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2016年9月5日 星期一

[醫如往常] Senior Clerkship Day 5: Ob/Gyn Grand Round: OAB; overactive bladder syndrome

OAB; overactive bladder syndrome
R1 Dr. 謝 / Prof. 林
Symptom diagnosis: Urinary Urgent ± urgency incontinence, frequency or nocturia
  • Urgency : sudden compelling desire to urinate, difficult to defer
  • Frequency : > 8 times / 24hrs
  • Nocturia : > 1 times, wake to void
  • OAB-dry, OAB-wet : incontinence or not
    • Dry37%
    • Wet63%
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Impacts on quality of life
Depression
Daily activity
UTI
Falls, fracture
Quality of sleep
Skin infection or irritation
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Pathophysiology
  • Idiopathic
  • Inflammation
  • Myogenic  ( structure, receptors of cell membrane )
    • Enhanced coupling tetanic contraction
    • Increased connective tissue
    • M2: inhibit smooth muscle relaxation
    • M3: contract smooth muscle
    • Beta 3: smooth muscle relaxation
  • Neuroplasticity ( NGF, urothelium hypersensitivity, new spinal circuit,
    alter Na channel function, reawaken silent C-fiber )
  • 5-HT (serotonin) deficit ( Brain stem, spinal cord )
  • Low pH, high K
  • Afferent plasticity
TCA (5HT) can inhibit circuit
Lidocaine inhibit sodium
NGF antibody
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Etiology
Idiopathic
Neurologic
Spinal cord injury, Stroke, Multiple sclerosis, Dementia, Parkinson disease
Diabetic neuropathy
Inflammatory
UTI, post-RT
Structural
Tumor, BPH, Stones, post-OP
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Diagnosis
  1. Review medical history, and present symptoms
  2. Bladder diary: Record everyday urinary situation
  3. Exclude other disease, such as stress incontinence, mixed urinary incontinence, overflow incontinence, and transient incontinence
  4. Postvoid residual test, Urodynamic study ( not first-line evaluation )
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OABSS; overactive bladder symptom score questionnaire
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Pdet=Pves-Pabd
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  1. Behavioral therapies
    1. lifestyle modification, bladder training, pelvic floor muscle therapy,
  2. Pharmacotherapy
    1. antimuscarinics, Beta-3 agonists, Botulinum toxins, TCA, Capsaicin
  3. Surgery
    1. Augmentation cystoplasty, Sacral Neuromodulation, percutaneous tibial nerve stimulation
  4. Treat specific causes
    1. UTI
    2. Atrophic urethritis
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Frequent nocturia episodes, a suboptimal response to treatment, and small bladder capacity predict the need for persistent antimuscarinic therapy or re-treatment after discontinuation of antimuscarinics in female overactive bladder
Menopause, 2016 ( in press )
For predicting persistent or re-treatment for female OAB
  • Suboptimal response
  • Nocturia<2
  • VSD: volume at strong desire to void

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Discussion
  1. Nocturia : a new predictor of the need of persistent treatment or re-treatment
  2. If the change of OABSS is less than 3, continuous therapy is recommended
  3. Strong desire volume is an another predictor, thus, women with small bladder
    capacity may need continuous therapy
  4. Urodynamic study, Age, OABSS, urinary NGF/Cr, etc. are not predictors in this study.
  5. Limitation : sample size, only one medication (solifenacin), low ROC area 
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林教授講評
Severe IC(interstitial cystitis), OAB(Overactive bladder) overlap
很難治，轉給泌尿科
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Urodynamics
  • Anatomy: POP; Pelvic organ prolapse
  • Functional: OAB, SUI; Stress Urinary Incontinence
  • LUTD; Lower Urinary Tract Dysfunction
  • Sexual dysfunction: FSFI: Female Sexual Function Index
  • Anorectal incontinence



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2016年9月4日 星期日

[醫如往常] Senior Clerkship Day 3: Ob/Gyn Grand Round: Nerve-sparring radical hysterectomy

NSRH/CRH for cervical cancer
Nerve-sparring radical hysterectomy
Conventional radical hysterectomy
Outcome 沒有顯著差異
Quality of life 有顯著差異
Surgery Indication: 1B to 2A
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Cardinal lig. dissection is the key element
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Sympathetic T10-L2 Para-aortic nerve → Sup. Hypogastric plexus
Parasympathetic L2-4 Pelvic splanchnic nerve→Inf. Hypogastric plexus (IHP)
(uterosarcral lig.)

Bladder br. of IHP save part of vesicouterine lig.

