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Beth S. Gottlieb, MD, MSDivision of Pediatric RheumatologyCohen Children’s Medical Center of New YorkNorth Shore-Long Island Jewish Health System

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TitleTitleAuthorsAuthors

Steven and Alexandra Cohen Children’s Medical Center of New YorkSteven and Alexandra Cohen Children’s Medical Center of New York

TitleTitleAuthorsAuthors

Steven and Alexandra Cohen Children’s Medical Center of New YorkSteven and Alexandra Cohen Children’s Medical Center of New York

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MethodsMethods

BackgroundBackground ResultsResults

AcknowledgementsAcknowledgements

ConclusionConclusion

References References

•Clostridium difficile is an important cause of nosocomial diarrhea in adults and possibly in children. •There is a high rate of asymptomatic C. difficile carriage in children < 2 years of age.•Data on rates of carriage in older children is limited. •Many laboratories including CCMC now use highly sensitive polymerase chain reaction (PCR)-based stool assays to detect C. difficile toxin DNA.•However, a positive PCR result indicates only the presence of toxin-encoding DNA, not necessarily toxin production and disease.•Therefore, there is the potential for false positive results.•We sought to determine the background detection rate of C. difficile by PCR in pediatric patients without diarrhea but at high risk for developing CDAD.

•Informed consent was obtained & stool was collected from inpatients 1-18 years of age without diarrhea. •Stools were tested for C. difficile toxin DNA using a commercial C. difficile toxin PCR assay (Xpert C. difficile/Epi, Cepheid, Sunnydale, CA). •Results of PCR tests of stools from inpatients 1-18 years with diarrhea who had specimens submitted to the clinical laboratory were also tabulated. •A cytotoxicity assay (Cytotoxicity Assay for Clostridium difficile Toxin, Bartels, Carlsbad, CA) was performed as per the manufacturer’s instructions on PCR-positive specimens. •Demographic, clinical, and laboratory data for both groups were abstracted. •Proportions were compared using Fisher’s Exact Test and medians were compared using Mann-Whitney test.

•C. difficile PCR assays are frequently positive in hospitalized children both with and without diarrhea. •Stools positive by PCR are frequently negative by cytotoxicity testing. •Given our results, it is unclear what proportion of positive PCR tests in symptomatic patients are true-positive results (versus false-positive) given the absence of a gold standard for the diagnosis of C. difficile disease.•Our findings suggest that a positive C. difficile PCR test result in a hospitalized child with diarrhea should be interpreted with caution.

1. Ellis ME, Mandal BK, Dunbar EM. Clostridium difficile and its cytotoxin in infants admitted to the hospital with infectious gastroenteritis. Br Med J. 1984; 288: 524-26.

2. Oguz F, Uysal G, Dasdemir S. The role of Clostridium difficile in childhood nosocomial diarrhea. Scand J Infect Dis. 2011; 33: 731-733.

3. Burgner D, Siarakas S, Eagles G et al. A prospective study of Clostridium difficile infection and colonization in pediatric oncology patients. Pediatr Infect Dis J. 1997; 16:1131-1134.

4. Sandora TJ, Fung M, Flaherty K et al. Epidemiology and risk factors for Clostridium difficile infection in children. Pediatr Infect Dis J. 2011; 30: 580-584.

5. Luna RA, Boyanton BL, Mehta S. Rapid stool- based diagnosis of Clostridium difficile infection by real- time PCR in a children’s hospital. J Clin Microbiol. 2011; 49: 851-57.

Stools from asymptomatic pediatric inpatients at risk for Clostridium difficile-associated diarrhea frequently test positive for C. difficile using a commercial PCR assay.

Jill Leibowitz MD, Vijaya L Soma MD, Lorry G Rubin MD Division of Infectious Diseases

Steven and Alexandra Cohen Children’s Medical Center of New York

Stools from asymptomatic pediatric inpatients at risk for Clostridium difficile-associated diarrhea frequently test positive for C. difficile using a commercial PCR assay.

