary glands, especially extremely rare from minor salivary gland origin. CASE REPORT: We report about a 52 year old male patient presenting with painless, non-ulcerating tumor at the floor of the mouth. Since 2 weeks ingestion was painful. Further ENT-investigations including endoscopy and scans (MRT, ultrasound) showed no pathology. In particular, growth of cervical lymph nodes and distant metastases were not revealed. A biopsy showed a rare salivary duct carcinoma of comedo-type originated from the left sublingual salivary gland. The patient underwent an operation: the tumor was extirpated in toto, and, as the pathohistological investigation confirmed, in sano. Additionally a neck dissection at the left side was performed, followed by the radiation of the tumor region and the lymphatic neck regions, after sufficient reconvalescence. The tumor-classification was pT3, pN0. Since 3 years no recurrence of the tumor was confirmed. DISCUSSION: Salivary duct carcinoma is a rare, high-grade malignant epithelial neoplasm, which occurs almost exclusively in the parotid. As presented, it can also occur in minor salivary glands. Histologically it is composed of structures that resemble expanded salivary glands. Comedonecrosis of these structures is a frequent feature. There is some histologic similarity to intraductal carcinoma of the breast. Differential diagnosis comprises adenoid cystic carcinoma, metastases of other adenocarcinomas, such as the carcinoma of the thyroid gland and the comedocarcinoma of the breast. Because of its poor prognosis a rapid and radical therapy is recommended. CONCLUSION: A rare case of a salivary duct carcinoma of sublingual salivary gland origin is presented. Usually the prognosis is poor. The adequate therapy is the complete surgical extirpation including the surgery of the regional lymphatic nodes and the additional radiotherapy.

[Granular cell tumor -- a rare tumor lesion of submandibular gland origin]. / Granularzelltumor, ein seltener Tumor der Glandula submandibularis.

RATIONALE: Granular cell tumors are rare entities with manifestations in nearly all organ systems. Appearance in salivary glands is observed in singular cases. CASE REPORT: We report a 54 years old female patient with a painless submandibular tumor lesion. Using a submandibular approach the tumor was totally removed with the submandibular gland. Histologically a benign granular cell tumor was diagnosed. The immunohistological examination revealed positive for S-100 antigen and vimentin, negative for KL1 antigen. The clinical follow-up for about 3 years was without complications or tumor recidive. CONCLUSIONS: A differential diagnosis of a submandibular mass is the rare manifestation of a benign granular cell tumor. The neurogen origin can be proven immunohistologically by positive S-100 antigen detection. The complete surgical resection is the therapy of choice.

[Malignant transformation of a juvenile papilloma in a 11 year old boy]. / Maligne Entartung einer juvenilen Kehlkopfpapillomatose bei einem 11-jährigen Jungen.

BACKGROUND: The juvenile laryngeal papilloma is the most common benign neoplasm in children. Often the entity shows an elongated recurrent course of disease with an expansion into the tracheo-bronchial system. Sporadic malignant transformation in adults with a papilloma was reported after treatment with radiotherapy alone or in combination with the intake of additional toxins (e. g. nicotine). Similar reports of a malignant transformation of juvenile papillomas without additional risk factors is very rarely reported. CASE REPORT: We report about an 11 year old boy, who suffered from a juvenile laryngeal papilloma. The multiple laser surgical procedures and a therapy with interferon resulted in a short-term remissions. A tracheotomy was inevitable. Seven months after the first diagnosis of the papilloma a regional metastatic squamous cell carcinoma was found. In spite of combined radiotherapy and chemotherapy the boy died 11 months later. CONCLUSIONS: The spontaneous malignant transformation of a juvenile papilloma in a squamous cell carcinoma is extremely rare. The surgical intervention as well the radiotherapy and chemotherapy using interferon was unsuccessful due to the high grade of malignancy. In view of the very short time interval between first diagnosis of juvenile papilloma and the subsequent malignant transformation, one must consider either the potential presence of a very aggressive form of papilloma or alternative two coincident independent diseases.