Hypogastric nerve lat. Border of mesoderm
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F. Mota: EORTC Classification of radical hysterectomy
Classification of radical hysterectomy adopted by the Gynecological Cancer Group of the European Organization for Research and Treatment of Cancer

1. Simple hysterectomy (type I).
2. Modified radical hysterectomy (type II): Theuterus, paracervical tissues, and upper vagina (1–2 cm) are removed after dissection of the uretersto the point of their entry to the bladder. The uterine arteries are ligated, and the medial half of the parametria (there was no consensus in this concept) and proximal uterosacral ligaments are resected.
3. Radical hysterectomy (type III): En bloc removal of the uterus with the upper third of the vagina along with the paravaginal and paracervical tissues. The uterine vessels are ligated at their origin, and the entire width of the parametria is resected bilaterally. Removal of as much of the uterosacral ligaments as possible.
4. Extended radical hysterectomy (type IV): Differ from the type III procedure—three fourths of the vagina and paravaginal tissue are excised.
5. Partial exenteration (type V): The terminal ureteror a segment of the bladder or rectum is removed along with the uterus and parametria (supralevatorial).
A) Types II–V hysterectomies are completed witha systematic bilateral pelvic lymphadenectomy,half the way along the common iliac artery downto the femoral ring, including the presacral, bothexternal, internal, and interiliac and the obturatornodes (at least to the level of the obturator nerve).
B) Removal of the tubes and ovaries is not part ofradical hysterectomy per se.
F. MOTA, Int J Gynecol Cancer 2008, 18, 1136–1138
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FIGO stage
Endometrial cancer, Figo stage II
Primary tumor (T)


TNM
FIGO
Surgical-Pathologic Findings
Categories
Stages

TX

Primary tumor cannot be assessed
T0

No evidence of primary tumor
Tis

Carcinoma in situ (preinvasive carcinoma)
T1
I
Cervical carcinoma confined to the cervix (disregard extension to the corpus)
T1a
IA
Invasive carcinoma diagnosed only by microscopy; stromal invasion with a maximum depth of 5.0 mm measured from the base of the epithelium and a horizontal spread of 7.0 mm or less; vascular space involvement, venous or lymphatic, does not affect classification
T1a1
IA1
Measured stromal invasion ≤ 3.0 mm in depth and ≤ 7.0 mm in horizontal spread
T1a2
IA2
Measured stromal invasion > 3.0 mm and ≤ 5.0 mm with a horizontal spread ≤ 7.0 mm
T1b
IB
Clinically visible lesion confined to the cervix or microscopic lesion greater than T1a/IA2
T1b1
IB1
Clinically visible lesion ≤ 4.0 cm in greatest dimension
T1b2
IB2
Clinically visible lesion > 4.0 cm in greatest dimension
T2
II
Cervical carcinoma invades beyond uterus but not to pelvic wall or to lower third of vagina
T2a
IIA
Tumor without parametrial invasion
T2a1
IIA1
Clinically visible lesion ≤ 4.0 cm in greatest dimension
T2a2
IIA2
Clinically visible lesion > 4.0 cm in greatest dimension
T2b
IIB
Tumor with parametrial invasion
T3
III
Tumor extends to pelvic wall and/or involves lower third of vagina and/or causes hydronephrosis or nonfunctional kidney
T3a
IIIA
Tumor involves lower third of vagina, no extension to pelvic wall
T3b
IIIB
Tumor extends to pelvic wall and/or causes hydronephrosis or nonfunctional kidney
T4
IV
Tumor invades mucosa of bladder or rectum and/or extends beyond true pelvis (bullous edema is not sufficient to classify a tumor as T4)
T4a
IVA
Tumor invades mucosa of bladder or rectum (bullous edema is not sufficient to classify a tumor as T4)
T4b
IVB
Tumor extends beyond true pelvis
Regional lymph nodes (N)


NX
Regional lymph nodes cannot be assessed

N0
No regional lymph node metastasis

N1
Regional lymph node metastasis

Distant metastasis (M)


M0
No distant metastasis

M1
Distant metastasis (including peritoneal spread; involvement of supraclavicular, mediastinal, or para-aortic lymph nodes; and lung, liver, or bone)

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台灣40% 都要 RT
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Hydronephros when pregnancy is mostly at the right side due to right Ureter angulation
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2016年8月30日 星期二

