OBJECTIVES

General Objective: To present a case of a nine month-old Filipino baby from Quezon City who initially presented with rashes and eventually was diagnosed to have hypersensitivity reaction and advised admission for further diagnostic and management.

Specific Objectives: 1. To describe the clinical manifestations, clinical course and management of a case of a staphylococcus scalded skin syndrome. 2. To describe the late and possible complications of staphylococcus scalded skin syndrome, intervention, and early detection of complications. 3. To give information on the update of staphylococcus scalded skin syndrome.

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CASE PRESENTATION

This is a case of a nine month-old Filipino baby from Quezon City who was admitted in our institution on September 13 2013. The patient was born full term to a 33-year old G2P2 (2002) married, nonasthmatic, non-hypertensive, non-diabetic, non-smoker and non-alcoholic beverage drinker mother at a government hospital with APGAR score of 8/9, birth weight of 2.4 kg, unrecalled birth length, 39 weeks by ballard score. 2 week prior to admission patient developed sty on left eye. No other associated symptom such as fever, rashes, cough and cold. Then patient was brought to AMD with unrecalled diagnosis and given erythromycin zalf. This condition was persisted until 1 week prior to admission patient developed rashes on abdomen, and on the next day the rashes was appear on chest, back, and upper and lower extremities. No associated symptom was noted, such as fever, cough and colds. 3 days prior to admission, due to persisted condition, patient was consult with AMD and given cetirizine once daily. 2 days prior to admission, due to persisted condition, patient was brought to government hospital, diagnosed case of hyprsensitivity reactions secondary to food intake. Patient was given diphenhydramine intramuscular. 1 day prior to admission patient developed more rashes on all extremities, trunk and abdomen. No other associated symptom associated such as fever. Few hour prior to admission, patient had purulent discharge on both eye. Patient was brought to AMD then advides to be admitted hence admitted. Upon admission in our institution, patient was awake, irritable, and not in cardiorespiratory distress. Vital signs were normal (Temperature of 37.3C, PR of 140/min, RR of 50/min). Anthropometric measurements showed weight of 8.4 kg, height of 60 cm, head circumference, chest circumference and abdominal circumference within normal range. Pertinent physical examination revealed erithematous macular rashes SSSS Page 2

on face, trunk, abdomen, upper and lower extremities. Bilateral purulent discharge on both ayes. Dry lips, hyperemic pharyngeal wall with exudates. Bilateral palpable cervical lymph nodes. Chest finding was unremarkable. Neurological examination of the patient was essentially normal. COURSE IN THE WARDS: Upon admission Patient was put on hypoallergenic diet for age, and venoclyse with d5 0.3 Nacl mild, patient noted with fair appetite and decrease oral intake. Patient monitored with stable vital signs and consistently remained to have adequate urine output at 1-2 cc/kg/hr throughout her hospital stay. On the first hospital day patient still noted with purulent eye discharge and rashes. Work up was done which included CBC, Eye discharge gram stain culture sensitivity and urinalysis. Intravenous Oxacillin at 119 mg/kg/day and Erythromycin eye ointment were started empirically. Salbutamol neb was put on PRN. On the 2nd hospital day, desquamation on perioral was noted. Patient remained with purulent eye discharge, hence gram stain and culture of the discharge was requested which eventually showed positive for Pseudomonas aeruginosa sensitive to penicillins hence oxacillin was continued while Erythromycin eye ointment was shifted to tobramycin on the 5th hospital day.

Prior to the repair of the myelomeningocele on the 5 th HD, cranial ultrasound was done and was unremarkable. Patient tolerated the procedure well however gram stain and culture from the mass was not submitted to the laboratory because of contamination. Patients condition remained stable and five days post -operatively (11th HD,) leakage from the operative site was noted and another repair was contemplated after blood transfusion was given due to low hemoglobin level as a result of the first operation. Ceftriaxone was shifted to Meropenem at 125 mg/kg/day because of persistent bowel movement of more than six times and the leakage from the operative site. Meropenem was completed for 14 days and a repeat cranial ultrasound was done on the 23rd HD which was normal. No further leakage was noted for three more days and patient was eventually discharged on the 27 th HD, 31st DOL. The persistent bowel movement of more than six times per day of the patient since admission may not be due to an infection but secondary to lack of anal sphincteric tone as part of the myelomeningocele. Patient was also referred to pediatric urologist for possible urologic problems like urinary dribbling or high-pressure bladder or sphincter dyssynergy in patient whose do not leak urine which also normally are present among cases of myelomeningocele. KUB ultrasound was contemplated after one to two months from discharge. SSSS Page 3

