A rare case of unilateral scrotoschisis in a full term infant delivered by Caesarean section is reported.

Aims:

To correlate the age at surgery, liver function tests, and hepatic and portal tract histo-pathological changes with surgical outcome in the form of disappearance of jaundice in extrahepatic biliary atresia (EHBA).

Materials and Methods:

This is a retrospective study of 39 cases of EHBA. There were 19 males and 10 females. Kasai's portoenterostomy (KPE) along with liver biopsy was performed in these patients; for purpose of correlation this biopsy was considered to be the preoperative biopsy. These patients were divided into three groups based upon surgical outcome: (A) disappearance of jaundice; (B) initial disappearance of jaundice with recurrence after 3 months; and (C) persistence of jaundice. Postoperatively, liver function tests and liver biopsies were repeated at 3 months after the KPE.

Results:

There were 11 patients in group A (28%), 21 patients in group B (54%), and seven patients in group C (18%). The age at surgery was comparable in all the three groups. The postoperative levels of serum bilirubin, alkaline phosphatase (ALP), and gamma glutamyl transpeptidase (GGTP) showed statistically significant improvement as compared with the preoperative levels in group A and B patients. Patients belonging to group C showed no improvement in the liver functions following surgery. The preoperative hepatic histopathological changes (hepatocellular alteration, cholestasis, bile ductular proliferation, and bile duct inflammation) showed a significant difference among the three groups; patients with lesser degrees of pre-existing histopathological changes had better outcome following surgery. Fibrosis was seen in all the three groups preoperatively but the difference was not statistically significant. Group C had significant fibrosis in more than 50% patients. Additional findings, viz. ductal plate malformation (9 patients, 23%) and giant cell transformation (19 patients, 49%) did not show any correlation with surgical outcomes.

Conclusions:

The liver function tests and the histopathological features appeared to affect the final surgical outcome of these patients. Higher degree of cholestasis, hepatocellular alteration, bile ductule proliferation, bile duct inflammation showed definite correlation with poor surgical outcome. High grade hepatic fibrosis and portal edema showed a trend towards poor outcome but did not achieve statistical significance.

Aim:

To analyse the clinicopathological features of neonatal mucormycosis

Materials and Methods:

Retrospective analysis of cases of neonatal gastrointestinal mucormycosis.

Results:

There were six neonates with male: female ratio of 1:1. Except one all were preterm babies. The clinical presentation was abdominal distension in the majority. All were clinically diagnosed as either NEC or toxic megacolon with perforation. Neonatal gastrointestinal mucormycosis was not suspected clinically in any. All the children were explored immediately. Biopsy revealed transmural hemorrhagic necrosis/infarction of the intestinal wall with fungal hyphae.

Conclusions:

The physicians should have a high index of suspicion for gastrointestinal tract mucormycosis in neonates with metabolic disturbances who present with abdominal distension and pneumoperitoneum. Early diagnosis and aggressive medical and surgical treatment may improve the outcome of neonates with this potentially lethal invasive disease.

Aims:

To analyse the factors affecting clinical and functional outcome of Veau-Wardill-Kilner palatoplasty in various types of cleft palate.

Materials and Methods:

Demographic data were retrieved from case records and a detailed speech, language and hearing and an orthodontic analysis were carried out prospectively.

Results:

Mean age at operation was 2.7 years; whereas mean age at the time of evaluation was 6 years. Most of the patients (43.3%, 13/30) had a bilateral cleft lip and palate. The postoperative fistula had developed in 31% (4/13) of the patients with bilateral clefts and in 17% (1/6) and 9% (1/11) of the patients with left unilateral and isolated cleft palate respectively (P<0.05). Eight per cent (2/24) of the patients operated before 2 years of age developed a fistula as compared to 66.6% (4/6) of the patients who had undergone a repair after 2 years of age (P<0.01). Severe speech abnormality was seen in 33.4% of the patients having postoperative fistula as compared to 16.6% of non-fistula patients (P<0.05). Derangement of speech was found in 66.6% of the patients who had undergone surgery after the age of 2 years as compared to the patients (13%, 3/24) undergoing correction before 2 years of age (P<0.05). Hearing loss was seen most commonly in patients with bilateral cleft palate as compared to the other varieties (P>0.05). Tympanic membrane (TM) abnormalities were also more common in bilateral cleft patients (P<0.05). Mean maxillary arch length, arch circumference and maxillary inter-canine and inter-molar width were significantly reduced as compared to the control group (P<0.001).