Jill Leibowitz MD, Vijaya L Soma MD, Lorry G Rubin MD Division of Infectious Diseases

Steven and Alexandra Cohen Children’s Medical Center of New York

Clinical Features PCR + PCR - P value

Median age 7 y 6.5 y p=0.71

Median Hosp days 5 4 p=0.6

Abx prior 30 days 77% 78% p=1.0

Median days Abx during past 30 days

4 3 p=0.87

Hosp. prior 60 days 31% 28% p=1.0

The authors acknowledge the contribution of the personnel of the LIJ microbiology laboratory.

Asymptomatic Inpatients

Symptomatic Patients(n=121)

PCR POS (n=25) 21%

PCR NEG (n=96) 79%

Cytotoxicity Assay(n=23)

POS (n=14)61%

NEG (n=9)39%

PICU n=15Onc n=31

Other n=65

PICU n=3 (20%)Onc n=11 (35%)Other n=11(15%)

AsymptomaticPatients(n=53)

PCR POS (n=13) 24.5%

PCR NEG (n=40) 75.5%

Cytotoxicity Assay(n=13)

POS (n=1)9%

NEG (n=12)91%

PICU n=24 Onc n=9

Other n=20

PICU n=7 (29%)Onc n=1 (11%)

Other n=5 (25%)

Treatment-Treatment involves feeding an appropriate protein

sufficient diet with attention to any other associated

nutritional deficiencies

• In our case, we treated with a standard, nutritionally-

complete formula via NG tube to overcome behavioral

feeding problems

• Must monitor for development of refeeding syndrome

manifested by:

• Hypophosphatemia

• Hypokalemia

• Hypomagnesemia

• Pancreatitis

Discussion• Kwashiorkor in North America has been reported in infants

on inappropriate low protein (often hypoallergenic) diets

• The differential diagnosis of dermatitis and edema includes:

-Atopic dermatitis, viral exanthema, staphylococcal scalded skin

syndrome, zinc deficiency, scabies, tinia corporis, Langerhans

cell histiocytosis, epidermolysis bullosa

• The diagnosis of kwashiorkor was delayed in our case ---

even with a bizarre dietary history, consultation with a

dermatologist and presentation with the typical features of

kwashiorkor --- likely due to its low prevalence in the United

States

Conclusion• When a child presents with skin manifestations and a clearly

inappropriate diet, careful consideration must be given to

conditions caused by nutritional deficiencies, including locally

rare but well-characterized nutritional diseases

 

Background• Kwashiorkor – Definition: severe protein-calorie malnutrition

characterized by relatively greater deficiency of protein over

calories

• Characteristic clinical features: edema, dermatoses (‘flaky-

paint’ dermatosis), abdominal distension, and hepatomegaly

• Common in developing regions of the world such as South

Asia and Sub-Saharan Africa due to food insufficiency

• Rare in the US without a predisposing condition.

• Kwashiorkor has been reported in US infants placed on

inappropriate diets, e.g., rice or almond milk based diets

Case History• 3.5 year old boy from suburban Long Island, NY

• Presented with a worsening, 2-month-long rash and recent

development of pedal edema with difficulty walking

• Behavioral feeding issues, characterized by a progressively

limited dietary intake, began at about 12 months of age after

several viral illnesses

• Ultimately, his diet consisted of only orange juice and a

commercial banana purée preparation over the last 4 to 6

months prior to presentation

• Clinical features:

•BMI <3%

•irritability

•rounded facies

•protuberant abdomen without hepatomegaly

•a diffuse, erythematous, pruritic, ‘flaking-paint’ rash

•pedal edema

Significant Lab Values

TEST RESULT NORMAL RANGEAlbumin 2.6 g/dL 3.3 – 5 g/dL

Transferrin 70 mg/dL 200 – 360 mg/dL

Thyroid binding globulin

11 μg/ml 12 – 26 μg/ml

Prealbumin 10 mg/dL 20 – 40 mg/dL

Linoleic acid 1232 μmol/L 1600 – 3500 μmol/L

Zinc 0.55 μg/ml 0.6 -1.2 μg/ml

Copper 0.47 μg/ml 0.8 - 1.8 μg/ml

KWASHIORKOR IN A STUBBORN TODDLER

Samuel Bitton, Toba Weinstein, Jeremiah Levine, Michael PetteiCohen Children’s Medical Center, North Shore-Long Island Jewish Health System, New Hyde Park, New York

