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Obstetric & Pediatric

combine meeting
Presenter: R3 劉庭維
Supervisor: 呂孟哲醫師、陳瑛芳主任
General profile
• Chart no: 154xxx14
• Name: 陳 o 柔之子 B
• Gender: male
• Gestation age: 37+2 weeks
• Date of birth: 2022/12/18 10:42
• Admission: 2022/12/18 10:42
Chief complaint
• Cleft lip and palate and large port
wine stain over left face, trunk, and
limbs after birth.
Maternal History
G1P1
in vitro fertilization(+)
- Medical History :
HBsAg(-) HBeAg(-)VDRL(-) Anti-HIV(-) GBS(-)
- During Pregnancy :
Preeclampsia : No
Eclampsia : No
Abruptio Placenta : No
Placenta Previa : No
Diabetes Mellitus : No
Birth History
Standby (-)
- Type of Delivery : C/S
Twin B, GA 37+2 wks, BBW: 2505gm(AGA), Body height: 44cm,
Head circumference:33 cm
- Apgar score: 9->10
Asphyxia : No
Meconium stain : No
Nachal cord : No
Resp. Distress : No
DOIC( > 1 min) : No
Present Illness
Before birth • Level II echo: Cleft palate and cleft lip
were noted

After birth • Cleft palate and cleft lip


• Large port wine stain over left face,
trunk, and limbs
Physical examination
General appearance:
Acute ill looking
Vital signs:
T(℃): 36.5, P(/min): 150, RR(/min): 46, BP(mmHg):78/26, SpO2: 100%
Head: No bulging or
depressed anterior
Chest: fontanelle
No deformity,
Symmetric expansion ENT: enlarged cornea
with decreased sclera
CV: at left eye, cleft lip,
RHB, no audible heart cleft palate
murmur Skin:
Large port-wine stains
Abdomen: soft, flat over bilateral face, trunk,
normoactive bowel and limbs,
sound
Extremities:
Left upper limbs slightly
bigger than right upper
limb
cleft lip, cleft palate
1. Large port-wine stains over bilateral face, trunk, and limbs
2. Left upper limbs slightly bigger than right upper limb
1. Large port-wine stains over bilateral face, trunk, and limbs
2. Left upper limbs slightly bigger than right upper limb
enlarged cornea with
decreased sclera at left eye
Impression
1. Twin B, term male baby with gestational age 37+2
weeks, birth weight 2505 grams, delivered by
cesarean
2. Major congenital anomaly with cleft lip and palate,
highly suspected left eye glaucoma, large port-wine
stains over left face, trunk, and limbs, bigger size at
left upper limb
=> suspected Sturge-Weber syndrome
=> suspected Klippel-Trenaunay syndrome
Plan
Diagnostic:
• Check CXR and KUB
• Check CBC/DC, CRP, IgM, Ca, glucose, B/C
• Arrange brain echo, heart echo, abdominal echo
• Consult ophthalmologist for left eye highly suspected
glaucoma
• Family already contact TMUH plastic surgeon Dr. Chen
after prenatal level II echo showed cleft lips and cleft
palate
Therapeutic:
• Use special bottle for cleft palate and lips
WBC( 白血球計數 ) 12.92 ×10^3/μL [9.10-34.00]

RBC( 紅血球計數 ) 4.15 ×10^6/μL [4.70-6.10]