Conclusions:

Socially acceptable quality of speech can be achieved in more than 85% of the patients. The postoperative fistula is associated with poor speech; bilateral cleft and older age being the risk factors for fistula formation. Many patients require audiological surveillance even when asymptomatic. Maxillary growth is impaired in all the patients despite early surgery.

All the currently known reconstructions for aphallia are carried out around puberty. We describe a novel technique as a temporizing procedure which would see the patient through childhood. This would not cause any hindrance while undertaking any of the other established procedures later on after puberty.

A novel technique of bladder augmentation is reported, wherein the distal dilated ends of tortuous ureters were used for ureterocystoplasty while proximal remaining ureters reimplanted back into the native bladder.

A neonate with unilateral complete duplex system with congenital giant megaureter of the upper moiety presenting as abdominal lump is reported. A left upper moiety nephroureterectomy was performed. Such an anomaly with this presentation has not been reported in neonates.

A 6-month-old boy presented with pallor, large left hypochondrial mass and hepatomegaly. Computerized tomography (CT) revealed cystic lesions in bilateral adrenals, liver and retroperitoneal lymph nodes and a lytic left femur lesion. There was hemorrhagic aspirate with round blue cells. Excised left sided mass with adjacent lymph nodes and biopsies of others confirmed well differentiated neuroblastoma. He received 4 cycles of chemotherapy with remaining lesions markedly reduced at 2 months CT scan. At 2 year follow up he is doing well.

We report a case of acute urinary retention due to intravesical auto knotting of infant feeding tube in a child treated successfully by endoscopic approach.

A rare case of thoracic fetus in fetu is reported. Complete excision was curative.

Report of the use of video assisted thoracic surgery for traumatic diaphragmatic hernia repair in two children

Aim:

In children suffering from severe hepatosplenic schistosomiasis, the surgical protocol includes splenectomy and autoimplantation of spleen morsels in the major omentum, which has the potential of reducing the overwhelming postsplenectomy infection (OPSI). The purpose of the study was to evaluate the remaining splenosis, with the mean postoperative followup of 12.1 ± 5.6 years; to compare the efficacy of the 2 evaluation tools.

Materials and Methods:

Nineteen patients underwent, when they were children, portal decompression and autoimplantation of spleen. After a mean age of 23.4 ± 5.3 years, they were investigated regarding the number of infections and OPSI. They had undergone hepatosplenic scintigraphy with labeled colloidal SnTc99m and abdominal ultrasound. It was considered efficient splenosis when the patients presented with 5 or more spleen nodules. The evaluation was performed by 3 observers.

Results:

None of the patients had increased infection rate or developed OPSI. Sixteen (84.2%) presented efficient splenosis.

Conclusions:

The remaining postoperative splenosis was considered efficient in the majority of patients in the long-term followup; and nuclear medicine was considered the gold standard for splenosis evaluation.

Aims:

To analyze whether outcome of neonates having esophageal atresia with or without tracheoesophageal fistula (EA±TEF) associated with anorectal malformation (ARM) can be improved by doing surgery in 2 stages.

Materials and Methods:

A prospective study of neonates having both EA±TEF and ARM from 2004 to 2011. The patients with favorable parameters were operated in a single stage, whereas others underwent first-stage decompression surgery for ARM. Thereafter, once septicemia was under control and ventilator care available, second-stage surgery for EA±TEF was performed.

Results:

Total 70 neonates (single stage = 20, 2 stages = 30, expired after colostomy = 9, only EA±TEF repair needed = 11) were enrolled. The admission rate for this association was 1 per 290. Forty-one percent (24/70) neonates had VACTERL association and 8.6% (6/70) neonates had multiple gastrointestinal atresias. Sepsis screen was positive in 71.4% (50/70). The survival was 45% (9/20) in neonates operated in a single stage and 53.3% (16/30) when operated in 2 stages (P = 0.04). Data analysis of 50 patients revealed that the survived neonates had significantly better birth weight, better gestational age, negative sepsis screen, no cardiac diseases, no pneumonia, and 2-stage surgery (P value 0.002, 0.003, 0.02, 0.02, 0.04, and 0.04, respectively). The day of presentation and abdominal distension had no significant effect (P value 0.06 and 0.06, respectively). This was further supported by stepwise logistic regression analysis.

