Large Congenital Hydrocephalus:Extended Survival without Neurosurgery.

PROFESSIONAL PRESENTATION: This work was presented at the 57th Quarterly Grand Rounds at Fairview Developmental Center, Costa Mesa, California on October 17, 2012. ACKNOWLEDGMENT: Authors are grateful to Stephan Reynolds CRT, and Indira Makwana for records. CONFLICT OF INTEREST: None of the authors has any conflict of interest with the information provided in this article. ABSTRACT: We report the case of a 45-year-old patient with large congenital hydrocephalus. Patient was born prematurely at 26-weeks' gestation with a weight of 1.14Kg. His head-circumferences have been: Birth, 27.9 cms; age 3-months, 40.6 cms; 18-months, 74 cms; 36-months, 80 cms; current, 88.4 cms (normal adult: 52-58 cms). During infancy, his prognosis was considered "terminal" due to rapid progression of severe hydrocephalus; therefore no neurosurgical intervention was undertaken. At 18-months' age, he was admitted to our developmental center where he is presently comfortable and clinically stable. Due to habitual unilateral decubitus, his head is now markedly flattened on the right (deformational plagiocephaly), and disproportionately long and narrow (scaphocephaly: occipitofrontal length 36.8 cms, biparietal width 12.5; ratio 2.9). We credit patient's extended survival to diligent professional care. INDICES USED: PubMed.

Human bites: bloodborne pathogen risk and postexposure follow-up algorithm.

Human bites may transmit bloodborne pathogens (BPs) by exposing the biter's oral mucosa to the bitee's blood and the bite wound to biter's saliva. Consequently, bites may require postexposure follow-up per the Occupational Safety and Health Administration (OSHA) BP standard. Literature reveals that BP transmission via bites is rare. Review of available records in our developmental center identified no bite-related BP transmission between 1993 and 2011. To avoid unnecessary testing while remaining OSHA-compliant, we propose an algorithm for selective follow-up of bites. Since hepatitis B virus can be transmitted by mucosal exposure to blood and, rarely, also by nonintact skin exposure to blood-free saliva, all biters and bitees require hepatitis B follow-up. Since hepatitis C virus and human immunodeficiency virus (HIV) transmissions require "visible blood" exposure, and since saliva is usually bloodfree, risk of HCV-HIV transmission from biter to bitee is negligible. Therefore biters need HCV-HIV testing only after bloody saliva bites. Since biter's oral mucosa invariably gets exposed to bitee's blood (reverse exposure), all bitees should be tested for HCV-HIV infectivity. Our proposed algorithm may prevent harm and waste from unnecessary biter testing and venipuncture.

Necrotizing fasciitis: diagnostic challenges in a mute bedridden patient with atypical laboratory parameters.

A 27-year-old mute bedridden patient required parenteral corticosteroids and antibiotics, and hospitalization for an acute respiratory illness. After 2 days, staff noted a ~0.3 cm blister on the patient's right heel. Within 19 hours, blistering increased and the foot became partly gangrenous. The patient developed high fever (40.3°C), and leukocytosis (count: 13 × 10(9)/L; was 6.5 × 10(9)/L ten days earlier). Necrotizing fasciitis (NF) was diagnosed and treated with emergency leg amputation. Histopathology revealed necrosis of fascia, muscle, subcutaneous tissue, and skin. In bedridden patients, corticosteroids may particularly facilitate serious infections, and initial NF blistering may be mistaken for pressure ulcers. Vigilant and frequent whole body monitoring is necessary for all patients incapable of verbalizing their symptoms.

Piperacillin-induced immune hemolysis presenting with tachycardia and cardiac arrest.

A 20-year-old nonverbal patient with profound developmental disabilities was treated with intravenous piperacillin-tazobactam for respiratory infection. After 8 days, he became afebrile with normal pulmonary status, but his pulse remained inexplicably rapid (114/minute). Investigations revealed severe normochromic normocytic hemolytic anemia (hemoglobin: 40 g/L, reticulocytes: 9.4%, nucleated erythrocytes: 5%). While being hospitalized, patient experienced sudden cardiac arrest from which he was successfully resuscitated. He had no blood loss or intrinsic heart disease to explain the acute anemia or cardiac arrest. He had uneventfully received piperacillin-tazobactam on 7 occasions during the preceding 5 years for >50 days. Patient was treated with intravenous crystalloids, methylprednisolone and transfusion of 3 units of packed erythrocytes. Piperacillin-tazobactam was discontinued. A direct antiglobulin test was positive for immunoglobulin G and complement. Antibody to piperacillin was detected in patient's serum by the "immune-complex" method confirming "piperacillin-induced immune hemolytic anemia (PIHA)". On discharge (day 15), patient's hemoglobin improved to 115 g/L (baseline: 131 g/L). Vigilant clinical and hematological monitoring for anemia is indicated in piperacillin-treated patients, particularly in those unable to verbalize their discomfort. Repeated piperacillin exposure may sensitize and predispose patients to PIHA.

