Gangrene of an occipital encephalocele.
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Brain↗
Biomedical subjects
J K Mahajan
Publications and source records attributed to J K Mahajan.
Extrahepatic portal hypertension: quality of life and somatic growth after surgery.
AIM: This study was carried out in order to determine whether children with extrahepatic portal hypertension show any improvement in growth parameters and quality of life after elective surgery. PATIENTS AND METHODS: All patients with extrahepatic portal hypertension who underwent surgery between April 1999 and March 2002 were studied prospectively. Height and weight before and after surgery were converted into Z scores for comparison. The quality of life was assessed by changes in scholastic ability, physical activity, social interaction, and economic effects on the family. RESULTS: At the end of a minimum follow-up period of 1 year, of the 30 children, 50 % and 76 % showed improvement in weight and height Z scores, respectively, compared to their scores while on medical management. Z scores below - 2 SD for weight and height were seen in 57 % and 37 % of patients prior to surgery, and only in 40 % and 20 %, respectively at the end of follow-up, which ranged from 1 - 4 years. The improvement in height and weight was more in those who had undergone splenectomy with either devascularization or central splenorenal shunt compared to those who had undergone side-to-side lienorenal shunt without splenectomy. The children, who had dropped out of school while on medical management, either rejoined school or attended vocational courses after surgery. There was an improvement in school performance and physical activity in 85 % of the children. Improvement in personality was seen in 73 % of the affected patients. The cost incurred for surgery was one-third of the amount spent while on medical management. CONCLUSIONS: Growth parameters improved significantly in children after surgical intervention for portal hypertension. Overall improvement in scholastic abilities, physical activity, and social interaction was noted in a majority of the patients. As a one-time procedure in a developing country, surgery is also more cost effective.
Adolescent↗
The vesicoureteral reflux dysplasia syndrome in patients with posterior urethral valves.
PURPOSE: We retrospectively reviewed outcomes in children followed for posterior urethral valves and the vesicoureteral reflux dysplasia (VURD) syndrome. MATERIALS AND METHODS: Of 65 patients with posterior urethral valves 13 fulfilled the criteria for the VURD syndrome. Patients were assessed with diethylenetriamine pentaacetic acid and dimercapto-succinic acid scans at followup to evaluate stability of renal function, presence of renal scarring and drainage of the contralateral kidney. Student's t test and the chi-square test were used for statistical analysis, with p < or = 0.05 considered significant. RESULTS: Based on the dimercapto-succinic acid scan reports, patients were classified into group 1 (6 patients), in which the contralateral kidney had no evidence of scarring, and group 2 (7 patients), in which the contralateral kidney had evidence of scars. Patients in group 1 had no breakthrough urinary tract infections or history of urinary incontinence. There was no dilatation of the contralateral upper urinary tract, and 5 of 6 patients had prompt ureteral drainage on scans. They also attained a long-term serum creatinine of 0.6 +/- 0.0 mg/dl. Patients in group 2 had a significant incidence of breakthrough urinary tract infections (p < or = 0.03) and diurnal incontinence (p < or = 0.01). Hydroureteronephrosis and slow drainage were seen in 6 of 7 patients on scans. The long-term mean serum creatinine attained in this group was 0.9 +/- 0.3 mg/dl. CONCLUSIONS: About half of the patients with the VURD syndrome had renal scarring in the contralateral kidney. These patients had evidence of hydroureteronephrosis, slow drainage of the ureter in association with breakthrough urinary tract infections and diurnal incontinence. Although serum creatinine in patients with the VURD syndrome and renal scarring in the contralateral kidney was not statistically different from that in children with a normal contralateral kidney in the short term, in the long term the deleterious effects of renal scarring, viz hypertension, proteinuria and renal failure, are likely to manifest. The presence of the VURD syndrome may not always be as good a prognostic indicator as believed previously.
Child↗
Pseudoexstrophy with epispadias.
The exstrophy variants are uncommon anomalies. The variants of the exstrophy complex have all the stigmata of the classical exstrophy such as divergent recti, widened symphysis pubis, and low-set umbilicus; however, the urinary tract is intact to a varying degree. Pseudoexstrophy, an exstrophy variant, is very rarely associated with epispadias. We report an unusual case of pseudoexstrophy with epispadias, in whom the intact bladder was initially covered by a mucous membrane which later epithelialized. The epispadias was repaired using a penile disassembly technique with posterior and ventral placement of bladder neck and urethra. Although the urethral meatus was hypospadiac, the child had achieved dry intervals with occasional stress incontinence.
