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INTRODUCTION Encephalocele, sometimes known by the Latin name cranium bifidum, is a neural tube defect characterized by sac-like protrusions of the brain and the membranes that cover it through openings in the skull. These defects are caused by failure of the neural tube to close completely during fetal development. Encephaloceles cause a groove down the middle of the skull, or between the forehead and nose, or on the back side of the skull. The severity of encephalocele varies, depending on its location. Encephaloceles are generally classified as nasofrontal, nasoethmoidal, or naso-orbital, however, there can be some overlap in the type of encephalocele. If the bulging portion contains only cerebrospinal fluid and the overlaying membrane, it may be called a meningocele. If brain tissue is present, it may be referred to as an encephalomeningocele. PATIENTS DATA Jomarie Mae Cabug, 6 days old, born on November 28, 2012 time 3:00 A.M. at Diosdado Macapagal Hospital in Caloocan City was admitted at DJFMH on December 3, 2012 at 10:00 P.M. with chief complaints of seizure episodes and presence of mass at nasofrontal area. ADMITTING DIAGNOSIS Encephalocele, Naso Frontal Area, Sepsis Neonatorum PATIENTS HISTORY Case of 6 days old, female, admitted at DJFMH due to seizure episodes, and presence of mass nasofrontal area.Admitted by DR. LOCQUIAO,Resident Pediatrician. Born to 34 years old G4P3(3-0-1-3) delivered by a normal spontaneous delivery at Diosdado Macapagal Hospital in Caloocan City. Apgar score 8, 9 Ballard Scoring 38 weeks, Body Weight 2800 grams. PNCU at Diosdado Macapagal Hospital 8 times, which started at three months Age of Gestation with regular intake of multivitamins and ferrous sulfatel.Abdominal ultrasound done at 4 months AOG,which revealed normal result. At 6 mos AOG, urinalysis revealed normal result. Repeat transabdominal ultrasound done at 9 months AOG no congenital anomaly was noted except findings of microcephaly. There was no maternal illness noted during the duration of pregnancy. At birth, patient was noted with nasofrontal mass, directly admitted at Neonatal Intensive Care Unit, hook to oxygen support via cannula and venoclysis with D10Water. Started with Ceftazidime 150mg every 12 hours and Amikacin 15mg once a day. At 4 A.M. patient was noted with seizures episodes 3 times, given with diazepam, started with Phenytoin. Antiboitics Ceftazidime was shifted to Cefuroxime 200mg every 12 hours. No laboratory work up was done or requested. At 5 A.M., seizure episodes became more frequent incurring every 15 minutes. They decided to transfer patient to Philippine General Hospital, but was not carried out due to financial condition. FAMILY HISTORY Father: Age 32 yrs. Occupation: None Educational Attainment: High School Graduate Mother: Age 34 yrs. Occupation: Housekeeper Educational Attainment: High School Graduate Siblings :( number/health status) G1- Male 18years old- High School Graduate G2-Female -16 years old High School Graduate G3-Patient No history of Allergy, Tuberculosis, Blood Disease , Renal Disease , Cardiac Disease, Hypertension, Diabetes, Asthma. SOCIAL INFORMATION: Number of household members 8 Housing owner (parents) room poorly ventilated Water supply Maynilad Toilet utilization- manual flushes latrine





SYSTEM REVIEW CNS : (+) seizure RSPI : (- )cough CVS : (-) DOB ENDOCRINE : (-) tremor GIT : (-) diarrhea GUT : (- ) hematoma PHYSICAL EXAMINATION General survey Poor activity on stimulation NICRD Vital Signs CR : 138- 100 bpm Temperature : 33.32 RR : 30 bpm shallow breathing Anthropometric measurements Wt - 2863 grams HC 30 cm CH 32 cm AC 28 cm HL 45 cm REGIONAL EXAMINATION HEENT microcephaly, open flat fontanelle , (+) mass nasofrontal area with yellowish discharge, (-) flaring , pinkish lips CHEST, HEART & LUNGS : ( inspection, palpation, percussion, auscultation ) SCE, no retraction, BS (+) wheezes, Adynamic Precordion Bradycardia regular rhythm (+) mass ABDOMEN : globular abdomen, umbilical stump dry, soft no palpable mass GENITALIA : grossly normal, majora covering minora RECTAL EXAM : no gross deformities, good pulse SKIN : good skin NEUROLOGICAL EXAMINATION: Reflexes (for new born) (+) grasp reflex


DIAGNOSTIC PROCEDURES Admitted at Dr. Jose Fabella Medical Hospital on December 3, 2012 at 10 P.M. with admitting diagnosis as Encephalocele, Naso Frontal Area Sepsis Neotorum. Significant laboratory data is as follows: DEPARTMENT OF PATHOLOGY AND LABORATORIES COMPLETE BLOOD COUNT Hemoglobin Hematocrit RBC MCV MCH MCHC WBC DIFFERENTIAL COUNT Neutrophil Lymphocytes Monocytes Basophils Eosinophils Platelet Sodium Potassium Chloride Result 159 0.47 4.46 104 36 34 10.3 0.60 0.25 0.01 0.07 0.07 198 RESULT 138.37 L mmol/L 4.96 mmol/ L 97.06 Lmmol/L Norma Values 140 - 220 g/L 0.30 - 0.60 % 3.9 - 5.5 x10-12/L 98 108 fL 31 37 pg 30 33 g/Dl 3 19.5 x 10n9/L 0.00 0.61 % 0.01 0.31 % 0.00 0.01 % 0.00 0.03 % 0.00 0. 03 % 84 478 x 10 9/L REFERENCE RANGE 146 157 3.7 5.5 101 110