[醫如往常] Senior Clerkship Day 1: Ob/Gyn

HLHS; Hypoplastic left heart syndrome

LV hypoplasia

RA→pulmonary artery
Epidemiology: United States statistics
Incidence of hypoplastic left heart syndrome is 0.16-0.36 per 1000 live births.[21] It comprises 1.2-1.5% of all congenital heart defects.[22, 23] Hypoplastic left heart syndrome accounts for 7-9% of all congenital heart disease diagnosed in the first year of life.[12] The rate of occurrence is increased in patients with Turner syndrome, Noonan syndrome, Smith-Lemli-Opitz syndrome, or Holt-Oram syndrome. Certain chromosomal duplications, translocations, and deletions are also associated with hypoplastic left heart syndrome.
http://emedicine.medscape.com/article/890196-overview#a6

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Placenta previa

前置胎盤(placenta previa)是指妊娠28週後，胎盤附著於子宮下段甚至達到胎盤下緣或覆蓋子宮頸內口其位置低於胎兒先露部。

< 病因 >
子宮內膜病變或損傷: 多次刮宮術、分娩、子宮手術史。
胎盤面積過大；
胎盤異常；
受精卵滋養層發育遲緩。
< 分類 >
根據胎盤邊緣與子宮頸內口的關係分：
  1. 完全性（中央性）前置胎盤：胎盤組織完全覆蓋子宮頸內口。
  2. 部分性前置胎盤：為子宮頸內口部分被胎盤組織所覆蓋。
  3. 邊緣性前置胎盤：胎盤附著於子宮下段，邊緣未達到子宮頸內口。
< 臨床表現 > 
症狀
  1. 妊娠晚期或臨產時，發生無誘因、無痛性反復陰道流血。
  2. 完全性前置胎盤初次出血時間早，多在妊娠28週左右，稱為警戒性出血。
體徵
  1. 一般情況與出血量有關。
  2. 婦科檢查：子宮軟，無壓痛，大小與妊娠週數相符。
  3. 由於子宮下段有胎盤佔據，影響胎先露部入盆，胎先露高浮，易併發胎位異常。
< 鑒別診斷 >
  1. placenta previa應與Ⅰ度胎盤早剝、臍帶帆狀附著、前置血管破裂、胎盤邊緣血竇破裂、宮頸病變等產前出血相鑒別。
  2. placenta previa應與Ⅰ度placental abruption鑒別；
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Mirror syndrome

56%會IUFD; intrauterine fetal demise

Mirror syndrome or triple oedema or Ballantyne syndrome is a rare disorder affecting pregnant women. It describes the unusual association of fetal and placental hydrops with maternal preeclampsia.[1]
The name "mirror syndrome" refers to the similarity between maternal oedema and fetal hydrops. It was first described in 1892 by John William Ballantyne.
It can be associated with twin-to-twin transfusion syndrome.
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REDV; Reversed End Diastolic Velocity
臍帶動脈血流阻力(PI)可以用來評估胎盤的功能。如果阻力增加，表示胎盤的功能可能不佳，常見於子癲前症、羊水不足、或子宮內生長受限(IUGR)的胎兒。

舒張型的波型逆轉(表示舒張期時血流逆流)，稱之為REDV (Reversed End Diastolic Velocity)
測量臍帶動脈血流阻力並不是常規的項目，有必要才做。臍帶動脈血流阻力也不是決定是否必須提前生產的唯一參數。
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TOF; Tetralogy of Fallot

Pink TOF with PDA
15% associated syndromes: Alagille syndrome, trisomy 21, DiGeorge

Alagille syndrome

除了膽管缺少的情形外，在以下五種臨床表徵中有其中三種：
1. 膽汁鬱積
2. 心臟疾病(常見週邊肺動脈狹窄)
3. 骨骼異常(常見蝴蝶狀脊柱)
4. 眼部疾病 (常見posterior embryotoxon)
5. 特殊的外觀長相
JAG1. 88%
NOTCH2.

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Endometrial cancer, Figo stage II
Primary tumor (T)