KWASHIORKOR IN A STUBBORN TODDLER

Samuel Bitton, Toba Weinstein, Jeremiah Levine, Michael PetteiCohen Children’s Medical Center, North Shore-Long Island Jewish Health System, New Hyde Park, New York

BEFORE 48 HOURS AFTER

Erythematous, desquamating rash that involved most of the patient’s body and pedal edema, both markedly improved

after 48 hrs of NG tube feeds

 

AbstractIntroduction: Severe pediatric asthma, if not immediately and aggressively treated, may progress to acute respiratory failure requiring mechanical ventilation in the PICU. Hypothesis: Timely initiation of IV terbutaline infusion in severe asthma reduces the incidence of acute respiratory failure.Methods: A retrospective chart review of patients admitted to the PICU with severe asthma and received continuous intravenous infusion of terbutaline was conducted. The patients were divided into 2 categories; patients who were transported to our PICU from outlying emergency departments (ED) and patients who were transferred from our own ED to the PICU. We evaluated these patients’ responses to terbutaline and outcome. Results: One hundred and twenty (120) patients were studied, 42 females and 78 males with a mean age of 6.8 y ± 4.2 y. One hundred eighteen (118) patients survived and 2 patients died (brain death) in the PICU from an earlier episode of cardiac arrest at home. Thirty-five (35) patients were transferred from outlying ED(s) and 85 patients from our ED. Seventy-six patients (76) had their terbutaline infusion started prior to their arrival in the PICU and 44 patients had it started after. Seventy-six patients (63%) did not require respiratory mechanical support and were breathing spontaneously throughout their course, 21 patients (17%) received BiPAP and 23 patients (19%) required tracheal intubation and mechanical ventilation. The periods of Pre-PICU terbutaline infusion were 2.61 h ± 2.47 h, 2.04 h ± 2.54 h and 0.97 h ± 1.59 h, respectively (p=0.015). Patients transported from outlying hospitals’ ED(s) had shorter mean durations of IV terbutaline than those transferred from our ED, measuring 0.69 h ± 1.38 h and 2.91 h ± 2.47 h, respectively (p=0.000).Conclusions: Early administration of continuous infusion of terbutaline in the ED may reduce the need for mechanical respiratory (invasive and non-invasive) support in severe pediatric asthma. Patients who were transferred from outlying, non-tertiary care, hospitals’ ED(s) to our PICU, showed a higher incidence of progression to respiratory failure in association with shorter durations of pre-PICU intravenous terbutaline treatments.

Results

• Of the 120 patients studied, 118 survived and 2 died (brain death) in the PICU from an earlier episode of cardiac arrest at home.

• Thirty-five (35) patients were transferred from outlying ED(s) and 85 patients from our ED; these two categories of patients were clinically and demographically similar (figure 1).

• In 25 patients (71%) from outlying EDs, and in only 17 pts (20%) from our own ED terbutaline started after arrival in the PICU (p<0.05)

• Overall, patients from outlying EDs had a significantly shorter mean pre-PICU duration of IV terbutaline treatment than those from our ED; 0.69 h ± 1.38 h vs 2.91 h ± 2.47 h, respectively (p=0.000),

• Similarly, there was a significant difference in requirement for mechanical ventilation between the two groups; 21 pts-60%- from outlying EDs and 14 pts -16%- from own ED (p=0.001).

• Seventy-six patients (63%) did not require respiratory mechanical support; 21 patients (17%) required BiPAP and 23 patients (19%) required tracheal intubation and mechanical ventilation; the periods of pre-PICU terbutaline infusion for them were 2.61 h ± 2.47 h, 2.04 h ± 2.54 h and 0.97 h ± 1.59 h, respectively (p=0.015; figure 2).

• None of the patients experienced any life-threatening adverse events due to IV terbutaline.