Conclusions:

In a limited resources scenario, the survival rate of babies with this association can be improved by treating ARM first and then for EA±TEF in second stage, once mechanical ventilator care became available and sepsis was under control.

Aim:

Our experience of 4 cases of urethral duplication is reported here.

Materials and Methods:

A retrospective chart review.

Results:

The age at presentation varied from newborn to 10 years. The clinical presentation ranged from prepubic sinus to diphallus urethra. There were 2 each incomplete duplication with only external openings (Type IA) and complete duplication of Effmann Type IIA2. All underwent complete excision of accessory urethra and corrections of associated anomalies.

Conclusions:

Urethral duplications have a varied presentation. At follow up, all are asymptomatic with good cosmetic result.

Aim:

To report the surgical complications of Ascaris lumbricoides infestation in children.

Materials and Methods:

This is a retrospective study and cases of intestinal ascariasis managed conservatively were excluded.

Results:

Sixteen children presented with Ascariasis sequelae, which included ileal volvulus (n=5), perforations (n=4), intussusception (n=1), biliary ascariasis (n-1) and impacted multiple worm boluses (n=5). Plain abdominal radiographs showed pneumoperitoneum (3), cigar bundle appearance (3) and multiple air and fluid levels (13). Sonography showed floating worms with free fluid (2), sluggish peristalsis and moderate free fluid (7) and intestinal worm bolus (11). The surgical procedures included milking of worms (in all), bowel resection (6), closure of perforation (3) and manual reduction of intussusception (1). Biliary ascariasis was managed conservatively and the progress monitored with sonography. There were 3 deaths all of whom had intestinal volvulus, bowel necrosis and toxemia.

Conclusion:

Sonography can be helpful in diagnosing the presence of worms, its complications and in evaluating response to treatment. Early surgical intervention in those with worm bolus, peritonism, and volvulus may salvage bowel and reduce mortality.

Aim:

To evaluate our results of definitive repair of anorectal malformations in patients with delayed presentation, during and beyond adolescence.

Material and Methods:

It is a retrospective analysis of all adolescent patients presenting for the first time for definitive repairs and innate patients - colostomy performed during the neonatal period, but who had lost to follow-up. It includes 15 patients (2 male and 13 female) aged from 13 to 32 years. Three well-decompressed female patients were managed by primary anterior sagittal anorectoplasty (ASARP). Twelve patients underwent staged procedures. Five patients (two male and three female) underwent posterior sagittal anorectoplasty (PSARP). The oldest male patient underwent abdominal-PSARP.

Results:

All of them attained socially acceptable fecal continence at follow-up of 1–4½ years. They are satisfied with the functional and cosmetic outcome of repair of their anomalies.

Conclusions:

Prospects of fecal continence are good when definitive repair of anorectal malformations is done by an expert, even in the adolescent age group and beyond.

Extrarenal calyces are one of the rare renal anomalies associated with the collecting system. Their association with renal ectopia or horse shoe kidney is known. But, here in, we are reporting an intraoperative surprise, where extrarenal calyces were associated with pelviureteric junction obstruction and routine preoperative imaging did not show any light on the diagnosis.

Urethral syringoceles are cystic dilatations of paired bulbourethral glands of Cowper. They can cause voiding dysfunction in male children and usually occur in isolation. We report a rare association of Cowper's syringocele with posterior urethral valves in a 4-year-old child, which was successfully managed endoscopically.

A rare case of Amyand's hernia in an infant who presented with chronic discharging scrotal sinus is reported.

Report of incidentally detected sewing needles on plain abdominal radiographs in two patients without any prior history of ingestion or of being pierced: one in the liver and the other in the peritoneum encased by omentum. These case reports point out a rare mode of entry of needles into the abdominal cavity by penetration from outside.

A child with isolated renal mucormycosis who was successfully treated with systemic anti-fungal therapy and nephrectomy is reported.

Two uncommon cases of locally invasive pulmonary inflammatory myofibroblastic tumor are reported. Diagnosis was established by a prior thoracotomy and incisional biopsy. Complete excision was curative and both children remain asymptomatic at last follow up.

A 2-year-old boy with heterotaxy syndrome with associated polysplenia, ventricular septal defect, and malrotation of gut with volvulus is presented. There was delay in diagnosis due to the unusual plain abdominal radiography findings. At laparotomy, the midgut was also found to be enclosed in a thin transparent cocoon.