TNM
FIGO
Surgical-Pathologic Findings
Categories
Stages

TX

Primary tumor cannot be assessed
T0

No evidence of primary tumor
Tis

Carcinoma in situ (preinvasive carcinoma)
T1
I
Cervical carcinoma confined to the cervix (disregard extension to the corpus)
T1a
IA
Invasive carcinoma diagnosed only by microscopy; stromal invasion with a maximum depth of 5.0 mm measured from the base of the epithelium and a horizontal spread of 7.0 mm or less; vascular space involvement, venous or lymphatic, does not affect classification
T1a1
IA1
Measured stromal invasion ≤ 3.0 mm in depth and ≤ 7.0 mm in horizontal spread
T1a2
IA2
Measured stromal invasion > 3.0 mm and ≤ 5.0 mm with a horizontal spread ≤ 7.0 mm
T1b
IB
Clinically visible lesion confined to the cervix or microscopic lesion greater than T1a/IA2
T1b1
IB1
Clinically visible lesion ≤ 4.0 cm in greatest dimension
T1b2
IB2
Clinically visible lesion > 4.0 cm in greatest dimension
T2
II
Cervical carcinoma invades beyond uterus but not to pelvic wall or to lower third of vagina
T2a
IIA
Tumor without parametrial invasion
T2a1
IIA1
Clinically visible lesion ≤ 4.0 cm in greatest dimension
T2a2
IIA2
Clinically visible lesion > 4.0 cm in greatest dimension
T2b
IIB
Tumor with parametrial invasion
T3
III
Tumor extends to pelvic wall and/or involves lower third of vagina and/or causes hydronephrosis or nonfunctional kidney
T3a
IIIA
Tumor involves lower third of vagina, no extension to pelvic wall
T3b
IIIB
Tumor extends to pelvic wall and/or causes hydronephrosis or nonfunctional kidney
T4
IV
Tumor invades mucosa of bladder or rectum and/or extends beyond true pelvis (bullous edema is not sufficient to classify a tumor as T4)
T4a
IVA
Tumor invades mucosa of bladder or rectum (bullous edema is not sufficient to classify a tumor as T4)
T4b
IVB
Tumor extends beyond true pelvis
Regional lymph nodes (N)


NX
Regional lymph nodes cannot be assessed

N0
No regional lymph node metastasis

N1
Regional lymph node metastasis

Distant metastasis (M)


M0
No distant metastasis

M1
Distant metastasis (including peritoneal spread; involvement of supraclavicular, mediastinal, or para-aortic lymph nodes; and lung, liver, or bone)

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ATHBSO optimal debulking surgery
Total abdominal hysterectomy-bilateral salpingo-oophorectomy
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PGD; Preimplantation genetic diagnosis
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Spinaocerebellar ataxia SCA

「企鵝家族」
體染色體顯性CAG
ATXN1 Gene
SCA3佔在臺灣人中所有顯性脊髓小腦萎縮症的45%。

Spasticity
Opththalmoplegia
Dystonia
Chorea
Sensory neuropathy
Fasciculation

  • SCA1：第6對染色體6p23，正常人CAG重複6-36，患者39-83
  • SCA2：第12對染色體（12q24.1）；正常人CAG重複16-30，患者34-49
  • SCA3（MJD）：第14對染色體14q21；正常人CAG重複13-44，患者63-85
  • SCA6：第19對染色體 19p13.1-13.2，正常人CAG重複4-16，患者21-27
  • SCA7：第3對染色體 3p21.1-p12，正常人CAG重複7-35，患者37-130
  • DRPLA：第12對染色體（12p13），正常人CAG重複6-35，患者49-88
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Dandy-Walker syndrome, DWS

Dandy-Walker malformation (DWM) is the most common posterior fossa malformation, characterised by the triad of:
  • hypoplasia of the vermis and cephalad rotation of the vermian remnant
  • cystic dilatation of the fourth ventricle extending posteriorly 
  • enlarged posterior fossa with torcular-lambdoid inversion (torcular lying above the level of the lambdoid due to abnormally high tentorium)


  1. 小腦蚓部全部或部分缺如。
  2. 四腦室形成巨大水囊。
  3. 可能有水腦症（hydrocephalus）。
  4. 後顱窩大，小腦膜tentorial cerebelli、側竇、及竇𠠬（torcular herophili）上抬。
發生率約為 1/25,000至1/35,000，其中大部分合併水腦症，可能兼有的其中樞神經系統異常為胼胝體缺如（agenesis of corpus callosum）、大腦或小腦皮質的灰質異位症（cortical heterotopia）、腦幹神經核發育異常、裂腦畸形（shizencephaly）、及腦膨出（encephalocele）。其他系統的發育異常以心臟缺陷最為常見。
Dandy-Walker氏囊雖屬小腦畸形，但確實發生的原因不明，個別病例則有染色體的異常。Dandy-Walker氏囊可由產前超音波檢查及核磁共振檢查診斷，統計上約1-4%的胎兒水腦症病例屬於Dandy-Walker氏囊。
出生後的手術治療方法包括腦室至腹腔引流管植入手術（ventriculoperitoneal shunt）、水囊腹腔引流（cystoperitoneal shunt）、或內視鏡手術等。無其他中樞神經系統畸形或染色體異常的Dandy-Walker氏囊病例，預後比較良好。

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Inv(9)(p12q13) most common
第九對染色體倒置對胎兒腦部沒有影響 inv(9)(p11q13)據一般文獻記載為一正常之變異，佔人口比例之1-3%,此結果對胎兒沒有影響. 

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