Impact of our new protocol on the outcome of the neonates with congenital diaphragmatic hernia

Background/purpose

Congenital diaphragmatic hernia (CDH) remains a defiant challenge for pediatric surgeons. Since 2003, we developed a new protocol aiming for the better outcome. In this study, the usefulness of our new protocol was evaluated.

Materials and methods

Forty-six neonates with CDH at the age of less than 24 h were divided into two groups based on the difference of era and treatment protocols. In Group 1, 15 patients were treated between 1997 and 2002 and 31 patients were treated between 2003 and 2011 in Group 2. In the latter group, a new protocol was introduced focusing on the prevention of lung edema as well as lung injury by steroid administration and on the stabilization of cardiopulmonary function using continuous d-mannitol infusion. The survival rate and the postoperative intubation period (POIP) were compared between the two groups.

Results

The overall survival rate was significantly increased from 53 % (8/15) to 81 % (25/31) (p < 0.05). In isolated CDH, the survival rate was increased from 58 to 93 %. The average POIP was remarkably shortened from 39.0 to 4.4 days (p < 0.01).

Conclusion

Our new protocol remarkably improved the survival rate and shortened the period of mechanical ventilation in neonates with CDH.     

Vocal cord inflammatory myofibroblastic tumor with mucoid deposits harboring TIMP3–ALK fusion: A potential diagnostic pitfall

A 35‐year‐old Japanese man who had experienced hoarseness for 10 years presented with a vocal cord lesion. A gross examination revealed a left vocal cord polyp occupying two‐thirds of the vocal space. The endoscopically resected lesion contained scattered atypical fibroblastic, stellate, or ganglion‐like cells with mucoid stroma. Vacuolated cells were also seen. Lymphoplasmacytic infiltrate was largely undetectable. A vocal cord polyp was first suspected, but well‐differentiated liposarcoma and inflammatory myofibroblastic tumor (IMT) were included in the differential diagnoses. The tumor cells were positive for anaplastic lymphoma kinase (ALK), calponin, and vimentin, and negative for other smooth muscle markers by immunohistochemistry. Structures resembling myofibroblasts were not observed by electron microscopy, which confirmed abundant rough endoplasmic reticulum in the tumor cells and accumulated lipid droplets in some tumor cells. ALK gene rearrangement was detected by fluorescence in situ hybridization, and TIMP3–ALK fusion was confirmed by 5′ rapid ampli?cation of cDNA ends. We diagnosed the lesion as an IMT, and an ALK‐rearranged stellate cell tumor may be postulated. This is the first report of a fusion partner gene of ALK in a case of laryngeal IMT.     

Primary ovarian angiosarcoma: A case report and literature review

Primary ovarian angiosarcoma is extremely rare. Only 16 cases have histologicaliy been reported to date In the Ilterature. A case of angiosarcoma arising In the right ovary of a 46-year-old female is presented. Grossly, the resected right ovary was completely replaced by a solid tumor mass, which revealed multiple necrotic and/or hemorrhagic foci. This case revealed the typical histological features of angiosarcoma with sinusoldal and solid patterns of anaplastic tumor cells. Immunohlstochemically, tumor cells were strongly and diffusely positive for CD31 and CD34, in particular, along the cytoplasmic membrane of the tumor cells. Ultrastructurally, tumor cells possessed the intermediate junctions between tumor cells, discontinuous basal laminae attached to the irregularly shaped blood vessels and occasional cytoplasmic pinocytotlc vesicles. These findings confirmed the case as being one of angiosarcoma of the ovary. The patient died 9 months after surgery as a result of developed multlfocal brain metastases. A total of 17 cases reported as primary ovarian anglosarcoma, including this presented case, are clinicopathologically reviewed.     

Tetralogy of Fallot with Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta and Hypoplastic Left Pulmonary Artery

A case of tetralogy of Fallot associated with anomalous origin of the right pulmonary artery from the ascending aorta and hypoplastic left pulmonary artery was successfully repaired. Following the simultaneous procedure with banding of the right pulmonary artery and replacement of modified Blalock-Taussig shunt to the left pulmonary artery at 34 days of age, the intracardiac repair was performed successfully at 1 year and 10 months of age. The patient was well 6 months postoperatively, without unilateral pulmonary hypertension.     

Complete response of a stage IV mucinous cystadenocarcinoma of the ovary to systemic chemotherapy employing paclitaxel and carboplatin

BACKGROUND: The survival rate of patients with advanced-stage mucinous cystadenocarcinoma of the ovary is dismal and no best treatment is known. We report a case of complete response of a stage IV mucinous cystadenocarcinoma of the ovary to systemic chemotherapy employing paclitaxel and carboplatin. CASE: A 51-year-old nullipara diagnosed with International Federation of Gynecology and Obstetrics stage IV mucinous cystadenocarcinoma of the ovary underwent cytoreductive surgery followed by systemic chemotherapy employing paclitaxel and carboplatin every 4 weeks for 3 courses. The patient tolerated chemotherapy well, demonstrated a remarkable response showing no evidence of malignancy at a second-look laparotomy. As a consolidation chemotherapy after negative second-look laparotomy, she underwent another three courses of chemotherapy of the same regimen, and is showing no evidence of disease. CONCLUSION: Paclitaxel and carboplatin may be effective in treating mucinous cystadenocarcinoma of the ovary.     

Amygdaloid kindling in glutamate transporter (GLAST) knockout mice

PURPOSE: Glutamate is the predominant excitatory neurotransmitter in the mammalian central nervous system. We previously reported abnormal glutamate release during seizures after kindling. GLAST and GLT-1 are astrocytic glutamate transporters, highly concentrated in the cerebellum and the telencephalon, respectively. We have investigated whether stages of amygdala kindling in knockout (KO) mice deficient in GLAST are the same as those of wild mice. METHODS: Electrodes were implanted into the basolateral amygdala, bilaterally, in C57BL/6J mice and GLAST KO mice under anesthesia. Once-daily stimulation was applied on one side through these electrodes, and bilateral EEG recordings were obtained. RESULTS: The behavioral manifestations of kindling in mice were (a) arrest of behavior, (b) head nodding, (c) forelimb clonus, (d) bilateral forelimb clonus with rearing, (e) tonic generalized convulsion with elevation of tail and falling with generalized tonic convulsion (GTC). Results of kindling: (a) Mutant mice kindled more slowly than wild-type mice (more stimulations were required to reach each stage in mutants); (b) after kindling was completed, additional stimulations induced shorter afterdischarges (ADs) in mutants than in wild-type mice; (c) Interictal epileptic spikes were more frequent in the mutants than in the wild-type mice, both in the stimulated amygdala and in the contralateral amygdala. CONCLUSIONS: GLAST KO mice, which are missing one type of astrocytic glutamate transporter, demonstrate kindling of an epileptic focus. However, the kindling is significantly slower in the GLAST mutant than in wild-type mice. These findings suggest that glutamate transport by the astrocytic GLAST transporter may have a role in normal epileptogenesis.     

Extrusion and fragmentation of hydrogel exoplant 11 years after scleral buckling surgery

MAI or MIRAgel is a hydrogel developed as a scleral buckling material. We identified upper lid masses and a limitation of supraduction in the left eye of a patient who was associated with extrusion and fragmentation of the MIRAgel episcleral exoplant 11 years after scleral buckling surgery. The material could not be removed completely because of fragmentation. Fragmentation and extrusion of MIRAgel may be rare, but periodic long-term follow-up examinations should be performed after this product has been used.