Conclusions• Early administration of continuous infusion of

terbutaline in the ED may reduce the need for mechanical respiratory (invasive and non-invasive) support in severe pediatric asthma.

• Patients who were transferred from outlying, non-tertiary care, hospitals’ ED(s) to our PICU, showed a higher incidence of progression to respiratory failure in association with shorter durations of pre-PICU intravenous terbutaline treatments.

Speculations • Outlying, non-tertiary care, hospitals’ ED(s) may not

have experience with IV terbutaline, its potential side effects and the required patient monitoring; this may result in a delayed IV terbutaline administration and a higher incidence of acute respiratory failure.

Introduction

•Preventing acute asthma patients from deteriorating to an acute respiratory failure status is crucial as it reduces morbidity, mortality rate and treatment costs within the PICU. •Our study focused on the efficacy of continuous IV administration of terbutaline, in the pre-PICU phase, in severe pediatric asthma.

Hypothesis:

• We tested the hypothesis that early administration of intravenous terbutaline, well before admission to the PICU, is beneficial in preventing acute respiratory failure and its resulting need for invasive or non-invasive mechanical ventilation.

Materials and Methods

•A retrospective chart review of patients admitted to the PICU over a period of 3 years (2007 – 2010) with severe asthma and who received continuous intravenous infusion of terbutaline, was conducted. •The patients were divided into 2 categories:

• patients who were transported to our PICU from outlying, non-tertiary care hospitals’ emergency departments (ED)

• patients who were transferred from our own ED to the PICU.

•We evaluated these patients’ periods of pre-PICU IV terbutaline treatment, their responses to it and their outcome. •The study received IRB approval.

Statistical Analysis (MinitabR):

•The patients’ demographics and vital signs (respiratory rate and heart rate) prior to initiation of terbutaline drips, in the 2 categories of patients, were compared by the unpaired t test. •The periods of pre-PICU terbutaline IV treatment for patients transported from outlying hospitals’ ED and from our own ED, were also compared by the unpaired t test. •Periods of pre-PICU IV terbutaline treatment for patients who did not require any mechanical respiratory support, patients who were successfully treated by non-invasive support (BiPAP) and those requiring invasive mechanical ventilation via a tracheal tubes, were compared by ANOVA. •Non-parametric data regarding the breakdown of the number of patients requiring or not requiring mechanical ventilation, in the 2 groups, were analyzed by the Chi Square test. •We rejected the null hypothesis at a p≤0.05.

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Table 1: Patient dataFigure 1: Summary of results

Figure 2:

Early Administration of Intravenous Terbutaline in Severe Pediatric Asthma May Reduce the Incidence of Acute Respiratory Failure

Sule Doymaz1, M.D., James Schneider1, M. D., Mayer Sagy2, M. D.

The Divisions of Pediatric Critical Care Medicine at 1Cohen Children’s Medical Center of New York, New Hyde Park, NY and 2New York University Medical Center, New York, NY.

 Pts from

 nAge (years)

Loading Dose (mcg/kg

)

Max. Drip Dose

(mcg/kg/min)

Resp Rate Prior to IV Terb

Heart Rate Prior to IV Terb

Pre-PICU Terb

(hrs)

Mech. Vent.(inv+non-

inv)

Outlying

EDs35 8.43

±4.89

4.2 ±0.7

0.8 ± 0.6

39.4 ±14.1

139.9 ±20.5

0.69 ± 1.38

21/35 (60%)

The LIJ ED

85 6.26 ±3.94

4.4 ±0.6

0.7 ± 0.6

40.2 ±13.4

143.8 ± 23.7

2.91 ± 2.47*

14/85 (16%)

 p value

  NS (>0.05)

NS (>0.05)

NS (>0.05)NS

(>0.05)NS

(>0.05)*0.000 0.001

MV- Mechanical Ventilation, NIPPV – Noninvasive positive pressure ventilation (BiPAP); SV – spontaneous ventilation

 

