Aplasia cutis congenita with rare association and unusual presentation

Aplasia cutis congenita [ ACC] is a congenital absence of skin most commonly affecting scalp. In very rare instances it can involve extremities and trunk. It is usually diagnosed clinically at birth. Most cases are sporadic with a few reports of familial occurrence. Here, we present two cases of which one had scalp involvement correlating to antenatal methimazole intake and the other one had unusual presentation with involvement of deep tissues up to the periosteum of right lower limb. Both cases were managed conservatively and successfully discharged home. But unfortunately were lost to follow-up. ACC is a rare condition with varying degree of severity depending upon the extent and depth of the involvement of the skin and underlying tissues. Mild cases do not need specific intervention while severe cases need to be managed by a multidisciplinary team of pediatrician, dermatologist and plastic surgeon. Methimazole is a known teratogen associated with this condition hence should be avoided in pregnancy

Impact of pre-eclampsia on neonatal outcome at tertiary care center

Objective: To determine the impact of pre-eclampsia on neonatal outcome at a tertiary care hospital

Methods: This was a cross-sectional study conducted in the Neonatal Intensive Care Unit [NICU] and High Dependency Unit [HDU] of Liaquat National Hospital over a period of six months, i.e from June 2015 till December 2015. The inclusion criteria includes all neonates born to pregnant women with systolic blood pressure from 130 to 200 mmHg, Diastolic blood pressure of >110mmHg and proteinuria >1 on dip stick or >300mg/l in 24 hour urinary specimen irrespective of gestational age

The exclusion criteria was all babies born to mother with chronic hypertension, glucose intolerance, renal disease, multiple pregnancy, congenitally abnormal fetus or sepsis

Results: A total of 80 babies met the inclusion criteria and were included in our study of which only 39 babies were admitted in NICU. Majority of babies were discharged home. Approximately 48.71 % babies managed to stay for 72 hours. Moreover, the major chunk of babies were delivered moderate to late preterm in the study. A total of 23 out of 39 babies presented with IUGR [Intrauterine Growth Restriction] which constitute about 58.97% of total. Regarding the impact of eclampsia on neonatal hematological profile, 25 [64.10%] developed thrombocytopenia, 8 [20.51%] developed anaemia while 6 [15.38%] were found showing no fluctuation in their haematological profiles

Conclusion: Babies born to pre-eclamptic mothers were found to have higher rates of NICU admissions and longer period of stay compare to those who born to non-eclamptic mothers

Moreover, premature birth and Respiratory Distress Syndrome [RDS] also impose significant risk to newborn babies

Early identification and collaborative approach regarding pre-eclampsia could help us mitigate the poor outcome of neonates born to eclamptic mothers

Gullian-barre syndrome with bilateral facial nerve palsy.a rare entity

Bilateral facial nerve paralysis, as compared to unilateral palsyis an extremely rare presentation with serious aetiologies including post infectious like in Guillain-Barre syndrome, infectious including Lyme disease, Infectious Mononucleosis, autoimmune, malignancy [leukaemia] and traumas

We present a rare case of bilateral facial paralysis due to Guillain-Barré syndrome which was successfully managed at Department of Paediatrics, Liaquat National Hospital. Patient presented in June 2015, she is on regular follow-up with last visit in march 2016

Success of locally devised continuous positive pressure ventilation [CPAP] alone or in combination with surfactant therapy in the management of respiratory distress syndrome in preterm neonates

Objective: To study the effect of implementing locally devised CPAP alone or in combination with INSURE protocol [Intubation, Surfactant, Extubation] in preterm neonates with respiratory distress syndrome in a tertiary care hospital

Methods: The study was conducted from August 2012 to August 2014 in Neonatal Intensive Care Unit [NICU] of Liaquat National Hospital, Karachi. Total 113 neonates were enrolled in the study. They were grouped in two categories on the basis of severity of respiratory distress syndrome [RDS]. Infants with clinical evidence of respiratory distress and radiological evidence of patchy reticulogranularpattern were given a trial of bubble CPAPalone. Neonates with severe RDS were given INSURE protocol along with bubble CPAP. The intervention was considered to be successful if respiratory distress improved and the baby could be successfully weaned off from CPAP

