Thursday, November 17, 2016

Epithelial ovarian tumors in children: some high yield points

Ovarian germ cell tumors are the most common neoplastic tumors of the ovary in children. Less common are tumors derived from the ovarian surface epithelium, accounting for 15% of ovarian neoplastic tumors. These epithelial tumors are rarely seen in premenarchal patients.

Ovarian epithelial tumors in children are either mucinous or serous. Each one of these two categories can be divided into a benign subgroup, malignant subgroup, or subgroup of tumors of low malignant potential, also known as borderline tumors (where the lack of invasion into the ovarian stroma is characteristic, despite varying levels of nuclear atypia). Thus a patient may have a mucinous cystadenoma, mucinous cystadenocarcinoma, or mucinous tumors of borderline malignancy.

The overall intraoperative management of this group of tumors is based on adult protocols. At minimum, thorough staging of the abdomen is required, with focus on careful examination of the contralateral ovary, as bilateral disease is common with these tumors.









Thursday, November 10, 2016

Do we need an esophagram to evaluate a spontaneous pneumomediastinum in kids?

The paper was published in JPS a few days after the subject was brought up amongst our group.  Basically, what do we do with a kiddo who presents with a pneumomediastinum with no antecedent history of trauma or retching, and who is otherwise doing well with no fever or signs of mediastinitis.

In this paper, the authors retrospectively reviewed 27 patient with a spontaneous pneumomediastinum(SPM) who underwent an esophagram as part of their evaluation. The authors noted that none of the patient had a demonstrable injury to the esophagus. They concluded that an esophagram is not necessary for the evaluation of SPM. They felt the source of the air in this group of patients was alveolar disruption and not an esophageal injury.

Needles to say, a series of 27 patients is not sufficient to make a final conclusion on the subject. That being said, the series helps us support any decision we make regarding clinical follow up on a patient with a SPM, who is otherwise well with no other signs of esophageal injury such as dysphagia or pleural effusion on a CXR. 

Wednesday, November 2, 2016

What's the deal with the abdoimno-scrotal hydrocele?

As it's name implies, an abdomino-scrotal hydrocele is a hydrocele that extends into the peritoneal cavity. It is a rare variant that makes up 3% of pediatric hydroceles. A recent meta-analysis by Dout et al published in the Journal of Pediatric Surgery looked at a total of 116 cases of abdomino-scrotal hydroceles in an attempt to better define this entity in terms of natural history, management, and clinical implications.

The authors describe a wide variability in natural history reported; ranging form hydroceles that spontaneously resolved to ones that continued to grow. The management strategies were equally varied; ranging from a combined inguinal/laparoscopic approaches to a more simple scrotal approach.

What I found most useful in this study was the association between these hydroceles and testicular dysmorphism.  Based on this report, the incidence of dysmorphism was around 28%. This was felt to be related to the amount of pressure exerted by these hydroceles on the surrounding tissue, including the testicular vessels.

This is relevant because it suggests that there may be benefit to more urgent intervention for an abdomino-scrotal hydrocele compared to the garden-variety non-communicating hydrocele, which one may opt to observe for a period of time.


Monday, September 28, 2015

What is the value of tumor resection in kids with high risk neuroblastoma?


In a recent review of available literature on the subject of the impact of surgical resection on the outcome of patients with high risk NB, I came across this report by Englum et al in Pediatric Blood and Cancer.  

The authors looked at the impact of gross total resection (GTR) vs less than gross total resection on overall survival in 87 patients operated on in 14 different institutions.  Although no significant difference in OS (~50%) between the two groups could be found, the data did show a significant improvement in OS when more than 90% resection of the tumor was performed.

When the data was broken down by subgroups based on extent of resection, the results were as follows: 5-year survival was 53% after GTR, 64% after more than 90% resection , 35% after less than 90% resection, and 14% after biopsy only or no surgery.

As with all retrospective studies, it is difficult to tease out potential confounding variables that determine outcome in these patients, especially with a condition as complex as neuroblastoma.  This study does, however, add some more information that can help us when deciding how hard to continue to push when that last bit of tumor just will not come off that IVC safely!


Wednesday, May 13, 2015

Do we really need screening UGI's before G tube placement?

The logic behind performing screening UGI studies before g tube placement has always escaped me.  I understand the rationale behind screening for malro before a Nissen +/- g tube, since reflux could be due to some level of partial obstruction from malro; but why UGI's for g tubes without reflux?  It certainly is not a useful test to screen for reflux.  We definitely do not need to know where the stomach is before place a g tube, so why the radiation exposure?