AbstractIntroduction: Severe pediatric asthma, if not immediately and aggressively treated, may progress to acute respiratory failure requiring mechanical ventilation in the PICU. Hypothesis: Timely initiation of IV terbutaline infusion in severe asthma reduces the incidence of acute respiratory failure.Methods: A retrospective chart review of patients admitted to the PICU with severe asthma and received continuous intravenous infusion of terbutaline was conducted. The patients were divided into 2 categories; patients who were transported to our PICU from outlying emergency departments (ED) and patients who were transferred from our own ED to the PICU. We evaluated these patients’ responses to terbutaline and outcome. Results: One hundred and twenty (120) patients were studied, 42 females and 78 males with a mean age of 6.8 y ± 4.2 y. One hundred eighteen (118) patients survived and 2 patients died (brain death) in the PICU from an earlier episode of cardiac arrest at home. Thirty-five (35) patients were transferred from outlying ED(s) and 85 patients from our ED. Seventy-six patients (76) had their terbutaline infusion started prior to their arrival in the PICU and 44 patients had it started after. Seventy-six patients (63%) did not require respiratory mechanical support and were breathing spontaneously throughout their course, 21 patients (17%) received BiPAP and 23 patients (19%) required tracheal intubation and mechanical ventilation. The periods of Pre-PICU terbutaline infusion were 2.61 h ± 2.47 h, 2.04 h ± 2.54 h and 0.97 h ± 1.59 h, respectively (p=0.015). Patients transported from outlying hospitals’ ED(s) had shorter mean durations of IV terbutaline than those transferred from our ED, measuring 0.69 h ± 1.38 h and 2.91 h ± 2.47 h, respectively (p=0.000).Conclusions: Early administration of continuous infusion of terbutaline in the ED may reduce the need for mechanical respiratory (invasive and non-invasive) support in severe pediatric asthma. Patients who were transferred from outlying, non-tertiary care, hospitals’ ED(s) to our PICU, showed a higher incidence of progression to respiratory failure in association with shorter durations of pre-PICU intravenous terbutaline treatments.

Results

• Of the 120 patients studied, 118 survived and 2 died (brain death) in the PICU from an earlier episode of cardiac arrest at home.

• Thirty-five (35) patients were transferred from outlying ED(s) and 85 patients from our ED; these two categories of patients were clinically and demographically similar (figure 1).

• In 25 patients (71%) from outlying EDs, and in only 17 pts (20%) from our own ED terbutaline started after arrival in the PICU (p<0.05)

• Overall, patients from outlying EDs had a significantly shorter mean pre-PICU duration of IV terbutaline treatment than those from our ED; 0.69 h ± 1.38 h vs 2.91 h ± 2.47 h, respectively (p=0.000),

• Similarly, there was a significant difference in requirement for mechanical ventilation between the two groups; 21 pts-60%- from outlying EDs and 14 pts -16%- from own ED (p=0.001).

• Seventy-six patients (63%) did not require respiratory mechanical support; 21 patients (17%) required BiPAP and 23 patients (19%) required tracheal intubation and mechanical ventilation; the periods of pre-PICU terbutaline infusion for them were 2.61 h ± 2.47 h, 2.04 h ± 2.54 h and 0.97 h ± 1.59 h, respectively (p=0.015; figure 2).

• None of the patients experienced any life-threatening adverse events due to IV terbutaline.

Conclusions• Early administration of continuous infusion of

terbutaline in the ED may reduce the need for mechanical respiratory (invasive and non-invasive) support in severe pediatric asthma.

• Patients who were transferred from outlying, non-tertiary care, hospitals’ ED(s) to our PICU, showed a higher incidence of progression to respiratory failure in association with shorter durations of pre-PICU intravenous terbutaline treatments.

Speculations • Outlying, non-tertiary care, hospitals’ ED(s) may not

have experience with IV terbutaline, its potential side effects and the required patient monitoring; this may result in a delayed IV terbutaline administration and a higher incidence of acute respiratory failure.

Introduction

•Preventing acute asthma patients from deteriorating to an acute respiratory failure status is crucial as it reduces morbidity, mortality rate and treatment costs within the PICU. •Our study focused on the efficacy of continuous IV administration of terbutaline, in the pre-PICU phase, in severe pediatric asthma.