HGB( 血色素 ) 15.3 g/dL [15.0-24.0]
HCT( 血比容 ) 42.7 % [44.0-70.0]
MCV( 平均血球容積 ) 102.9 fL [99.0-115.0]
MCH( 平均紅血球血紅素
36.9 pg [33.0-39.0]
量)
MCHC( 平均紅血球血紅素
35.8 g/dL [31.0-37.0]
濃度 )
RDW-CV( 紅血球體積分布
14.8 % [11.0-16.0]
寬度 )
Platelet( 血小板計數 ) 247 ×10^3/μL [84-478]
Neutrophil( 嗜中性白血球 ) 59.0 % [40.0-74.0]
Lymphocyte( 淋巴球 ) 21.3 % [19.0-48.0]
Monocyte( 單核球 ) 9.8 % [3.4-9.0]
Eosinophil( 嗜酸性白血球 ) 4.9 % [0.0-7.0]
Basophil( 嗜鹼性白血球 ) 3.3 % [0.0-1.5]
Atypical lymphocyte( 非典型
1.7 % [0.0-0.0]
淋巴球 )
CRP (C- 反應蛋白 ) <0.10 mg/dL [0.00-0.50]
Ca ( 鈣 ) 9.0 mg/dL [9.0-10.6]
Random Glucose 57 mg/dL [80-140]
IgM <5.0 mg/dL
Ophthalmologist consultation
• IOP(Intraocular pressure): 14/16 mmHg (within normal limit)
• Eyelid: Port-wine stain at left V1 dermatone with eyelid involvement
• Cornea: (od) clear, 10mm in diameter (os) opacity, 10mm in diameter
• Conjunctiva: (ou) np
• AC: (ou) formed
• Lens: (ou) clear
• Fundus: (os) no choroid hemagioma was noted
[Assessment]
• Corneal opacity (os), cause to be determined, r/o SWS
• Regular oph. OPD f/u.
Cardiac echo
• PFO VS ASD 0.4 cm with left to right atrial
shunt
• Normal LV function
• Echo follow up in 3 months
Abdominal echo
• The abdomen is distended with gas.
• Liver: Homogeneous echogenicity, no enlargement, no space taking lesion.
• Spleen: Negative.
• Pancreas: Not visualized due to gas.
• Gall bladder : Not distended.
• Biliary tree: Negative.
• Right Kidney and perirenal area: central echo separation, 0.374 cm.
• Left Kidney and perirenal area: central echo separation 0.337 cm.
• Aorta and IVC of upper abdomen: Negative.

Imp.:
• 1.Bilateral pelviectasis, mild
• 2.Gaseous abdomen.

• Suggest F/U 3-6 months later.


Brain echo
• There were increase echogenicity over left
parenchyma was found over frontal, parietal and
periventricular area.
• No subdural space presented over bilateral
frontoparietal convexities

• Impression: left brain PIH(periventricular


intraparenchymal hyperechogenicity) r/o brain
edema r/o sturger weber syndrome
Admission course
• He could tolerate oral feeding under special
bottle for cleft palate and lips without lip
cyanosis, dyspnea or choking.
• No specific signs and symptoms.
Discharge diagnosis
1. Twin B, term male baby with gestational age 37+2 weeks,
birth weight 2505 grams, delivered by cesarean.
2. Major congenital anomaly with cleft lip and palate,
congenital left corneal opacity, large port-wine stains over
left face, trunk, and limbs, bigger size at left upper limb,
suspect Sturge-Weber syndrome or Klippel-Trenaunay
syndrome, other syndrome can’t be totally excluded
3. Atrial septal defect or patent foramen ovale
4. Left brain periventricular intraparenchymal
hyperechogenicity
5. Bilateral renal pelviectasis, mild
Discussion
Cleft lips and palate

Uptodate “Etiology, prenatal diagnosis, obstetric management, and recurrence of cleft lip and/or
palate”
Cleft lips and palate
• Infants with a palatal cleft generally cannot
generate sufficient negative intraoral pressure to
suck milk effectively from bottle or breast but often
can be bottle fed with formula or expressed breast
milk with use of adaptive feeding equipment (eg,
squeezable bottle, modified nipple, spoon).
• Infants with cleft lips and no palatal abnormality
often can feed from bottle or breast without
special devices.

Uptodate “Neonatal brachial plexus palsy”


Squeezable bottle with modified nipple
貝親奶瓶的特色是
1. 使用塑料奶瓶,餵奶時可以擠壓,幫助
奶水進入寶寶嘴裡。
2. 較大較軟的奶嘴,較大的奶嘴比較不會
滑到裂隙 ( 鼻腔 ) 中。奶嘴有凹槽的一邊
較硬,另一邊較軟,使用時較軟側要靠
舌頭這邊,較硬側靠上顎。寶寶靠舌頭
的壓力讓奶嘴開口打開,讓奶水進入口
腔,不必經過吸吮的動作。
3. 奶嘴開口是 Y 型,而非圓孔。舌頭擠壓
時 Y 型開口會打開,沒擠壓時開口會關
閉。以免奶水進入太多,讓寶寶嗆到。
4. 白色的節流器,控制奶水流量,也可避
免寶寶嗆到。

From 陳國鼎的顱顏世界
北醫顱顏中心 - 唇顎裂病人治療流程
北醫顱顏中心 - 唇顎裂病人治療流程
北醫顱顏中心 - 唇顎裂病人治療流程
Thanks for listening

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