ULTRASOUND REPORT December 4,2012 Cranial Sonogram shows a single midline ventricle and echogenic fused thalami,This is absence of the right temporo parietal lobe and corpus callosum. No abdominal calcifications noted. Impression: Consider Alobar Holoproscencephaly ,Absence of the Right Temporoparietal Lobe and Corpus Callosum. HOSPITAL COURSE: The patient was admitted at Pediatric Intensive Care Unit (PICU) on December 3,2012 at 10:00 P.M. with vital signs :CR : 138-100 bpm , Temp: 33.3C 36.8C , Wt: 2863 grms., TFR : 100 (2863grms) TFI : 429.45 cc ,O2 at 3LPM via nasal cannula , (+) focal seizure , Hgt :80mg/dl. Diazepam TIV given stat at Left hand IV. Emergency intubation done with size 3 ET at level 8 cm. With IVF D5IMB at 142cc and Ca 3cc with a total level of 145 cc to run for 8 hrs for 24 hours (17 -18 gtts. /min.) . Laboratories requested as follows: CBC, CXR AP/LAT and Skull X-Ray CRANIAL. , Blood Culture, Serum Electrolyte. Medications: Cefotaxime 150mg IV every 12hours (D3) ,Amikacin 43mg TIV once a day (D3) .Dopamine Drip started 0.5cc incorporated to D5Water 12cc total 12,5cc to run at 0.5cc per hour using an infusion pump .Loading of Phenobarbital 57 mg TIV (20) then 7 mg TIV every 12 hours as MD (5) to be given12 hours after the loading dose. Hook to SIMV with the following set up: RO2 - 60% Rr 40%, PIP/PEEP 15/5. Closely monitored for the recurrence of seizure. Kept thermoregulated. On December 9, 2012, patient was seizure free for 24 hrs. Blood culture result to be followed up. Medications as follows: Phenobarbital 7mg TIV every 12hrs.Cefotaxime 285mg TIV every 12 hrs. Phenytoin 15mg TIV every 12 hrs. For possible lumbar puncture once wean from ventilator. Diet: Gavage Feeding 5cc every eight hours with special aspiration precaution .Check for residuals. Intake and output was monitored every shift .Kept thermoregulated .Closely watch for the recurrence of seizure episode. On isolette,fairly active,with oxygen support via endotracheal tube connection to mechanical ventilator in SIMV MODE F1O2-RR/PEEP-15/5 .With on going IVF infusing well @ right foot with side drip of D5W 12cc + Dopamine 0.5cc : OGT draining to cloudy secretions,with heplock noted to the left hand ,afebrile ,T-37.2 C,suctioned secretions as needed,kept dressing on nasofrontal area.



ENCEPHALOCELE: This is a rare disorder in which the bones of the skull do not close completely, c r e a t i n g a g a p t h r o u g h w h i c h c e r e b r a l s p i n a l fluid, brain tissue and the membrane that covers the brain can protrude into a sac-like formation. CLASSIFICATION OF ENCEPHALOCELE Encephaloceles are generally classified into the following categories based on where they occur and what they involve: nasofrontal - present in the nose and the forehead

nasoethmoidal - present in the nose and the ethmoid sinus

meningocele - if the bulging portion contains only cerebrospinal fluid and the overlaying membrane

encephalomeningocele - if brain tissue is also involved

SYMPTOMS OF ENCEPHALOCELE Build up of too much fluid in the brain Complete loss of strength in the arms and legs An unusually small head Uncoordinated movement of the voluntary muscles, such as those involved in walking and reaching Developmental delay Vision problems Mental and growth retardation Seizures DIAGNOSTIC PROCEDURES Ultrasound CT Scan MRI TREATMENT: Craniotomy Craniofacial reconstruction Endoscopic Endonasal Approach (EEA) Minimally invasive surgical procedure uses the nose as a natural corridor to reach and remove these lesions. There are no incisions in the face or skull.



Currently, the only effective treatment for encephaloceles is reparative surgery, generally performed during infancy. The extent to which it can be corrected depends on the location and size of the encephaloceles; however large protrusions can be removed without causing major disability. Surgery repositions the bulging area back into the skull, removes the protrusions, and corrects the deformities, typically relieving pressure that can delay normal brain development. Occasionally, shunts are placed to drain excess cerebrospinal fluid from the brain. The goals of treatment include:

closure of open skin defects to prevent infection and desiccation of brain tissue removal of nonfunctional extracranial cerebral tissue with water-tight closure of the dura total craniofacial reconstruction with particular emphasis on avoiding the long-nose deformity (nasal elongation that results from depression of the cribiform plate and nasal placode). Without proper management, the long-nose deformity can be more obvious after repair.


(Clinical Practicum)

Submitted by: Lourdes S. Mangahas, R.M.

Submitted to: Ms. Teresita T. Parica, R.N.,R.M.,M.A.N.