[The interesting case -- case no. 70]. / Der interessante Fall Nr. 70.

We will report two cases of newborn girls, who could not be breast fed because of a benign tumour in their oral cavity. These polypoid lesions were located in both cases in the labial aspect of the dental ridge. Tumours were fleshy, firm, dark-brown with a broad-based attachment to the alveolar ridge. The underlying bone was not affected. The size of neoplasm was in one case 3 x 2 cm, in the other one 2 x 1.5 cm. Excision was performed in both cases at the day after birth. The postoperative course was uncomplicated. A regular oral feeding was immediately possible. Histological findings showed large tumour cells with abundant granular eosinophilic cytoplasm and a prominent vascularity. Cells did not show immunostaining for laminin or S-100 protein. All these findings are characteristic of congenital granular cell tumour and its synonym congenital epulis. There is a strong predilection for newborn females. It differs from adult granular cell tumours by its prominent vascularity, the presence of scattered remnants of odontogenic epithelium, and the strong phosphatase activity. These lesions are always cured by local excision. The exact nature of this condition is still not clear, and there is little support for its originating from odontogenic epithelial cells.

[Choristoma in the vocal fold]. / Choristom des Taschenbandes.

Choristoma in the larynx is rare and related to thyroid or glial tissue. The manifestation of salivary gland tissue in the larynx has not been reported to date. We present the case of an 80 year old male complaining of hoarseness and productive coughing. A left side tumourous swelling was seen in the larynx with intact vocal cord mobility. Using microlaryngoscopy the mass was resected without any intra- or postoperative problems. Histologically, a choristoma-heterotopic salivary gland tissue in the muscle and fat tissue--was found. The mucosa was intact and there were no signs of malignancy. Differential diagnosis of such masses in the larynx include benign lesions as well as specific infections, e.g. tuberculosis, sarcoidosis, amyloidosis and Wegener's granulomatosis. Especially in non-smokers, sarcoma, lymphoma and melanoma should be separated from the frequent squamous cell carcinoma of the laryngeal tissue. In rare cases, heterotopic tissue can mimic a tumourous mass. Intralaryngeal resection is the therapy of choice and should be recommended to the patient.

[Cochlear implants in adults: indications and realization. Special cases and technical parameters of the implantation systems]. / Kochleaimplantat bei Erwachsenen: Indikation und Durchführung. Teil II: Spezielle Aspekte und Technik der Implantatsysteme.

From the technical point of view, cochlear implant (CI) surgery in adults is a well established procedure. Pre- and postoperative evaluation, surgical approach and rehabilitation programs are standardized, presenting CI as a low risk procedure with rare complications similar to those of middle ear surgery. Part 1 described the diagnostics, surgical procedure and possible complications. The second part will cover the contraindications, special cases as well as the technical parameters of the modern implant system.

[Cochlear implants in adults: indications and realizations. Part I: Diagnosis, operation techniques and results]. / Kocheaimplantat bei Erwachsenen: Indikation und Durchführung. Teil I: Diagnostik, Operationsverfahren und Ergebnisse.

From the technical point of view, cochlear implant (CI) surgery in adults is a well established procedure. Pre- and postoperative evaluation, surgical approach and rehabilitation programs are standardized, presenting CI as a low risk procedure with rare complications similar to those of middle ear surgery. Modern advanced CI devices offer technical reliability and multiple speech encoding strategies and recently, even small processors which are worn behind the ear. Further technical advances are continuously available, even for implanted patients. Upgrading is usually easy because the outer parts of the implant are exchangeable and software upgrades can be transmitted transcutaneously to the implant. Assuming that medical and rehabilitation requirements are available, there are only very few indications against CI in adults. Candidate patients should therefore be transferred to CI-centres where a decision can be taken depending on presurgical evaluation results.

Kochleaimplantat bei Erwachsenen: Indikation und Durchführung : Teil I: Diagnostik, Operationsverfahren und Ergebnisse.