Bladder Exstrophy↗
Extrahepatic portal hypertension in children: observations on three surgical procedures.
This paper presents a comparative prospective study of three modalities of surgical treatment for extrahepatic portal hypertension in children: central splenorenal shunt after splenectomy (CSS), side-to-side lienorenal shunt (SSLR) without splenectomy, and splenectomy and gastroesophageal devascularization (SGD). In an 18-month period, 27 procedures were performed: 10 CSS, 10 SSLR, and seven SGD. The outcomes were evaluated by fall in portal pressures, hematological parameters, shunt patency, splenic regression, and disappearance of esophageal varices. All three procedures were comparable in the fall of portal pressure after surgery. The average blood loss and operating time were statistically significant in favor of SSLR compared with CSS. At 3-month follow-up, shunt patency was confirmed by duplex Doppler study in all the patients in the SSLR group and in nine out of 10 patients in the CSS group. In the CSS and SGD groups, hypersplenism resolved in all the patients. In the SSLR group, blood counts improved in only five out of eight affected children. No patient re-bled during a follow-up of 3-5 years. There were no cases of hepatic encephalopathy or overwhelming postsplenectomy sepsis. In conclusion, CSS is useful when there is a large spleen, severe hypersplenism, and a shuntable splenic vein. SSLR is suitable when there is only mild splenomegaly, mild hypersplenism, and a shuntable splenic vein. Splenectomy and devascularization is the choice when there is no shuntable splenic vein.
Adolescent↗
Risk factors in the primary management of anorectal malformations in Northern India.
The purpose of this study was to determine the major factors influencing the survival of babies with anorectal malformation (ARM) during the primary management in the neonatal period in a tertiary referral center. The outcome of 125 neonates with ARM, treated in a single hospital during a 2-year period, was analyzed retrospectively with particular reference to birth weight, time of arrival, and the type of ARM, including pouch colon and major associated anomalies, to see whether any of them had a significant effect on initial survival. High ARM (HARM) was present in 75, low ARM (LARM) in 36, and pouch colon in 14 babies. There were a total of 28 deaths (22%). In babies with isolated HARM weighing >2.5 kg and brought to the hospital within the first 48 h of life, the survival was 100%, which dropped to 80% when brought later than 48 h. Of the babies with birth weight <2.5 kg and isolated HARM but who were brought to the hospital within the first 48 h of life, 80% survived, whereas of those reaching the hospital after 48 h, only 55% survived. Two babies with pouch colon and two of the four babies with LARM and associated anomalies died. In the HARM group, of the 31 babies with major associated anomalies, only 37% with birth weight <2.5 kg and 58% with birth weight >2.5 kg survived. Low birth weight, major associated anomalies, and delay in referral were the three important factors that influenced the outcomes of babies with ARM during the neonatal period.
Anal Canal↗
Oesophageal atresia and biliary atresia: a rare association.
Oesophageal atresia is known to be associated with cardiovascular, gastrointestinal, genitourinary and skeletal anomalies. Its association with biliary atresia is very rare and only a few cases have been reported in the literature. Although biliary atresia could be part of a multiple anomaly syndrome, its isolated occurrence is even rare. We present a case of oesophageal atresia associated with biliary atresia.
Abnormalities, Multiple↗
An audit of neonatal colostomy for high anorectal malformation: the developing world perspective.
A high divided sigmoid colostomy has been recommended for staged management of high anorectal malformation. We audited our cases of neonatal colostomy for high anorectal malformation to assess its effectiveness. A retrospective study was carried out of all surgical newborns admitted with high imperforate anus as the single diagnosis at our centre between December 1998 and December 2000. Morbidity and mortality were analysed after retrospective stratification into two groups (group A: birth weight >2.5 kg; group B: birth weight <2.5 kg). The chi square test was used to test the statistical significance in terms of outcome in the two groups. Overall mortality was 16%. Group A consisted of 34 babies: 30 with divided sigmoid colostomy and four with transverse loop colostomy. One baby with a divided sigmoid colostomy died from wound complications and septicaemia (mortality 2.9%). All four babies with transverse loop colostomy done under local anaesthesia survived, despite being sick on arrival. Group B consisted of 16 babies: 15 with sigmoid colostomy and one with transverse loop colostomy, with seven deaths (44%). None of the five babies with transverse loop colostomy done under local anaesthesia died, despite being sick on arrival, whereas all eight babies who died had undergone sigmoid colostomy under general anaesthesia. The difference in the outcomes of babies in groups A and B is highly significant ( p <.01). Sick, small (<2.5 kg) and septic babies arriving late to the unit do not appear to tolerate general anaesthesia and divided sigmoid colostomy well, despite that procedure's long-term advantages. Divided sigmoid colostomy has produced excellent results in babies >2.5 kg, but in the context of the developing world and limited critical care availability, transverse loop colostomy under local anaesthesia may save lives.