Hypothesis:

• We tested the hypothesis that early administration of intravenous terbutaline, well before admission to the PICU, is beneficial in preventing acute respiratory failure and its resulting need for invasive or non-invasive mechanical ventilation.

Materials and Methods

•A retrospective chart review of patients admitted to the PICU over a period of 3 years (2007 – 2010) with severe asthma and who received continuous intravenous infusion of terbutaline, was conducted. •The patients were divided into 2 categories:

• patients who were transported to our PICU from outlying, non-tertiary care hospitals’ emergency departments (ED)

• patients who were transferred from our own ED to the PICU.

•We evaluated these patients’ periods of pre-PICU IV terbutaline treatment, their responses to it and their outcome. •The study received IRB approval.

Statistical Analysis (MinitabR):

•The patients’ demographics and vital signs (respiratory rate and heart rate) prior to initiation of terbutaline drips, in the 2 categories of patients, were compared by the unpaired t test. •The periods of pre-PICU terbutaline IV treatment for patients transported from outlying hospitals’ ED and from our own ED, were also compared by the unpaired t test. •Periods of pre-PICU IV terbutaline treatment for patients who did not require any mechanical respiratory support, patients who were successfully treated by non-invasive support (BiPAP) and those requiring invasive mechanical ventilation via a tracheal tubes, were compared by ANOVA. •Non-parametric data regarding the breakdown of the number of patients requiring or not requiring mechanical ventilation, in the 2 groups, were analyzed by the Chi Square test. •We rejected the null hypothesis at a p≤0.05.

#732 Please

place NHS/LIJlogo/

emblem here

Contact Information:sdoymaz@hotmail.com

Table 1: Patient dataFigure 1: Summary of results

Figure 2:

Early Administration of Intravenous Terbutaline in Severe Pediatric Asthma May Reduce the Incidence of Acute Respiratory Failure

Sule Doymaz1, M.D., James Schneider1, M. D., Mayer Sagy2, M. D.

The Divisions of Pediatric Critical Care Medicine at 1Cohen Children’s Medical Center of New York, New Hyde Park, NY and 2New York University Medical Center, New York, NY.

 Pts from

 nAge (years)

Loading Dose (mcg/kg

)

Max. Drip Dose

(mcg/kg/min)

Resp Rate Prior to IV Terb

Heart Rate Prior to IV Terb

Pre-PICU Terb

(hrs)

Mech. Vent.(inv+non-

inv)

Outlying

EDs35 8.43

±4.89

4.2 ±0.7

0.8 ± 0.6

39.4 ±14.1

139.9 ±20.5

0.69 ± 1.38

21/35 (60%)

The LIJ ED

85 6.26 ±3.94

4.4 ±0.6

0.7 ± 0.6

40.2 ±13.4

143.8 ± 23.7

2.91 ± 2.47*

14/85 (16%)

 p value

  NS (>0.05)

NS (>0.05)

NS (>0.05)NS

(>0.05)NS

(>0.05)*0.000 0.001

MV- Mechanical Ventilation, NIPPV – Noninvasive positive pressure ventilation (BiPAP); SV – spontaneous ventilation

TreatmentTreatment

History & Physical ExaminationHistory & Physical Examination

IntroductionIntroduction FindingsFindings

ConclusionConclusion

TreatmentTreatment

ReferencesReferences

• Ulcerative colitis (UC) is a chronic inflammatory disease

• UC is limited primarily to the colonic mucosa with continuous involvement

• Etiology of UC is unknown• Evidence suggesting both genetic and environmental

factors play a role in disease pathogenesis• Vaginoplasty using sigmoid colon is an accepted

technique for creation of a neovagina• UC affecting the neovagina is a rare phenomenon

reported in only four adult patients1,2,3,4 

• 17 year old XY female with a history of gonadal dysgenesis with sigmoid graft vaginoplasty during infancy

• Initial presentation:• Bloody, mucousy vaginal discharge• Abdominal pain• Bloody diarrhea • Weight loss

• Findings on physical examination:• Perineal ulcers• Neovaginal prolapse with ulcerations• Diffuse abdominal pain • No peritoneal signs 