Serious pH1N1 influenza illness and pneumonia despite inactivated vaccine: success of prompt diagnosis and treatment.

A 42-year-old bedridden patient suddenly became seriously ill with an unexplained fever (39 degrees C) and hypoxemia (pulse oximetry oxygen saturation: patient, 90%; normal, >98%). He had received the inactivated vaccine for pandemic 2009 H1N1-influenza (pH1N1) 41 days earlier. He had no cough, sore throat, or pharyngitis. Therefore, he did not satisfy the Centers for Disease Control criteria for an "influenza-like illness." Nevertheless, his nasopharyngeal swab was tested by rapid enzyme-linked immunosorbent assay for influenza A and found positive. He was promptly treated with supplemental oxygen and oseltamivir (75 mg twice daily) for 5 days. On day 6, reverse transcriptase-polymerase chain reaction test confirmed the virus to be pH1N1. A chest radiograph was normal on day 1 but revealed bilobar pneumonia on day 2. This was considered bacterial superinfection and empirically treated for 10 days with 3 g of piperacillin and 375 mg of tazobactam. The patient fully recovered. This case of pH1N1 vaccine failure occurred because no vaccine is 100% protective, and immune response may be poorer in patients with chronic medical problems. Vaccine failure was not due to immunodeficiency or improper vaccine handling. We credit this patient's recovery to our facility's heightened surveillance for influenza even among the vaccinated individuals, and also in those without classic influenza-like illness.

Intermittent diarrhea as a delayed presentation of percutaneous endoscopic gastrostomy (PEG)-associated fistula.

A 60-year-old patient with severe dysphagia, weight loss, and recurrent aspiration pneumonia required a percutaneous endoscopic gastrostomy (PEG) for long-term feeding. After 24 uneventful days, she developed an enigmatic recurring but intermittent diarrhea. On day 62, staff noted a feculent odor from her gastrostoma, along with undigested formula in her stools. This prompted her hospitalization. A plain abdominal radiograph demonstrated the PEG tube in the upper abdomen, but could not differentiate if its tip was misplaced. Next, an abdominal barium-contrast computed tomography scan was performed but was inadvertently misinterpreted as normal. Finally, a colonoscopy demonstrated that the tip of the PEG tube was malpositioned in the transverse colon, resulting in a colocutaneous fistula (CCF). The PEG tube was withdrawn uneventfully through the gastrostoma. A laparotomy was performed. Strong adhesions were found between the stomach and the colon; these were lysed and the CCF tract was excised. The patient recovered. CCF should be considered in the differential diagnosis of PEG patients with unexplained diarrhea even if the diarrhea is delayed or intermittent; the diagnosis should be confirmed by a tubogram.

Bloodborne pathogens exposure from occupational fingernail scratches.

Following occupational fingernail scratches (OFSs) in a developmental center, the source resident and the injured employee were tested for bloodborne pathogens (BPs). The pros and cons of this practice were scrutinized since fingernails usually contain no blood. Available records revealed no OFS-related BP transmission in 14 years. PubMed displayed no article linking OFS with BP. The facility's practice was discontinued as it was deemed unnecessary, wasteful, an impediment to the event's expeditious closure, an ineffective workers' compensation safeguard, and a potential source of venipuncture-related complications and false-positive laboratory results. Even long-standing practices require periodic scientific review.

The wheelchair thrombosis syndrome.