A study by Abbas et al noted that the incidence of unexpected malro identified by screening UGI's performed for patients undergoing a routine gastrostomy tube placement is 1.7% (5/229).

Even if one accepts that avoiding a potential catastrophic event in 2 patients is worth the risk of radiation (300 cGy/UGI study) to the other 98, this logic should be applicable to all preop patients (since the need for a g tube does not necessarily select out a population at higher risk of malrotation).
So why do we not screen all preop patients with UGI's?(not suggesting that we should, for the trolls out there!).

Some more data to help support what we do (or don't do). 

Thursday, October 30, 2014

Hepatico-Jejunostomy or Hepatico-duodenostomy after resection of a choledochal cyst?

Cholangitis!!!... is that not the main concern when food is passing right next to the hepatic ducts after a hepatico-duodenostomy (HD), with no interposed common bile  duct or sphincter of Oddi?  That was what I thought until I was told otherwise, and confirmed with the limited available evidence.

When a Kocherized (salute to the verb-nouns) duodenum can reach the hepatic ducts for reestablishing continuity after choledochal cyst resection, the laparoscopic operation becomes simpler, more physiologic, and avoids potential complications related to the roux-en-y reconstructions.  Additionally, it allows for future endoscopic access to the anastomosis in case an anastomotic stricture or intrahepatic stones develop. No much controversy there.

But what about cholangitis, how can it be OK for food to pass right by the hepatic ducts and not cause an infection.

In a paper by Santore et al out of CHOP, the authors retrospectively reviewed charts of 59 patients who underwent open resection by the same surgeon with reconstruction using either HD (66%) or HJ (34%). After a mean of 2.3 years follow up for HD and 3.5 years follow up for HJ, only one patient in the HJ developed cholangitis.  Additionally, there was no significant difference in the rate of postoperative leaks or strictures.

Another argument against reconstruction with a HD relates to the risk of bile gastritis.  A meta-analysis of several papers with a total of 679 patients (60% of whom underwent reconstruction with a HD) suggested that patients who are reconstructed using a HD are at increased risk of bile gastritis (5.9% for HD vs. 0% for HJ).  It is clear how reconstruction with a HJ completely diverts bile away from the stomach and thus prevents any bile gastritis.  It is unclear to me, however, how placing the anastomosis in the duodenum, and downstream of a perfectly healthy pylorus, is any different from the normal physiologic drainage of bile, and why that would cause gastritis.

At this point, it seems reasonable to me to chose a HD over a HJ if technically feasible.



Thursday, October 2, 2014

1.7 cm carcinoid in the appendectomy specimen, now what?

I have yet to walk away from a conversation about the management of incidentally found carcinoid tumors of the appendix satisfied.  Today, I was determined to find some kind of an answer.

My basic understanding is that an incidentally found lesion which is < 2 cm and with negative margins is essentially treated with the appendectomy.  Otherwise, a hemicolectomy is needed.

If we were to extrapolate form the adult literature, the simplest recommendation is that of the NCCN version 2.2014 (National Comprehensive Cancer Network). Simply stated, a tumor < or = 2cm completely removed with the specimen is essentially cured with no further operative management needed.  Surveillance is performed "as clinically indicated". The document does acknowledge the fact that the management of tumors between 1 and 2 cm with poor prognostic factors such as mesoappendiceal invasion, lymphovascular invasion, or atypical histologic features, is controversial.

The NANETS (North American Neuro Endocrine Tumor Society) guidelines officially take into account local invasion.  They recommend right hemicolectomy for patients with evidence of tumor invasion to the base of the appendix, if the tumor is >  2 cm, if tumor size cannot be determined, if tumor is incompletely resected, if there is lymphovascular invasion or invasion of the mesoappendix, in patients with intermediate or high grade tumors, and in patients with mixed histology tumors.

A recent paper, Kim et al reported their experience with the management of incidentally found carcinoid tumors. The authors noted that out of their 13 cases, only one tumor was larger than 2 cm (2.1 cm) and that was the only patient, out of three who had a right hemicolectomy, who was found to have regional metastatic disease.

As is the overall case with carcinoid of the appendix, the numbers are just too small to make solid recommendations, especially for tumors in the 1 to 2 cm range. A good conversation with the patient and family about the numbers and management options is central to decision making.

So much for finding an answer today.