Wolman Disease: A Mimic of Infant Leukemia.

BACKGROUND: Infant leukemia most commonly present with pallor and hepatosplenomegaly. The possibility of other differential diagnosis also has to be kept in mind during evaluation, as identifying the precise etiology for this clinical presentation is crucial for management. OBSERVATION: An infant, was referred to us with suspected infant leukemia and was subsequently diagnosed to have lysosomal acid lipase deficiency/Wolman disease with a novel 5 bp deletion "c.1180_1184del" in the last exon (exon 10) of the lipase A (LIPA) gene. CONCLUSIONS: Hepatosplenomegaly and pallor resulting from nutritional deficiency or bone marrow involvement in Wolman disease can mimic infant leukemia.

Pediatric Primary Ovarian Angiosarcoma: From Rarity to a Realization.

BACKGROUND: Angiosarcoma is an uncommon high-grade sarcoma in children. Visceral angiosarcoma involving the ovary is extremely rare. Because of the lack of recurrent cytogenetic alterations, histopathological identification of this uncommon tumor in unusual sites like the ovary demands pathologic expertise. Complete surgical resection and radiotherapy are the chief treatment modalities determining survival, with chemotherapy contributing a minor role. CASE: We discuss a 11-year-old prepubertal girl who presented with primary angiosarcoma of the ovary. SUMMARY AND CONCLUSION: Early realization of such exceptional presentations of these tumors is needed to achieve the best treatment outcome.

Nipple Areola Complex Involvement in Invasive Carcinoma Breast.

A variety of factors including tumor biology and distance of the tumor from the nipple have been associated with nipple areola complex involvement in patients with breast cancer. Preoperative understanding regarding these factors can help in modifying the surgical options including preservation of nipple areola complex (NAC) and breast conservation. Nipple sparing surgery (breast conservation surgery/skin sparing mastectomy with immediate reconstruction) for breast cancer has gained widespread interest with the aim of achieving oncologically safe and cosmetically acceptable outcome. To study the proportion of cases with nipple areola complex involvement in invasive carcinoma breast and to describe the variables predictive of nipple areola complex involvement in patients undergoing excision of tumor along with NAC for invasive carcinoma breast. A cross-sectional study was conducted among 136 patients who underwent mastectomy in Regional Cancer Center, Thiruvananthapuram. Tumor nipple distance was assessed preoperatively using mammogram and postoperatively by histopathological examination. Nipple involvement was seen only among 4.4%. Preoperative assessment of tumor to nipple distance, tumor size, location, and stage of the disease agrees with the histopathological examination. Only a small proportion of breast cancer has NAC involvement and preoperative assessment could help the surgeon in deciding on the feasibility of NAC preservation.

Wound and surface temperatures in vivo in torsional and longitudinal modalities of ultrasound in coaxial microincisional cataract surgery.

BACKGROUND: To study phacoemulsification probe shaft/wound and corneal surface/tip temperatures in vivo during longitudinal, torsional and combined phacoemulsification modes and their relationship to machine parameters. DESIGN: This was a prospective study at Chaithanya Eye Hospital and Research Institute, Trivandrum, India (tertiary). PARTICIPANTS: Twenty-two eyes of 22 patients were randomized into six groups depending on the grade of nuclear sclerosis (NS) and the type of ultrasound used: Group 1, torsional in NS2; Group 2, torsional in NS3; Group 3, torsional with intermittent longitudinal in NS2; Group 4, torsional with intermittent longitudinal in NS3; Group 5, longitudinal in NS2; Group 6, longitudinal in NS3. METHODS: Patients underwent phacoemulsification by torsional, longitudinal or combined modalities. A thermal camera was used to measure phaco probe temperatures. MAIN OUTCOME MEASURES: The mean probe shaft and tip temperatures were documented for different ultrasound modalities. RESULTS: The mean shaft and tip temperatures were: Group 1, 29.22°C±0.71°C and 28.4°C±0.88°C; Group 2, 32.12°C±0.62°C and 31.88°C±0.84°C; Group 3, 30.25°C±0.71°C and 29.35°C±0.62°C; Group 4, 31.95°C±0.65°C and 32.01°C±1.31°C; Group 5, 23°C and 27.6°C and Group 6, 23°C and 29.68°C, respectively. In all groups using longitudinal ultrasound, the phaco tip surface temperatures were higher than the shaft temperatures, except in Group 3. Shaft temperatures were higher than tip temperatures in cases using torsional phaco, except in Group 4. The mean temperature difference between groups was significant only for shaft temperatures (P=0.001). On thermal imaging, for torsional phaco, the rise in temperature of the probe from shaft to tip was dependent on the amplitude of ultrasound applied (P=0.009). CONCLUSION: The shaft temperatures were higher than over the phaco tip during torsional phacoemulsification.

Recurrent postcardiac injury syndrome mimicking cardiac perforation following transvenous pacing: An unusual presentation.

We report a case of recurrent postcardiac injury syndrome (PCIS) after pacemaker lead insertion. Each episode was attended by hemorrhagic pleuro-pericardial effusion with drop in hemoglobin levels leading us to consider cardiac perforation and subject the patient to surgical pericardiotomy. However, no perforation or active bleeding was detected on exploration. This unusual case illustrates the occurrence of PCIS following pacemaker lead insertion, mimicking cardiac perforation. This entity should be considered in patients who, after pacemaker lead insertion, develop pericardial and pleural effusion associated with markers of inflammation.