OBJECTIVE: To report a case of deep vein thrombosis (DVT) related to prolonged wheelchair use. CASE REPORT: A 48-year-old patient with spastic quadriplegia usually spent 10-12 hours daily in a wheelchair. He suddenly developed marked swelling of his right foot, leg and thigh. His plasma D-dimer level was 1,030 (normal <500) ng/ml. A duplex ultrasound revealed common femoral vein thrombosis. He was hospitalized and anticoagulated; his extremity swelling decreased considerably by day 45. Hypercoagulability work-up disclosed previously subclinical mild elevation of serum cardiolipin immunoglobulin G (antiphospholipid syndrome). This patient will receive longterm anticoagulation. CONCLUSION: Prolonged sitting in wheelchair may cause DVT. To enhance public recognition of this avoidable risk, we propose the term "wheelchair thrombosis syndrome."

The futility of universal pre-employment chest radiographs.

In a developmental center, a preemployment chest x-ray was required for all job applicants. We scrutinized the pros and cons of this practice through a review of the medical literature and our experience, and discussion with our colleagues. We concluded that such chest x-ray caused unwarranted radiation exposure, did not produce compliance with the tuberculosis laws, gave a false sense of security regarding workers' compensation risk management, was contrary to established occupational medicine practice guidelines, and was unnecessary and wasteful. We discontinued such chest x-rays. The purpose of the pre-employment examination should remain narrowly job related. Even long-established procedures require periodic utilization review.

Esophageal obstruction by a lemon that required esophagotomy: thoughts on prevention.

A patient with pica and Lennox Gastaut syndrome suddenly refused oral intake. Neck radiographs revealed no foreign body. Barium swallow identified an irregular filling defect in the cervical esophagus. Esophagoscopy showed a gold ball-like object (half a lemon) 3 cm distal to the cricopharyngeus. This object had to be removed by esophagotomy after failed attempts with flexible and rigid esophagoscopy, laryngoscopy, and a Foley catheter. The charge for her 39-day hospitalization was $282,761. She had ingeniously procured and swallowed the lemon despite a full-face plastic shield and staff surveillance. In nonverbal persons, medically unexplainable abrupt food-refusal is an emergency. Prevention necessitates attention to pica, impulsivity, dentition, food consistency, eating utensils, seizures, environment, adaptive equipment, and surveillance.

Identification of low bone mass in a developmental center: finger bone mineral density measurement in 562 residents.

OBJECTIVES: The objective of this study was to measure finger bone mineral density (BMD) in residents of a developmental center. DESIGN: A cross-sectional descriptive study performed during the residents' annual physical examination. SETTING: This study was conducted at a long-term care facility for people with severe developmental disabilities (mental retardation, cerebral palsy, epilepsy, and autism). PARTICIPANTS: Study participants included 562 (67%) of the 833 residents aged 30 years or more on whom we could measure BMD. MEASUREMENTS: We measured BMD by peripheral dual-energy x-ray absorptiometry (DXA) on the residents' middle fingers by Accudexa. The instrument converted the BMD values into T-scores relative to the manufacturer's reference young healthy population. We retrieved the residents' clinical variables from a centralized database. RESULTS: T-scores for the 562 residents were: <-2.5 standard deviation (SD) (osteoporotic): 98 (17%), -2.5 SD>t <-1 SD (osteopenic): 156 (28%), and >-1 SD (normal): 308 (55%). Multivariate regression analysis revealed that BMD was significantly lower in residents (compared with their counterparts) with inability to ambulate, male gender, white race, quadriplegia, profound mental retardation, and older age. CONCLUSION: There is a need for heightened osteoporosis surveillance and preventive effort in this population in which almost half of the residents had previously undiagnosed subnormal BMD, including one sixth who had osteoporosis.

End-of-life care for a man with developmental disabilities.

BACKGROUND: Science can artificially maintain many essential life functions. Does such care prolong life or dying? METHODS: A case is described of a patient with developmental disability with unknown health care choices who was hospitalized for drug-resistant urosepsis. He developed aspiration pneumonia, deep vein thrombosis, and respiratory arrest. He required gastrostomy, tracheostomy, artificial ventilation, parenteral nutrition, hemodialysis, multiple anti-infective agents, and blood transfusions. On day 58, a bioethics committee recommended against cardiopulmonary resuscitation. On day 66, the patient's conservator concurred but required continuation of artificial ventilation. To the dismay of some caretakers, the patient continued to receive intrusive care until his death on day 104. The hospital charge was $709,206. RESULTS AND CONCLUSION: Hospital care of patients with mental incapacity can be clinically and ethically challenging. End-of-life decisions can be facilitated when the patient's legal representative and physician actively advocate the patient's best interests and communicate frequently and openly. Suggestions are made for such exigencies.