Morphology studies of the human fetal cochlea in turner syndrome.

OBJECTIVES: Turner syndrome (TS) is the most frequent sex chromosome abnormality, and sensorineural hearing loss is common. We aimed to determine whether there are consistent morphologic cochlear abnormalities during gestational development that could be associated with TS. DESIGN: The histology of nine fetal temporal bones of TS autopsied after spontaneous abortion was studied. RESULTS: Gross morphologic examination of the TS cochleae failed to reveal a pattern of structural abnormalities that would explain the development of sensorineural hearing loss. Mondini-like cochlear dysplasia was observed in one 13-wk-old TS fetus. CONCLUSION: We could not demonstrate a consistent pattern of cochlear malformations.

Systematic screening and treatment evaluation of hereditary neck paragangliomas.

Familial paragangliomas of the neck are often bilateral and more aggressive than spontaneous forms. Tumors appear earlier (2nd-4th decade) often with diffuse, multifocal involvement. Without treatment, these tumors can lead to significant morbidity. Three families with succinate dehydrogenase subunit D (SDHD) germline mutations underwent clinical and genetic evaluation. Patients were screened using ultrasound and evaluated further with conventional and functional imaging. Tumors with a diameter >1.5 cm were surgically removed. Multicentric and bilateral tumors were detected in 9/13 (69%) and 8/13 (62%) patients, respectively. Surgical morbidity occurred in 64% of patients. Local recurrence was 57%, although this was lower in tumors with a diameter <2 cm. We recommend an algorithm for a systematic approach to the diagnosis, monitoring, and treatment of familial head and neck paragangliomas. Operative treatment in advanced stages often leads to unwanted morbidity, such that earlier detection and treatment of smaller tumors seems to be of benefit.

A critical comparison of robotic versus conventional laparoscopic splenectomies.

The benefit of robotic systems for general surgery is a matter of debate. We compare our initial series of robotic splenectomies with our first series of conventional laparoscopic ones. A retrospective analysis of the first six robotic versus the first six conventional laparoscopic splenectomies is presented. Patients were matched with regard to age, body-mass index, ASA score, and preoperative platelet levels. All procedures were performed by a single surgeon. Size and weight of the resected specimens were comparable in both groups. Median overall operating time was 154 (range, 115-292) min for the robotic and 127 (range, 95-174) min for the laparoscopic group. No complications occurred. There were no open conversions. The median postoperative hospital stay was 7 (robotic group) and 6 (laparoscopic group) days. Median average costs were 6927 dollars for the robotic procedure versus $4084 for the conventional laparoscopic procedure (p < 0.05). Minimally invasive splenectomies are feasible using either conventional laparoscopic techniques or the da Vinci robotic system. In this analysis, procedures performed with the da Vinci robotic system resulted in prolonged overall operative time and significantly higher procedural costs. The use of a robotic system for laparoscopic splenectomy offers, at this stage, no relevant benefit and thus is not justified.