From the technical point of view, cochlear implant (CI) surgery in adults is a well established procedure. Pre- and postoperative evaluation, surgical approach and rehabilitation programs are standardized, presenting CI as a low risk procedure with rare complications similar to those of middle ear surgery. Modern advanced CI devices offer technical reliability and multiple speech encoding strategies and recently, even small processors which are worn behind the ear. Further technical advances are continuously available, even for implanted patients. Upgrading is usually easy because the outer parts of the implant are exchangeable and software upgrades can be transmitted transcutaneously to the implant. Assuming that medical and rehabilitation requirements are available, there are only very few indications against CI in adults. Candidate patients should therefore be transferred to CI-centres where a decision can be taken depending on presurgical evaluation results.

Kochleaimplantat bei Erwachsenen: Indikation und Durchführung : Teil II: Spezielle Aspekte und Technik der Implantatsysteme.

From the technical point of view, cochlear implant (CI) surgery in adults is a well established procedure. Pre- and postoperative evaluation, surgical approach and rehabilitation programs are standardized, presenting CI as a low risk procedure with rare complications similar to those of middle ear surgery.Part 1 described the diagnostics, surgical procedures and possible complications. The second part will cover the contraindications, special cases as well as the technical parameters of the modern implantation system.

Mastoiditis and acute otitis media in children with cochlear implants: recommendations for medical management.

Acute otitis media (OM) or mastoiditis is a very dangerous condition for the ear after cochlear implantation. However, acute OM is very common in childhood and can occasionally occur in an implanted ear. Most cases of acute OM can be successfully treated with intravenous high-dosage antibiotics. In cases of mastoiditis and clinical signs of mastoid abscess, retroauricular drainage is necessary to prevent infection of the implant bed. In a series of 366 children given implants (1 to 14 years), acute OM occurred in 5.6% during a follow-up period of 1 to 8 years. Seven ears had to be opened by means of myringotomy. Five ears were opened by retroauricular incision with mastoid revision on the implanted side. Adenoidectomy and use of ventilation tubes before cochlear implantation, as well as careful subtotal mastoidectomy during the implantation, can reduce the incidence of acute OM in children after implantation. Early and subsequent treatment with operative mastoid drainage can prevent implant loss and should be performed at the implantation center.

[Complications of cochlear implant surgery in children and adults]. / Komplikationen der Cochlear Implant-Chirurgie bei Kindern und Erwachsenen.

BACKGROUND: In a retrospective analysis we evaluated the complication rate in 697 patients after cochlear implantation between 1985 and 1995 (366 children, 331 adults; 604 Nucleus, 50 Clarion and 42 other implants). RESULTS: Intraoperatively in 74 cases (10.6%) total or partial cochlear obliteration was found, a CSF gusher occurred in 7 cases. Minor complications such as seroma (8 adults), wound infections (14 patients), emphysema, and swelling were successfully treated with conservative methods and drugs. Seven (2.1%) adults showed facial nerve palsy postoperatively with incomplete recovery only in 2 of them (0.6%). No permanent facial nerve palsy was observed in children. Cholesteatoma developed in 18 adults (5.4%), which was treated by revision surgery. CONCLUSIONS: In conclusion, cochlear implant surgery is a safe procedure with a low complication rate. However, Cochlear implantation should be performed by well experienced ear surgeons who can properly handle intraoperative and postoperative problems. The patients should be informed about the typical risks of ear surgery including implant removal due to infection or technical defects, facial nerve palsy, vertigo and, especially in adults, increased tinnitus.

[Ultrasound evaluation of characteristics of cervical lymph nodes with special reference to color Doppler ultrasound. A contribution to differentiating reactive from metastatic lymph node involvement in the neck]. / Sonographische Dignitätsbeurteilung von Halslymphknoten unter besonderer Berücksichtigung der Farbdopplersonographie. Ein Beitrag zur Abgrenzung reaktiver von metastatisch befallenen Lymphknoten des Halses.