• First described pediatric case in which a patient developed UC in the colon with simultaneous involvement of the sigmoid neovagina

• Only reprted case in which a patient was refractory to medical therapy, including biologics

• Only reported case requiring total colectomy and vaginectomy

• Special consideration must be given to the potential long term consequences of using a colonic conduit for vaginal replacement 

• Recognition that ulcerative colitis can occur in patients with a colonic neovaginal graft is imperative when considering the risks and benefits of this technical approach to the creation of a neovagina

• Rectal bleeding and colonic inflammation can result from:• Acute or chronic infection• Intrinsic inflammatory bowel disease• Surgical diversion of segments of the colorectum

• Few case reports of UC involving a neovagina:  • In 1991, first case of UC of a neovagina and rectum

reported• Treated successfully with oral sulfasalazine2

• In 1992, patient with ulcerative colitis requiring subtotal colectomy• Neovaginal pathology consistent with diversion colitis or UC3 

• No treatment required for sigmoid neovagina  • In 2000, patient with UC of the neovagina and colon

• Treated with topical therapy4  • In 2010, patient with UC of the colon and neovagina

• Required total colectomy• Neovagina treated only with topical therapy1

1. Lima M, Ruggeri G, Randi B, et al : Vaginal replacement in the pediatric age group: a 34-year experience of intestinal vaginoplasty in children and young girls. Journal of Pediatric Surgery 2010; 45:2087-2091.2. Froese DP, Haggit RC, Friend WG: Ulcerative colitis in the autotransplanted neovagina. Gastroenterology 1991; 100:1749-1752.3. Hennigan TW, Theodorou NA: Ulcerative colitis and bleeding from a colonic vaginoplasty. Journal of the Royal Society of Medicine 1992; 85:418-419.4. Malka D, Anquetil C, Ruszniewski P: Ulcerative colitis in a sigmoid neovagina. The New England Journal of Medicine 2000; 343:369.

Ulcerative Colitis: From A to VUlcerative Colitis: From A to VToni Webster, Heather Appelbaum, Toba Weinstein, Nelson Rosen,

Jeremiah Levine Steven and Alexandra Cohen Children’s Medical Center, Hofstra North Shore-LIJ School of Medicine, New Hyde Park, New YorkSteven and Alexandra Cohen Children’s Medical Center, Hofstra North Shore-LIJ School of Medicine, New Hyde Park, New York

Ulcerative Colitis: From A to VUlcerative Colitis: From A to VToni Webster, Heather Appelbaum, Toba Weinstein, Nelson Rosen,

Jeremiah Levine Steven and Alexandra Cohen Children’s Medical Center, Hofstra North Shore-LIJ School of Medicine, New Hyde Park, New YorkSteven and Alexandra Cohen Children’s Medical Center, Hofstra North Shore-LIJ School of Medicine, New Hyde Park, New York

Figure 1.  (a) Patient lying with left side down demonstrating kissing perineal ulcers  (b) Patient in supine position demonstrating prolapsed vaginoplasty  (c)

Close view of ulcerated sigmoid neovagina and perineal ulcer

Figure 2.  (a) Neovaginal biopsy: island of intact colonic mucosa with surrounding ulceration (b) Neovaginal biopsy: chronic active colitis (c)

Colonic biopsy: colitis with erosions, granulation tissue and extensive crypt loss

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• Persistent neovaginal and colonic bleeding refractory to:• Intravenous and topical steroids• Methotrexate • Infliximab 

• Surgical intervention:• Subtotal colectomy

• Pathology demonstrated extensive mucosal ulceration and denudation with patchy hemorrhage consistent with fulminant pancolitis

• Vaginectomy and excision of remaining rectal tissue • Pathologic findings similar to previously excised colonic specimen

EvaluationEvaluation

• Significant laboratory results:• Leukocytosis• Thrombocytopenia

• Abdominal and pelvic CT:• Pancolitis

• Colonoscopy: • Active colitis with diffuse ulcerations• Neovaginal biopsies with diffuse ulcerations

DiscussionDiscussion