Colostomy↗
Prune-belly syndrome with anorectal malformation.
Associated abnormalities of other organ systems result in considerable long-term morbidity in patients of prune-belly syndrome (PBS). The gastrointestinal anomalies commonly observed with PBS are persistence of the embryonic wide mesentery and absence of fixation to the posterior abdominal wall, whereas anorectal malformations (ARM) are rarely associated with PBS. We describe one such case of PBS associated with high ARM and urethral stenosis in a one-day-old male newborn. The neonate underwent preliminary sigmoid colostomy for ARM, but he subsequently developed necrotizing gastritis and gastric perforation in the postoperative period and had to undergo partial gastrectomy and vesicostomy. The neonate developed fatal septicemia after second surgery. A review of all the reported cases of this association is presented and the dilemmas in the management of these cases are discussed.
Anal Canal↗
Bronchial transection: delayed diagnosis and successful repair.
Traumatic bronchial transection is usually recognized and repaired immediately after injury. Bronchial transection has a variety of clinical presentations due to air leak into the pleural cavity and it is very rare to have total absence of air leak from the transected bronchus at presentation. We present one such case of main right bronchus injury with total absence of initial clinical signs and symptoms, leading to a delay in the diagnosis. However, the surgical repair eight months after injury showed excellent recovery of the chronically collapsed lung.
Bronchi↗
Primary vesicoureteral reflux: progress of disease, somatic growth and renal parameters.
Thirty children (45 units) in the age group 1 day-12 yrs with primary vesicoureteral reflux were studied prospectively and periodically assessed for renal function, scarring, grade of reflux and somatic growth parameters. Four children (6 units) with grade IV reflux underwent ureteric reimplantation. Complete resolution with medical management was seen in all 12 units of grade I-III reflux and in 5 of the remaining 27 units of grade IV-V reflux over 6 months-6 yrs. This group showed highly significant improvement in height . The weight gain of the above 2 groups was statistically significant compared to those with persistent reflux. Focal defects were seen initially in 62 percent refluxing units. None of the patients showed deterioration in renal function or formation of new scars over the next 1-7 years. Three children on conservative management showed persistent growth retardation with associated breakthrough infection, hypertension, multiple renal scars and poor renal functional volume.
Disease Progression↗
Recurrent tracheoesophageal fistula after foreign body impaction.
We describe a case of recurrent tracheoesophageal fistula in a child previously operated for esophageal atresia and tracheoesophageal fistula following impaction of a foreign body. After extracting the foreign body, the diagnosis of recurrence was confirmed by esophagogram and bronchoscopy. The fistula was approached through transpleural thoracotomy and repair effected without significant difficulty. The possibility of unrecognized foreign body impactions as the etiological factor for late recurrence of tracheoesophageal fistula is discussed.
Bronchoscopy↗
Fundal tube esophagoplasty for esophageal reconstruction in atresia.
PURPOSE: This is a report of 4 patients with long gap/pure esophageal atresia, who, after an initial gastrostomy and cervical esophagostomy at birth, were treated with a new technique called fundal tube esophagoplasty. METHODS: The technique consisted of mobilization of the distal esophageal stump, division of the left gastric artery, and mobilization of upper half of stomach. Both walls of the stomach were incised in the region of the fundus starting on the lesser curvature in such a way that a tube was created out of the fundus, and the native esophageal stump appears to be an extension of this neoesophagus. This neoesophagus was brought to the neck by the retrosternal route and in a second stage, esophago-esophageal anastomosis was made. Thal's fundoplication was added in 2 cases. RESULTS: All the stages have been completed successfully in 4 babies. They have been followed up for periods ranging from 8 to 24 months after restoration of esophageal continuity. All the children are thriving. CONCLUSIONS: Fundal tube esophagoplasty (1) serves to provide a neoesophagus of natural caliber, (2) utilizes native esophagus in reconstruction, (3) does not produce a space-occupying problem in the mediastinum, and (4) preserves the gastric reservoir function. In this early experience, it appears to fulfill all the requirements of a good esophageal substitute.
Child, Preschool↗
Congenital diaphragmatic hernia associated with aplasia of left lobe of the liver.