BACKGROUND: Cervical mass due to lymphadenopathy is a common cause for consultation of an ENT specialist by patients. In many cases exact differentiation without biopsy between reactive and metastatic lymphnodes is difficult but crucial and necessary for each patient. Ultrasound is the imaging system with the highest sensitivity for the evaluation of pathological lymph nodes. However, differentiating benign and malignant lesions remains a problem. PATIENTS: In a prospective study, 138 cervical lymph nodes of 62 patients were evaluated according to conventional ultrasound criteria such as size, shape, brightness, demarcation, etc., and according to parameters of color doppler sonography such as intensity and localization of perfusion. The so called Pourcelot or resistance index, an objective parameter, was measured in order to examine a possible improvement of specificity in differential diagnosis of both entities. All lymph nodes were surgically removed and histologically examined after ultrasonography. RESULTS: 133 lymph nodes were evaluated in the study. Lymphadenitis was demonstrated in 72 cases, whereas 61 of the lumps showed metastases of squamous cell carcinoma of the head and neck region. Three patients with primary malignant lymphoma were excluded from the study. The conventional ultrasound parameters such as size, homogenity, shape and brightness did not reveal any substantial difference between the two groups. However, lymph node metastases significantly showed higher Doppler signals than the reactive ones. Most of the metastases were perfused in the periphery or had a diffuse spread of blood flow. The most valuable parameter from the prognostical point of view proved to be the Pourcelot Index with a threshold value less than 0.6 for metastases, which increased the specificity to 92% with a probability of p = 0.001. CONCLUSIONS: The results of this study demonstrate an increase of the ultrasound specificity in differentiation of pathological cervical lymph nodes using color flow imaging. Unfortunately, this method does not enable the physician to correctly diagnose the findings in all patients. Therefore histological evaluation is mandatory in all doubtful cases.

[Sonographic diagnosis of metastatic renal cell carcinoma to the head and neck region]. / Sonographische Diagnostik von Hypernephrommetastasen im Kopf-Hals-Bereich.

In the area of the head and neck metastases from distant primary tumours are rare in comparison to the common squamous cell carcinomas of the upper respiratory and digestive tract. Thus correct preoperative diagnosis may be difficult. Two cases with distant metastases of renal cell carcinoma to the head and neck region are presented in this report. In a 60-year-old male patient, diagnostic evaluation of unilateral epistaxis revealed a radioopacity of the maxillary sinus, six months after removal of a renal cell carcinoma. In the second case a slowly progressive indolent swelling of the left neck developed in a 56-year-old man six years after resection of a renal cell carcinoma and two years after surgical treatment of a pancreatic carcinoma. Following clinical examination, modern imaging techniques with special emphasis on colour Doppler sonography with a Siemens Quantum 2000 were used for diagnostic evaluation in both patients. Sonography of metastatic renal cell carcinoma to the maxillary sinus revealed complete opacity of the antrum. The cervical mass proved to the inhomogeneous and hypoechogenic and was difficult to distinguish from the lower parotid lobe and the vessel sheath. A common feature of both tumours was a high degree of perfusion which could be confirmed by superselective angiography. The histological examination of the surgical specimen showed an isolated metastasis of a renal cell carcinoma in both cases. In patients with a history of renal cell carcinoma the possibility of distant spread to the head and neck region should be taken into account even after a long period of complete remission. Colour Doppler sonography facilitates the distinction between the normally well perfused secondary tumours and squamous cell carcinomas which usually only have a minimal blood supply. Because of the high risk of profuse bleeding a biopsy should only be performed in the operating theatre.

[Temporal bone fracture after head trauma causing rhinoliquorrhea and meningitis]. / Felsenbeinlängsfraktur im Bereich des Karotiskanals als Ursache für rezidivierende Rhinoliquorrhö und Meningitis.