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DISCUSSION We are presented with 9 months old Filipino with following salient feature: a.) From the maternal history: Normal pregnancy ultrasound (latest done at 8th month AOG) Absence of folic acid intake of the mother b.) From the physical examination of patient Mass at the sacral area measuring 5 x 5 cm in diameter, not covered by intact skin. Positive Transillumination test of the sacral mass c.) Pertinent ultrasonologic findings: Ultrasound of the sacral mass revealed hypoechoic mass, measuring 38.5 x 17.5 mm in diameter. Cranial ultrasound was unremarkable Various types of masses may affect the presacral area in children. A presacral mass may be congenital or developmental or may arise from inflammation. The mass may have neural, vascular, lymphatic, or mesenchymal origins and may be primary (as in focal disease) or systemic (as in multifocal disease).1,10 In our patient, differential diagnoses of either myelomeningocele or meningocele are considered. In meningocele cases, the mass is covered by intact skin, and in most cases, infant has no neurological problem. In our patient, she was noted with bowel movement 6-10 times every day due to neurological problem which is lax anal spinchter and the mass was not covered by intact skin hence meningocele was ruled out. The presence of sacral mass not covered by intact skin, accompanying lax anal sphincter and impression of neural tube defect on ultrasound of the sacral mass point to a diagnosis of Myelomeningocele. Myelomeningocele produces dysfunction of many organs and structures, including the skeleton, skin, and gastrointestinal and genitourinary tracts, in addition to the peripheral nervous systlem and the CNS.9 The extent and degree of the symptoms and neurologic deficit depend on the location of the myelomeningocele and the associated lesions, such as: 10,12 flaccid paralysis of the lower extremities absence of deep tendon reflexes lack of response to touch and pain lower extremity deformities (clubfeet, ankle and/or knee contractures, and subluxation of the hips) constant urinary dribbling and a relaxed anal sphincter. high-pressure bladder and sphincter dyssynergy in patient whose do not leak urine. A lesion in the low sacral region causes bowel and bladder incontinence associated with anesthesia in the perineal area but with no impairment of motor function. Thus, a myelomeningocele above the midlumbar region tends to produce lower motor neuron signs.10 The cause of myelomeningocele is unknown, but as with all neural tube closure defects, a genetic predisposition exists; the risk of recurrence after one affected child is 3-4% and increases to 10% with 2 prior affected children. Nutritional and environmental factors have a role in the etiology of myelomeningocele as well. 5,6,7,10 In our case, mother denies family history of myelomeningocele, nor intake of certain drugs (eg, valproate which already known as folate antagonist ) which can also be a risk factor. In several studies, although there still a debate, folate is intricately involved in the prevention and etiology of neural tube defects (NTD). Research indicates that folate can reduce the incidence of NTD by about 70% and can also decrease the severity of these defects when they occur. Folate functions in single-carbon transfer reactions and exists in many chemical forms. Folic acid (pteroylmonoglutamic acid), occurs rarely in food but is the form used in vitamin supplements. 9,10 The mother of SSSS Page 5

