The management of exomphalos

BACKGROUND/PURPOSE: The management of exomphalos in the authors' department over a 26-year period is reported together with a technique for delayed closure of the ventral hernia resulting from conservative treatment of exomphalos major. METHODS: Patients were classified into exomphalos minor and major. Exomphalos minor was treated by early surgical closure. Exomphalos major was treated preferentially conservatively with delayed repair of the ventral hernia. RESULTS: There were 104 patients (68 boys and 36 girls; exomphalos minor, 45; exomphalos major, 59). Forty-two patients with exomphalos minor underwent operation. Three patients died before surgery, and 9 others postoperatively of overwhelming sepsis. Fifteen babies with exomphalos major needed early operation (skin closure only in 3 and prolene mesh repair in 12), there were 2 preoperative and 4 postoperative deaths. Forty-two patients were treated conservatively, among these, 8 died of sepsis. Thirty-four children had closure of the ventral hernia (prolene mesh, 7 and native tissue, 27); there was no morbidity. Two children died after laparotomy for adhesive intestinal obstruction. CONCLUSION: Mortality rate was related to sepsis, complications of delayed presentation, and severe congenital anomalies. There were no ill effects attributable to mercury or iodine absorption. Delayed ventral hernia repair by double breasting of the fibrous tissue sheet underlying the skin was found to be a reliable technique with low morbidity.     

Retroperitoneal pseudocyst

There is a paucity of reports in the literature describing non-traumatic retroperitoneal pseudocysts. We report a retroperitoneal pseudocyst in a young girl that developed within a short period of time for which no etiologic factor could be elicited on the basis of the clinical, investigative, operative or pathologic findings. Treatment was by primary complete excision. We propose that such pseudocysts can form without preceding trauma.     

Sacrococcygeal teratoma

This retrospective study details our experience regarding 72 patients with sacrococcygeal teratoma treated over a period of 17 years. The sex incidence was nearly equal, but there was a high proportion of Altmann type IV tumors. A preliminary colostomy before combined abdominosacral excision of large type III and IV lesions reduced morbidity. Sixty-six percent of the patients presented beyond the neonatal period; 14 had been treated elsewhere for bowel/urinary obstruction. Imaging studies included radiography, abdominal ultrasound, computed tomography, and magnetic resonance imaging (after 1995). In 60 patients the tumor was excised via the sacral route, 11 had a preliminary colostomy, and 1 had a vesicostomy. Eight children (5 with malignant lesions) required abdominosacral excision. After 1990, serial estimation of serum alpha-fetoprotein (AFP) was used to monitor tumor recurrence. There were 34 male and 38 female patients (age range 3 days-12 years); 47 had benign tumors, of which 42 were excised through the sacral route. Three patients underwent a preliminary colostomy and abdominosacral excision of the tumor with subsequent colostomy closure. There were 4 deaths in this group; no recurrence was seen in the surviving children with benign tumors. Twenty-five patients had malignant teratomas. In 18 of these the tumor was excised via the sacral route and 5 underwent abdominal-sacral excision. Eight had a preliminary colostomy and chemotherapy followed by excision of the residual tumor and colostomy closure. None of the initial 14 patients with malignant lesions survived beyond 2 years. Of the latter 11 (who received cisplatinum-based chemotherapy), 10 were alive 1 year after surgery. One patient is currently on preoperative chemotherapy and another developed recurrence of the tumor. The overall follow-up ranged from 3 months to 8 years; there has been no complaint of functional neurological deficit in any of the patients. As intrapelvic tumors tend to have a delayed diagnosis, this can be avoided by performing a rectal examination. There should be no recurrence after excision of a benign teratoma. Cisplatinum-based chemotherapy has improved the survival of patients with malignant tumors.     

