Endoscopic choroid plexus coagulation in infants with hydranencephaly or hydrocephalus with a minimal cortical mantle.

BACKGROUND/AIMS: This study evaluates endoscopic choroid plexus coagulation, in conjunction with third ventriculostomy when technically feasible, as a strategy to treat patients with hydranencephaly or hydrocephalus with a minimal cortical mantle. METHODS: We retrospectively reviewed patients with hydranencephaly (n = 4) or hydrocephalus with the cortical mantle <1 cm in maximal thickness (n = 4) who underwent endoscopic choroid plexus coagulation from 2007 to 2010. Endoscopic third ventriculostomy was performed simultaneously when technically feasible (in 3 of 8 patients). Endoscopic management was considered successful if a shunt was not subsequently required. RESULTS: Endoscopic management was successful in 4 of 8 patients (50%) who did not require a shunt over a median follow-up period of 15 months (range 2.5-24). Four patients (50%) failed endoscopic management and required a shunt 1 month to 11 weeks after endoscopic surgery. One patient died 4 days after shunt placement due to pneumonia and renal failure. Three of 4 patients who had a successful endoscopic procedure ultimately died of causes that were likely unrelated to hydrocephalus (pneumonia in 2 patients and sudden infant death syndrome in 1 patient). Besides failure to control hydrocephalus adequately in 4 patients, there were no additional complications noted after endoscopic surgeries. CONCLUSION: Endoscopic choroid plexus coagulation can enable some infants with hydranencephaly or massive hydrocephalus to avoid a ventriculoperitoneal shunt.

Lymphocytic hypophysitis in a patient presenting with sequential episodes of optic neuritis.

A 41-year-old man presented with left optic neuritis (ON) without evidence of other autoimmune disease or hormonal imbalance. MRI showed enlargement of the left optic nerve but no sellar lesion. The patient recovered after steroid therapy but later developed right ON and required treatment again. Follow-up MRI revealed an ill-defined, enlarging sellar lesion with enhancement extending into the right cavernous sinus, and the patient developed symptoms of fatigue and loss of libido. Hormonal studies revealed hypogonadism and hypocortisolism. All laboratory investigation for autoimmune and infectious diseases remained negative. A transsphenoidal biopsy of the lesion revealed lymphocytic hypophysitis. The concomitant development of lymphocytic hypophysitis and optic neuritis suggests a common and likely autoimmune etiology. Visual loss in patients with LYH can sometimes be due to ON rather than compression of the optic apparatus, with significant implications for treatment strategies.

Malignant transformation of an optic pathway glioma without prior radiation therapy.

Optic pathway gliomas (OPGs) arise from the optic nerves, optic chiasm, and/or hypothalamus and most commonly occur in childhood. Although these tumors can be quite challenging to manage, they are typically low-grade astrocytomas histologically, most commonly pilocytic astrocytomas. The few previously reported cases of malignant degeneration of an OPG occurred after external beam radiation therapy. The authors report the first case in the English literature of an OPG that transformed from a low-grade astrocytoma, with features most consistent with a pilocytic astrocytoma, to a malignant glioma without any exposure to radiation therapy.

Outcomes and management strategies after nondiagnostic stereotactic biopsies of brain lesions.

BACKGROUND/AIMS: A significant minority of stereotactic biopsies (SBs) of brain lesions is nondiagnostic, yet there are no optimal strategies for preventing nondiagnostic SB (NDSB) and for managing patients after NDSB. We performed this study in order to identify risk factors for NDSB, to determine how diagnoses are eventually reached in these patients, and to ascertain whether NDSB affects clinical outcomes. METHODS: Retrospective chart review of patients at our institution who underwent SB of brain lesions. RESULTS: Twenty-four out of 100 SBs were nondiagnostic. NDSB was less likely in contrast-enhancing brain lesions in immunocompetent patients, with larger lesions and in the setting of diagnostic findings on intraoperative frozen section analysis. Of 16 patients with adequate postoperative follow-up, a diagnosis was eventually reached in 11, via further review of the pathology, retrieval of additional tissue specimens or additional noninvasive testing. Survival times for patients with NDSB and eventual tumor diagnoses were within expected ranges for patients with similar tumors. Three of the 5 patients who never received a final diagnosis enjoyed prolonged survival without progressive symptoms. CONCLUSIONS: Surgeons should consider taking additional specimens in the case of nondiagnostic intraoperative frozen section during SB. If a tumor is suspected and final pathology is nondiagnostic, outside review of the slides may be helpful, and sampling further tissue should be considered. For diseases other than tumors, the diagnosis will generally be made without a repeat biopsy. The delays in diagnosis resulting from NDSB do not appear to affect survival, at least in patients eventually found to have brain tumors.

Cardiopulmonary resuscitation in patients with a Nuss bar--a case report and review of the literature.

Pectus excavatum (PE) is a common chest wall deformity that may produce a variety of physiological and psychological effects in children and adolescents. In addition, some of these patients have associated cardiac diseases (ie, mitral valve prolapse and Marfan syndrome). Recently, a minimally invasive surgical repair of PE that requires sternal bar placement has become increasingly frequent to enhance patients' cardiopulmonary functioning as well as their self-esteem. However, despite this innovative technique, it is possible for such patients to have a cardiac arrest while their sternal bar is in place. Whether the presence of a metal bar on the underside of their sternum may hinder resuscitative chest compressions (cardiopulmonary resuscitation) is an issue that concerns us, our patients, and their families; the answer requires further investigation. We present a 21-year-old man with PE who underwent a minimally invasive pectus repair but had a fatal cardiac event before bar removal. Paramedics conducting cardiopulmonary resuscitation on the patient later reported that they were unable to deliver effective cardiac compressions and that the sternal bar may have contributed to this.