Results: In this study 113 neonates were enrolled. Fifty neonates were initially managed with bubble CPAP alone out of which 29 [58%] improved. Neonates with severe RDS were 63 and received bubble CPAP along with INSURE protocol out of which 45 [71.4%] improved

Conclusion: Locally devised Bubble CPAP along with selective surfactant therapy is a safe and effective intervention in the management of respiratory distress syndrome in preterm. It decreases the need for mechanical ventilation and its subsequent complication

Effect of early release of tourniquet in total knee arthroplasty

To evaluate whether tourniquet release intraoperatively is better than postoperative release in reducing overall blood loss, duration of surgery, duration of tourniquet, length of hospital stay, wound related complications and transfusion requirement. Comparative study. Department of Surgery, The Aga Khan University Hospital, Karachi, from January 2004 to June 2007. One hundred and thirty patient files were reviewed retrospectively. Patients were divided into two groups. Group-A consisted of 65 patients with early deflation of tourniquet and group-B comprised of 65 patients with the release of tourniquet after applying compressive dressing. Total blood loss [determined by Gross method] and other study variables were noted as per objective and computed. There were 22 males and 108 females with comparable BMI. All had undergone posterior stabilized cemented total knee replacement. Calculated blood loss was 1.208 Land 1.108 Lin group-Aand B respectively [p = 0.27]. Significant increase in duration of surgery was noted in group-A patients. Four patients in group-B showed complication related to wound with 3 being minor and 1 requiring additional operation room visit. Mean length of hospital stay was 9 days. Transfusion frequency was higher in group-B despite comparable postoperative haemoglobin values. Intraoperative tourniquet release does not reduce overall blood loss with no effect in conserving blood after total knee replacement, however, this group had relatively shorter hospital stay

Low birth weight babies; frequently encountered problems at secondary level care

To determine the spectrum of problems in LBW neonates at Secondary care level and their immediate outcome. Descriptive Study. Agha Khan Hospital for Women and Children, Kareemabad and Agha Khan Hospital for Women and Children, Kharadar, from January 2009 till December 2009. Of the 4500 babies born in Agha Khan secondary hospitals, 429 were Low Birth Weight and 191 were admitted to the nursery. The ratio of males to females was 0.86:1.0 [199 males and 230 females]. Approximately 41% of the babies were less than 2 kgs and Preterm babies made up 20.9% of total low birth weight. Of the 191 babies, 99 [51.8%] had hyper bilirubinemia; 16[8.3%] had respiratory distress syndrome of the newborn; 16[8.3%] had vomiting and they were observed for necrotizing enterocolitis; 21[10.9%] had presumed sepsis; 12 [6.2%] had hypocalcemia; 11[5.7%] had hypoglycemia 08 [4.1%] had Meconium Aspiration Syndrome; 05 [2.4%] had thrombocytopenia; and 02 [1.04%] had hyperviscosity with hematocrit of more than 65%. Common causes of morbidity in LBW babies are jaundice, sepsis, Respiratory distress, hypoglycemia and hypothermia. Introduction of standard management guidelines aid in reduction of morbidity. With careful selection of cases and predetermined criteria for transfer to the tertiary level nursery, it is possible to care for a vast majority of the newborns in nurseries at secondary level

Locking Compression Plate in Musculoskeletal Oncology 'a Friend in Need'

BACKGROUND: We are presenting our experience in the use of locking compression plate (LCP) after juxta-articular oncological resections in addition to its use in pathologic fracture. METHODS: A retrospective audit of skeletal reconstruction using LCP in 25 cases of long bone tumors was performed from 2008 to 2010. Reconstruction following limb salvage surgery was done in 17 patients and internal fixation of pathological fracture was done in 8 patients. All patients were available for > 12 months of follow-up, and thus assessed for union at the resected ends. RESULTS: There were 8 males and 17 females in the study. The average age at the time of surgery was 30 years (range, 9 to 66 years). The minimum follow-up was 12 months (range, 12 to 32 months). All patients except three went on to heal successfully. Complications occurred in those three patients: wound infection in one, nonunion in another, and periprosthetic fracture in the other patient. In the remaining patients, union was achieved at an average of 6.5 months after reconstruction in curative resection and 4.75 months after fixation of pathological fractures. CONCLUSIONS: Joint sparing limb salvage surgery was made successfully possible after sekeletal reconstruction with LCP. Its use was also quite effective in pathological fractures with poor bone quality. Use of locking plates for musculoskeletal oncological reconstruction resulted in a good and predictable rate of union.