Evaluation of congenital diaphragmatic hernia in a tertiary health center of a developing country: management and survival

Aim The purpose was to evaluate the diagnosis and efficacy of management of congenital diaphragmatic hernia (CDH) in a tertiary health center of a developing country. Methods Forty-six children aged from 1 day to 7 years were studied. Parameters studied were age, sex, clinical features, and management. Results Fifty-six percent of patients presented in the neonatal period; however, none of them presented on the first day of life. The majority (91.3%) of patients had left-sided CDH. Respiratory distress was the most common clinical feature observed (91.3%). Chest X-ray confirmed the diagnosis in 82.6% of patients, and contrast study was needed in the remaining 17.4%. The survival rate was 87%. It was better in patients presenting late than those presenting in the early neonatal period. Stabilization in the preoperative period improved survival. Not using a chest tube had no adverse effect on survival. Conclusion The relatively increased survival rate of CDH in a tertiary health center of a developing country is attributed to delayed arrival to the center. Respiratory infections compound the survival. More studies are needed before it can be safely said that not using a chest tube has no adverse outcome. Late presentation has been associated with varied manifestations, hence proper clinical evaluation, a high index of suspicion and adequate management, which includes imaging and surgery after stabilization, gives excellent results.     

Long-term results of anterior sagittal anorectoplasty for the treatment of vestibular fistula

Purpose

Vestibular fistula is the commonest anorectal malformation in the female child. This article reports the treatment and long-term follow-up of 1206 patients of vestibular fistula treated by anterior sagittal anorectoplasty (ASARP) in a single center for 38 years.

Material and Methods

All patients of vestibular fistula admitted and operated on at the Department of Pediatric Surgery, King George Medical University (Lucknow, UP India), from 1970 were included in the study; the age ranged from 2 days to 40 years. The diagnosis was made by clinical examination. We differentiated between anovestibular fistula (AVF) and rectovestibular fistula (RVF) in that the latter is a longer narrow fistula closely applied to the posterior wall of the vagina. Preoperative investigations included hemogram and blood glucose. Echocardiography was done in those patients showing a physical sign of cardiac anomaly. All patients were operated on in the lithotomy position by ASARP; this was done without colostomy in 1169 patients. In 6 patients, preliminary colostomy was done because of excessive perineal excoriation, and 31 others had colostomy done elsewhere. The striated muscle complex was delineated by electrostimulation, and anoplasty was performed after anchoring the rectum within the muscle complex. Washing of the perineum after passage of stools with application of povidone-iodine ointment constituted the local care. Intravenous antibiotics were administered for 48 hours and oral antibiotics (including metronidazole) for 5 days. The patient was discharged home by the fifth day.

Results

Follow-up ranges from 3 months to 19 years; uneventful postoperative recovery was seen in 1147 patients. They had normal growth and development, normal appearance of the perineum, and a normal quality of life. Complications were seen in 60 patients (5%) of which 42 had AVF and 18 had RVF. Eight patients had postoperative wound disruption that was minor in 4 and required colostomy in the other 4. Four patients had recurrence of vestibular fistula thus creating an iatrogenic perineal canal; this could be repaired by a second ASARP in 3 patients and required colostomy and PSARP in one child. Anal stenosis was seen in 11 patients; this was treated by dilatation alone in 6 and required posterior Y-V plasty in 5. The rate of complications in RVF was lower than AVF probably because of lesser number of patients; there was no difference in stooling pattern or continence between uncomplicated patient of RVF and AVF; however, fecal staining was seen in all patients undergoing revision surgery for complications.

Conclusions

This experience with ASARP showed a good result in 95% patients in a single-stage procedure. The technical ease and minimal preoperative and postoperative measures make ASARP the procedure of choice for vestibular fistula in females at all ages. Until sufficient experience is gained, it may be safer to operate on patients with RVF under cover of a protective colostomy.     

What's your diagnosis?: Herpes simplex infection mimicking recurrent acute flares in systemic lupus erythematosus: A case report with review of literature

It is well known that there is an increased risk of infections in SLE patients on immunosuppression. These infections may mimic lupus flares with similar manifestations such as fever, oral ulcers, leukopenia/pancytopenia, renal involvement, pneumonitis, lymphadenopathy and others. The management of SLE in these two situations becomes contrasting. Viral infections are an important cause of morbidity and mortality in SLE patients but are often not suspected or investigated for. Herein, we present a case of SLE on immunosuppression who had recurrent herpes simplex infection, mimicking flares of the disease. The case highlights that one should have a high index of suspicion along with appropriate laboratory back up to diagnose these infections.