We report our experience in managing a temporal bone fracture after head trauma that had no apparent clinical signs. Recurrent CSF rhinorrhea and meningitis lead to extensive diagnostic procedures. Operative exploration of the temporal bone demonstrated a fracture line along the horizontal part of the carotid artery. The location of the fracture did not cause such typical symptoms as hearing impairment, facial paralysis, vertigo or tinnitus. Only CSF liquorrhea through the Eustachian tube indicated a fracture at the lateral skull base.

Complications in pediatric cochlear implant surgery.

In a retrospective analysis we evaluated the intra and postoperative complications in children who underwent cochlear implantation between 1984 and 1993 at the Medizinische Hochschule Hannover. The data and records of 366 children were collected and analyzed. Relevant parameters were major complications such as significant infection, intraoperative bleeding, facial nerve injury, implant loss and device failure, as well as lesser complications, including delayed wound healing, chronic pain and vertigo. Late complications such as cholesteatoma or electrode dislocations were also registered. Cases of acute otitis media were managed with conservative treatment. Data presented indicate that cochlear implant surgery in children is a reliable and safe procedure with a low percentage of severe complications. Problems related to ear surgery can occur and should be manageable with standard procedures. Careful operative techniques and sufficient personal experience can help avoid severe post-operative problems.

Adenoid cystic carcinoma of the skull base.

Adenoid cystic carcinoma (ACC) is a slowly growing tumor with a particular tendency to infiltrate the surrounding tissue by perineural spread. The clinical diagnosis may prove difficult due to the submucons extension of the tumor, especially at the skull base. This article outlines the clinical characteristics, diagnostics, and treatment modalities in a series of 56 patients with an ACC in the head and neck diagnosed between 1970 and 1998 in 32 females and 24 males. The youngest patient was aged 24 years, the oldest 77 years. The average age was 54 years. In 16 patients the tumor originated in the paranasal sinuses or the nasopharynx and involved the skull base. As a rule, several months passed between the manifestation of the first symptoms such as pain, blocked nose, epistaxis, or diplopia and the initial clinical diagnosis. All patients received surgical treatment, however, complete microscopical resection could only be achieved in approximately one third of the cases. Therefore, nine patients were postoperatively treated with radiotherapy. The average survival rates of the patients with an ACC of the skull base were only 99 months as compared to 144 months in the patients without skull base involvement.

[Otogenic brain abscess]. / Der otogene Hirnabszess.

BACKGROUND: Otogenic complications are rare but typical following acute or chronic ear infections like mastoiditis and cholesteatoma. A life-threatening sequela is the otogenic brain abscess located in the temporal lobe or cerebellum. PATIENTS: At the ENT Department of the Medical University of Hannover/Germany we treated 8 patients suffering from otogenic brain abscesses in the temporal lobe during the last three years. The average age of the 6 male and 2 female patients was 48 years. In 5 patients the abscess developed due to a cholesteatoma with superinfection. Three cases showed acute mastoiditis. All patients were operated using an otosurgical retroauricular approach, in five cases a classical radical mastoidectomy was performed. In two cases the abscess was reached via mastoidal approach and was subsequently drained. In two other cases the abscess was drained some days later by neurosurgical approach due to increased neurological symptoms. The other patients were treated with high-dosed antibiotics under regular clinical and radiological control. RESULTS: In 7 cases complete regression of the abscess was achieved. Five patients were discharged without further otological or central-nervous problems. One female patient developed severe meningitis with generalized thrombosis of the central blood sinus system and died in central circulatory failure. Two other patients developed a moderate psychopathologic syndrome and were admitted to rehabilitation institutions. CONCLUSIONS: The analysis of our patients shows that otogenic brain abscesses should be regarded especially as a severe complication of the untreated cholesteatoma. It is important to use modern imaging modalities like computer tomography or MRI for early detection of the intracerebral lesion and to perform an early otosurgical intervention. Under antibiotics and CT control, healing of this severe complication can be achieved in most cases. However, the danger of acute and chronic ear diseases has to be kept in mind in all medical disciplines.