Complications of total hip replacement

To determine the factors causing complications in unilateral total hip replacement. Analytical study. The Aga Khan University Hospital, Karachi, between 2000 and 2010. During the study period, 199 patients underwent elective unilateral total hip replacement at the Aga Khan University Hospital. Patients were divided into two groups on the basis of postoperative complications within 30 days of surgery. Significant factors at 5% significance level on univariate analysis were further analyzed by multivariate logistic regression. Postoperative complications occurred in 39 patients [19.6%]; dislocation being most common in 13 patients [6.5%], followed by wound infection in four [2%], all of these patients required intervention. Other minor complications which were managed conservatively included wound infection [2.5%], urinary tract infection [2.5%], dislocation [1%], pleural effusion and pneumonia [2%], deep venous thrombosis [0.5%] and myocardial infarction [0.5%]. On univariate analysis, patients with ASA III and IV, peri-operative blood transfusion, pre-operative hip deformity and post-operative bisphosphonate use were significantly associated with complications. On multi-variate analysis, adjusted odd's ratio for perioperative transfusion [3; 95% CI: 1.17-7.7] and hip deformity [3.2, 95% CI: 1.4-3.4] was found statistically significant. Pre-operative hip deformity and perioperative blood transfusion significantly influence the rates of complication after unilateral THR

Anthropometric measurements; newborns in urban Karachi population

Determination of anthropometric measurements, especially of birth weight of newborn in first few days after birth is important for the assessment of neonatal nutritional status, gestational maturity, and prediction of early neonatal death. [1] To determine the mean birth weight, mean birth length and mean head-circumference. [2] To seek association between income of the family and mean birth weight. Analytical cross-sectional study. Setting and Period: This study was done in the pediatric department of Liaquat National Hospital from March 2003 to September 2003. One hundred full terms, normal, singleton newborn babies were included and birth weight, length and head circumference were taken within 72 hours of birth. Overall mean birth weight was 2.890 kg, mean birth length 48.245cm and mean head circumference was 34.232 cm.The% of LBW babies was 13. The mean birth weight of babies belonging to group A [born to families with income >5000 Rs/mth] was 3.044 Kg and that of group B [born to families with income <5000 Rs/mth] was 2.736 Kg. Group Ahad 8% LBW babies whereas group B had 18%. Mean birth weight, length and head circumference of boys was 2.961 Kg, 48.776 cm and 34.316 cm respectively. Mean birth weight, length and head circumference of girls was 2.788 Kg, 47.480 cm and 34.109 cm respectively. Community based studies should be conducted from time to time in order to develop our own population data

Trend of complementary therapies in paediatric age group

To assess the knowledge of mothers regarding commonly used Complimentary alternative medicines, their usage and various therapeutic choices for common ailments. A cross-sectional Survey on a predesigned proforma was done from July 2007- August 2007 in the out patient department of a tertiary care hospital. Consent was taken from the participants and 375 complete proformas were included in the study out of 500 interviewed. Of the 375 mothers who were interviewed, 237 [63.2%] believed in alternative medicine whereas 138 [36.8%] did not believe in it. Complimentary alternative medicines [CAM] was used most often in children with respiratory tract and gastro intestinal symptoms, the most frequently used therapy was Honey 58.9% and Joshanda 31.2%, followed by Heeng 11.3% and brandy being used in 2.8% for respiratory complaints. Gastrointestinal symptoms were treated with Saunf water 28.4%, mint 20.6% and ajwain 17.7%.Higher education level was associated with more complementary medicine use. Majority of mothers used it for gastrointestinal problems and respiratory problems 24% and 22.2% respectively. Mothers are aware of various available CAM and are using them for different ailments like honey for respiratory illnesses, Saunf and Ajwain for gastrointestinal problems as they have been found to have linctus and antispasmodic effects respectively. It has also been observed that the CAM was used in combination with other therapeutic modalities and not alone