Spina Bifida Cystica; features and early postoperative outcomes an experience in Kampala

Byabato S (MBChB, M.Med), Kiryabwire J (MBChB, M.Med, FCS), *Galukande M (MBChB, M.Med, MSc, FCS) Department of Surgery, College of Health Science, Makerere University *Correspondence author: Galukande Moses, Department of Surgery,College of Health Science, Makerere University,Kampala, Uganda. Email: mosesg@img.co.ug

Abstract

Introduction: Spina Bifida is one of the most serious developmental disability of the infant. The prevalence of Spina Bifida varies across time, region, race and ethnicity. Worldwide, it accounts for as many as 4.7 in 10,000 live births. Although Spina bifida in its entirely is commonly seen in neurosurgical practice there is sparse literature on this disabling congenital anomaly in the Ugandan context. This study therefore aimed at describing the clinical features and early post operative outcomes.

 

Methods: A prospective descriptive study recruited children with Spina Bifida Cystica admitted to Neurosurgical ward over a period of 11 months from May 2010 to March 2011, at Uganda’s National Referral Hospital. Clinical presentations of these patients were obtained by performing a general and neurological examination and using a standardized questionnaire. The patients were followed up for two weeks to establish early outcomes.

 

Result: A total of 51 patients with Spina Bifida Cystica were studied, representing 3.9% of all patients admitted on the unit that period. Out of those 54.9% were males and 45.1% were females. The mean age of their mothers was 23.8 years and the second birth rank was the mostly affected. Family history of Spina Bifida was present in 7.8%. Nearly all patients (98%) had myelomeningocele with a most common site being lumbosacral (47.1%). Hydrocephalus was noted in 72.5% of patients before surgery and talipes equinovarus was the other most associated congenital anomaly.

 

Forty three patients were operated on. The rest 8 patients died before surgery. The mean preoperative hospital stay was 17.6 days.

Conclusion: Patients had long pre operative lapse before the definitive management was offered and all the deaths occurred during this period. Exploration of factors responsible for this delay may enhance opportuni-ties for timely intervention.

 

Key words: Spina Bifida Cystica, surgery, early outcomes

Introduction

Spina Bifida is a disorder of the cerebrospinal fluid sys-tem resulting from a failure of neural tube closure in the fetus and it can affect any part of the spinal cord. Over the past twenty-five years, a number of studies have dem-onstrated that environmental and genetic factors play an important part in Spinal Bifida and other neural tube defects etiology (1). Maternal ages, neural tube defect in previous pregnancy and low maternal folate level have been strongly associated with Spina Bifida (2,3).

 

The prevalence of Spina Bifida varies across time, by re-gion, race and ethnicity (4). Worldwide, it accounts for as many as 4.7 in 10,000 live births (5, 6). The range in prevalence in Western nations is roughly 0.1 – 1 per 1,000 live births; a few non-Western studies often quote higher rates, though again widely spread (7,8). In Africa; Airede reported the incidence of 7/1000 deliveries in the middle belt of Nigeria (9).

 

The incidence and mortality of Spinal Bifida Cystica in Uganda is not known. This study was carried out to de-scribe the characteristics of the Spina Bifida Cystica le-sions and the early post operative outcomes at Mulago Hospital in Kampala Uganda.

 

Methods

This prospective descriptive study was conducted at a neurosurgery unit of Mulago hospital. Mulago hospital is the national referral and a teaching hospital for Mak-erere University College of health sciences. It also serves as the regional referral hospital for Kampala. Fifty one patients with Spina Bifida Cystica admitted to that unit in the period of 11 months were consecutively enrolled to this study. Patients discharged before surgery against medical advice were excluded.

 

Patients were admitted to ward from the emergency ward and from neurosurgery outpatients after being reviewed by a neurosurgeon. Data collection was done using a structured and pre-coded questionnaire. History was taken followed by general and neurological examination. Birth ranking was ascertained by history provided by the mother, miscarriages before 28 weeks were not considered. The lesions were examined for the content of arachnoid sac and confirmed at surgery. The state of the lesions was noted as either ruptured or intact and any ruptured lesion with cloudy/thick drainage was considered infected. The size of the lesions were measured at their longest base diameters into small (1-3cm), medium (>3-5cm) and large (>5cm). The lesion levels were ascertained clinically into cervical, thoracic, thoracolumbar, lumbar,

 

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The muscle tone was checked and graded into normal, hypotonia and hypertonia. Hydrocephalus was determined by measurement of head circumference using tape measure and compared to the normal percentile Site of the lesion by age and sex. Cranial ultrasound scans requested for were done by senior radiologists.  The treatment given was the standard of care following the established ward protocol including but not limited to antibiotic choice and use. Mortality was considered as death of the baby before or within 2 weeks of surgery, cause of death was determined by either postmortem or on clinical basis as Size of defect discussed by the neurosurgical team. The questionnaires were checked for completeness by the principal inves- tigator. Epi info 2002 computer software package was used to analyse the data.

 

 

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before surgery was 17.6 days (range 4 – 50) (fi g. 1). No patient was operated on within 72hrs of admission. The mean age of patients at operation was 4.4 weeks (range 1 – 32). There was delayed admission for treatment of Spina Bifi da Cystica with average age on admission being 14 days. Table 2 summarizes the post op details. The cause of death to eight patients were identifi ed to

 

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be (table 2); a cardiac arrest during induction of anaes-thesia, five patients due to septicemia, one patient due to meningitis and one patient had respiratory arrest following difficulty in breathing in 2nd day of life with unknown cause of death. Antibiotics (ceftriaxone for in-patient and ampiclox for discharged patients) were given to all 43 operated patients post operatively for a period of 3 -10 days (mean 5.4 days) and preoperatively to 17 patients who had ruptured or infected lesions.

 

By the end of two weeks, 33 (76.7%) operated patients had been discharged and their mean postoperative ward stay was 5.7 days. Ten (23.3%) patients stayed in the ward for more than two weeks after operation; among those, 6 had immediate postoperative complication and 4 were waiting for the VP shunt insertion. Early postop-erative complication was statistically a significant cause of prolonged hospital stay. (RR of 7.71; 95% CI by Taylor series of 2.92-20.39 and P value < 0.05) There was no early postoperative death.

 

Seven (16.3%) patients had early postoperative com-plications which included; local wound infection in 4 patients, wound dehiscence in 2 patients and CSF leak in 1 patient (Table 3,4). Two out of 10 patients (20%) without hydrocephalus before surgery developed hydro-cephalus within two weeks of follow up. Two patients had their lower limb movement improved from weak to

Lesion characteristics

post operative complications