our patient denies intake of folic acid supplement all throughout the entire course of her pregnancy. Because the clinical manifestations of pre-sacral masses vary, imaging also plays an important role in the detection and differentiation of these masses. Information obtained from imaging is also critical for management, especially for surgical planning. 1,13 In fact, with laboratory and radioimaging in pregnancy, neural tube defect (NTD) such as myelomeningocele actually can be detect since 2nd trimester of pregnancy. During the second trimester, pregnant women can have a blood test called the quadruple screen. This test measures levels of four pregnancy hormones. One of the four hormones that have important role in myelomeningocele is Alpha-fetoprotein (AFP). This test screen can be used to detect myelomeningocele, Down syndrome, and other congenital diseases in the baby. If the quadruple screen test is positive, further testing is needed to confirm the diagnosis. Such tests may include pregnancy ultrasound and amniocentesis 10,14,15 The measurement of maternal serum AFP levels is more than 75% accurate in detecting an open neural tube defect at more than 15 weeks' gestation. In patients in whom a question persists, amniotic AFP can be obtained. It is a significantly more accurate test, especially at 15-20 weeks' gestation, and detects approximately 98% of all open neural tube defects, although this method is not the preferred screening test. 15 In our patient, the mother did not have quadruple screens and had pregnancy ultrasound only which is a common practice in a developing country like the Philippines. 10,14,15 Antenatal ultrasound alone however is sufficient in detecting myelomeningocele because it has been reported to be an effective tool for detecting NTDs such as myelomeningocele. 16,17 Detection of a NTD with fetal ultrasonography in the hands of a skilled ultrasonographer is usually 98% specific.16 Significant proportion of abnormalities such as the myelomeningocele is only detected in the second trimester.17 Major structural abnormalities can be detected by ultrasound examination, depending on the time of ultrasound scan. The recommended time and the number of ultrasound scans vary according to the national consensus, the experience of health care providers and other related factors. In the United Kingdom, from the early 1990's pregnant women are offered serum -fetoprotein screening for NTD's at 1518 weeks of gestation and an ultrasound scan at 18 22 weeks. Likewise, in France, three ultrasound scans are usually performed around the 12th, 22nd and the 32nd week of gestation. In the USA, there is no policy about the number and timing of ultrasound scans and it is mainly performed for selected patients, such as those with a positive family history, clinical symptoms or abnormal maternal serum -fetoprotein levels. This practice is somewhat similar to other country such as the Philippines. There is a long debate regarding which screening modality should be used as both the serum fetoprotein and ultrasound are effective, but some recent studies have shown that there is a trend towards increased use of first and second trimester ultrasound scan in a number of developing countries. The sensitivity differs, where the gestation, time allowed for scan, quality of equipment, training and skills of operators may affect the prenatal detection rates17 Ultrasound in the second trimester can detect myelomeningocele by using the standard biparietal cross-sectional view of the fetal head and visualizing the lateral ventricles: the "lemon sign" denotes the scalloping frontal bones, predictive of spina bifida, further, the "banana sign" refers to the abnormally shaped midbrain and an elongated cerebellum in the Arnold-Chiari malformation. Among fetuses with myelomeningocele, the "lemon sign" can be detected in 80% and the "banana sign" in 93% of cases.17 In our patient, ultrasound results were normal. This ultrasound result definitely will come to a question at the end why the myelomeningocele was only detected upon birth and not intranatally, or is it possible for the myelomeningocele to have developed during the last AOG which is a very remote possibility. SSSS Page 6

Other studies have reported different myelomeningocele detection rates. In a large study across Europe, the range varied from 33% to 100% in different countries, with a mean of 68% however there is no adequate data of detection rate in Philippines. Lower detection rate of ultrasound for detecting myelomeningocele can be due to lack of a national screening policy for myelomeningocele, not paying enough attention to find the myelomeningocele in routine ultrasound and low resolution ultrasound equipment. 17 A low resolution ultrasound equipment which might have been used during the ultrasound of the mother at 8th month AOG which led to non-detection of the myelomeningocele might have been the most appropriate reason for such event. Nearly all patients with myelomeningocele have future complications. The common complications of myelomeningocele are bladder dysfunction (neurogenic bladder) and hydrocephalus. Bladder dysfunction may adversely affect urinary continence and quality of life, and can also lead to progressive deterioration of the upper urinary tract and chronic renal disease. Thirty to forty percent of children with myelomeningocele develop some degree of renal dysfunction. Treatment to reduce bladder pressures and minimize urine stasis often prevents or attenuates this complication.10,12 At least 80% of patients with myelomeningocele usually are associated with type II Chiari malformation which our patient does not have and they have greater tendency to develop hydrocephalus. Generally, the lower the deformity is in the neuraxis (sacrum), the less likely is the risk of hydrocephalus. The possibility of hydrocephalus developing should always be considered, no matter what the spinal level location of the myelomeningocele is. Ventricular enlargement may be indolent and slow growing or may be rapid causing a bulging anterior fontanel, dilated scalp veins, setting-sun appearance of the eyes, irritability, and vomiting associated with an increased head circumference.12 In our patient, in order to have an early detection of such condition, mother was advised to closely monitor and report the head circumference every week. Orthopedic care should also begin early. If a clubfoot is present, a cast is applied; surgery is often necessary after casting. Hip joints are checked for dislocation. Affected children should be monitored for development of scoliosis, pathologic fractures, pressure sores, and muscle weakness and spasm.10,18 At present, with the advent of newer technology, one of the possible intervention for myelomeningocele is prenatal myelomeningocele repair . Repairing a myelomeningocele in utero, rather than after birth, reduces the risk for fetal or neonatal death and shunting by age 1 substantially improves neurologic and motor outcomes. However, it is not without maternal and fetal risks.19,20