A case of left-sided diaphragmatic hernia associated with congenital aplasia/agenesis of the left lobe (left lateral segment) of the liver is reported. This association of defects, to the best of the authors' knowledge, has not been reported previously.
Hernias, Diaphragmatic, Congenital↗
Pelviureteric junction obstruction: how much is the extent of the upper ureter with defective innervation needing resection?
BACKGROUND/PURPOSE: It is well realized that Anderson-Hyne's pyeloplasty fails to improve the drainage pattern and renal functional parameters in a few cases of hydronephrosis because of pelviureteric junction (PUJ) obstruction. The purpose of this study is to delineate the lower extent of the abnormally innervated pelviureteric junction by S-100 immunohistochemistry, which requires surgical removal. METHODS: Thirty pelviureteric junction (PUJ) specimens were analyzed by histology and by S-100 immunohistochemistry after serial sectioning of the resected ureter and compared with the variables age, calyceal separation, parenchymal thickness, differential renal function, glomerular filtration rate (GFR), and the measured constricted ureteral segment on gross examination. RESULTS: No significant correlation was noted (P >.05) when the histology was analyzed to find any association with any of the variables studied. The length of the visible constricted segment ranged from 2 mm to 15 mm (mean, 5.37 mm). The abnormally innervated segment was much longer than the length of the visible constricted segment in 24 and of the same length in 5 specimens. However, in 1 case, the abnormal innervation segment was shorter than the macroscopic constriction by 3 mm. The maximum difference in length between the visible constriction and the lower limit of defective innervation was 8 mm. CONCLUSIONS: The abnormally innervated ureteral segment below the PUJ in hydronephrosis is longer than the visible constricted segment at the time of surgery, and the ureter should be excised at least 8 mm or more beyond the visible lower limit of the constricted segment in cases of Anderson-Hyne's pyeloplasty.
Child↗
Testosterone therapy in microphallic hypospadias: topical or parenteral?
BACKGROUND/PURPOSE: Local or systemic application of testosterone is reported to stimulate penile growth. Intramuscular testosterone has been found to be effective in 50% of patients; however, variable results have been reported with topical testosterone. The current study is an attempt to compare the efficacy of intramuscular versus topical testosterone application. METHODS: A total of 26 consecutive patients with hypospadias and small penis (<2SD for given age) were studied prospectively. These patients were recruited alternately into group A or group B. Each group consisted of 13 patients. In group A, penile growth was accomplished by topical application of testosterone (Testoviron, oily solution containing testosterone propionate, 25 mg, and testosterone enanthate, 110 mg, equivalent to about 100 mg of testosterone, Schering, Germany) with a dose of 2 mg/kg/wk, for 3 weeks. While in group B, testosterone (same preparation as above) was administered by intramuscular injection weekly for 3 consecutive weeks. Penile length, diameter, and secondary effects were recorded before, during, and 3 weeks after the therapy by a single observer. RESULTS: Significant penile growth (P <.01) was noticed in both the groups of patients when compared with pretherapy with maximum response observed during the third week of therapy (reaching from an average pretherapy length of 2.0 cm and 1.8 cm to 3.18 cm and 3.11 cm posttherapy in group A and B patients, respectively). Seven patients in each group had growth of at least 50% compared with the initial size. The basal serum testosterone was within the normal range in both the groups. During therapy the serum testosterone was elevated above the basal level in all patients, but within the normal range except in 2 patients of group A. In these 2 children the serum testosterone level crossed the normal range. Linear growth did not alter significantly for the chronological age. Two patients of group A went on to have pubic hair, one of them had elevated testosterone level above the normal range. There was a surge in serum testosterone in all children, although significant penile enlargement was observed in 60% children in group A and 75% in group B. CONCLUSIONS: Although the desired therapeutic effect of testosterone was achieved in both the groups, this study failed to show any significant difference between the 2 routes of administration. However, in group A, (topical) serum testosterone crossed the normal range in 15% of patients and was associated with significant reversible side effects.
Administration, Topical↗
Anorectal malformation with malrotation of gut.
Infants with anorectal anomaly have a high risk of having other congenital anomalies, but associated gastrointestinal tract anomalies are quite rare. Malrotation of gut is rarely associated with anorectal anomaly. We report two such cases of anorectal malformation with malrotation of gut. The high index of suspicion, diagnostic difficulty and surgical management with avoidance of appendicectomy in these neonates is discussed.
Abnormalities, Multiple↗
Urethral polyp in a female newborn.
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Female↗