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Retrospective and prospective randomized studies suggest that fetal surgery of myelomeningocele before 26 weeks of gestation may preserve neuromotor function, reverse hindbrain herniation, and reduce the need for ventriculoperitoneal shunting. 21 For a child who is born with a myelomeningocele and who is treated aggressively, the mortality rate is 10-15%, and most deaths occur before age 4 yr, although life-threatening complications occur at all ages. At least 70% of survivors have normal intelligence, but learning problems and seizure disorders are more common than in the general population. Previous episodes of meningitis or ventriculitis adversely affect intellectual and cognitive function. Because myelomeningocele is a chronic disabling condition, periodic multidisciplinary follow-up is required for life. Renal dysfunction is one of the most important determinants of mortality. 10,12

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CONCLUSION Myelomeningocele is a birth defect in which the backbone and spinal canal do not close before birth. Although myelomeningocele can be detect as early as 2nd trimester of pregnancy, in our patient ultrasound revealed normal. Lower detection rate for detecting myelomeningocele can be due to lack of a national screening policy for myelomeningocele, not paying enough attention to find myelomeningocele in routine ultrasound and low equipment, especially In several developing country. Patient with myelomeningocele must be treated aggressively to decrease the mortality and closely monitored for possible complication in the future, because this problem might lead to disturbance of quality of life or grave and fatal consequences such as hydrocephalus which can lead to cognitive impairment and kidney failure if appropriate treatment is not undertaken. Myelomeningocele is a chronic disabling condition, and need periodic multidisciplinary follow-up for better future life.

RECOMMENDATIONS Patient must be monitored closely and continuously for possible complications like hydrocephalus. Parents/guardians/caregivers must be taught and advised to monitor patients head circumference, patients activity, bowel movement, and urine output every day. Family education is important because the management of this patient is lifelong. For any developing country, better education on proper nutrition and supplementation should be implemented. Referral of all pregnant women at 2022 weeks AOG to a well equipped ultrasound center with qualified operators for a formal search of fetal anomalies, including myelomeningocele must be available.

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APPENDIX
6/30/13 Urinalysis Color Transparency Sp.gravity Reaction Sugar Albumin Pus Cells Red Blood Cells Epithelial Cells Bacteria LIGHT YELLOW SLIGHTLY HAZY 1.005 6.5 NEGATIVE NEGATIVE 0-2 0-2 FEW RARE Leukocyte Nitrite Urobilinogen Blood Ketone Bilirubin NEGATIVE NEGATIVE NORMAL NEGATIVE NEGATIVE NEGATIVE

CBC Hgb Hct Platelet WBC Segmenter Lymphocyte Monocyte Eosinophil

6/29 14.9 40.8 323 14.84 59 24 11 6

7/8 (6am) 7/8 (7pm) 7/10 14.0 37.1 404 19.23 71 20 9 10.0 29 17.0 49 272 13.26 56 27 10 7

7/14 17.2 48 235 15.17 36 43 11 10

7/19 18.4 52 475 14.42 34 40 10 16

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REFERENCES

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