• Journal of Korean Neurosurgical Society
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• v.59 no.3
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• pp.204-213
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• 2016

Syndromic craniosynostosis has severe cranial stenosis and deformity, combined with hypoplastic maxillary bone and other developmental skeletal lesions. Among these various lesions, upper air way obstruction by hypoplastic maxillary bone could be the first life-threatening condition after birth. Aggressive cranial vault expansion for severely deformed cranial vaults due to multiple synostoses is necessary even in infancy, to normalize the intracranial pressure. Fronto-orbital advancement (FOA) is recommended for patients with hypoplastic anterior part of cranium induced by bicoronal and/or metopic synostoses, and posterior cranial vault expansion is recommended for those with flattening of the posterior part of the cranium by lambdoid synostosis. Although sufficient spontaneous reshaping of the cranium can be expected by expansive cranioplasty, keeping the cranial bone flap expanded sufficiently is often difficult when the initial expansion is performed during infancy. So far distraction osteogenesis (DO) is the only method to make it possible and to provide low rates of re-expansion of the cranial vault. DO is quite beneficial for both FOA and posterior cranial vault expansion, compared with the conventional methods. Associated hydrocephalus and chronic tonsillar herniation due to lambdoid synostosis can be surgically treatable. Abnormal venous drainages from the intracranial space and air way obstruction should be always considered at any surgical procedures. Neurosurgeons have to know well about the managements not only of the deformed cranial vault and the associated brain lesions but also of other multiple skeletal lesions associated with syndromic craniosynostosis, to improve treatment outcome.

• Journal of Korean Neurosurgical Society
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• v.41 no.5
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• pp.343-346
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• 2007

Hypoplasia of the internal carotid artery is a rare congenital anomaly. Agenesis, aplasia, and hypoplasia of the internal carotid artery [ICA] are frequently associated with cerebral aneurysms in the circle of Willis. Authors report two cases with congenital hypoplasia of the ICA accompanying with the aneurysms. Transfemoral cerebral angiography [TFCA] in one patient identified nonvisualization of the left ICA. Bilateral anterior cerebral artery [ACA] and middle cerebral artery [MCA] were supplied from the right ICA accompanying with two aneurysms at anterior communicating artery [AcoA] and A1 portion of the left ACA. TFCA in another patient demonstrated hypoplastic left ICA and left ACA filled from the right ICA accompanying with AcoA aneurysm. Left MCA was filled from basilar artery via posterior communicating artery [PcoA]. Skull base computed tomography [CT] in two patients showed hypoplastic carotid canal. Authors performed direct aneurysmal neck clipping. Follow up CT angiography [CTA] at one year after surgery did not show regrowth or new development of the aneurysm. In patients with hypoplastic ICA, neurosurgeons should be aware of the possibility of development of the aneurysms, presumably because of hemodynamic process. Direct aneurysmal neck clipping is a good treatment modality. After operation, regular CTA, magnetic resonance angiography [MRA] or TFCA is needed to find progressive lesion and to prevent cerebrovascular attack [CVA].

• Journal of Chest Surgery
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• v.43 no.2
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• pp.161-163
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• 2010

A 12-day-old female baby underwent a Norwood procedure for hypoplastic left heart syndrome. The left superior vena cava (LSVC), which was found incidentally during the operation, was divided to facilitate surgical exposure. After the operation, she developed signs of low cardiac output and died 7 hours afterward. Autopsy findings showed that the coronary sinus was atretic at the orifice without unroofing into both atria, rendering the LSVC the sole route of coronary sinus drainage. In patients with incidentally-found LSVC during surgery, special care should be taken to leave the LSVC intact because the LSVC may be the exclusive drainage vein of the coronary venous system.

• Journal of the korean academy of Pediatric Dentistry
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• v.23 no.3
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• pp.601-608
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• 1996

Robinow syndrome or fetal face syndrome is a rare inherited disorder characterized by short stature, mesomelic brachymelia, hypoplastic genitalia, and a typical facial appearence ("fetal face") with frontal bossing, hypertelorism, ear abnormalities, a short upturned nose, long philtrum, micrognathia, and macrocephaly. Intraoral features have included quite a few dental cavities, crowding, hypoplastic uvula, cleft lip or/and cleft palate, gingival hyperplasia, alveolar hyperplasia, enamel hypoplasia, delayed eruption, and congenital missing of the permanent teeth. We report on a 10 years old girl with Robinow syndrome. The patient had most of the typical anomalies of the syndrome and negative family history but, in addition, had mental retardation, hearing loss, and serous otitis media. Intraoral findings included dental cavities, crowding, hypoplastic uvula, repaired cleft palate, and mouth breathing. Dental treatment and V-tube insertion(by dept. of ENT) were performed under general anesthesia. In all cases of Robinow syndrome, thorough evaluation and united treatments with medical specialists should be performed.

• Journal of The Korean Dental Society of Anesthesiology
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• v.13 no.4
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• pp.221-224
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• 2013

Despite the laryngeal mask airway (LMA) has been widely used in anesthesia, its use is rare in the field of dentistry. Placing LMA in the oral cavity may interrupt dental treatment. However, there are some circumstances in which LMA is more advantageous than tracheal intubation for managing the airway. Especially, the reinforced LMA has a flexible tube shaft, rendering it more accessible for dental treatment. We report a case of dental treatment of a 3-year-old patient with hypoplastic left heart syndrome combined with pulmonary hypertension using reinforced LMA for airway management under general anesthesia. We also discuss the considerations of utilizing the reinforced LMA for dental treatment.

• Journal of Chest Surgery
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• v.42 no.1
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• pp.9-13
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• 2009

Background: Hypoplastic left heart syndrome is uniformly fatal if this condition is not properly treated. We reviewed the surgical results of treating hypoplastic left heart syndrome, and we evaluated the hemodynamics and functional status of these patients after they underwent a Fontan operation. Material and Method: To assess the surgical results, we retrospectively reviewed the medical records of 6 (M/F=4/2) patients who underwent a staged operation, including a Norwood procedure, a bidirectional Glenn procedure and a Fontan procedure between October 1997 to May 2005. The mean age of the patients was $17.3{\pm}10.8$ days (range: 9~36 days) at the $1^{st}$ staged operation, $8.9{\pm}7.1$ months (4.6~23.3 months) at the $2^{nd}$ staged operation (the Bidirectional Glenn procedure) and $32.4{\pm}9.8$ months at the final staged operation (the Fontan procedure). During the $2^{nd}$ staged operation, one of the patients received tricuspid valve repair due to regurgitation. All the patients underwent an extracardiac Fontan procedure using Gore-Tex conduit (20 mm: 2 patients, 18 mm: 4 patients) and one of them required fenestration. Result: 21 patients underwented a Norwood procedure. There were 7 early deaths and 4 interstage deaths. Bidirectional cavopulmonary shunt was performed in 10 patients and the Fontan procedure was done in 6 (mortality: 1 patient, Flow up loss: 1 patient, Awaiting a Fontan procedure: 2 patients). After the Fontan procedure, there was no complication except for one case of post operative bleedings. All the patients had good ventricular function and 2 had grade I tricuspid regurgitation, as noted on their echocardiography. The average follow up period after the Fontan procedure was $19.6{\pm}14.9$ months (range: 1.5~39.1 month). All the patients had normal sinus rhythm and they were put on aspirin and cardiac medication. During follow up period, all the patients had a good functional status (NYHA functional class I). Conclusion: All the patients who suffered with hypoplastic left heart syndrome and who underwent a Fontan procedure achieved a good hemodynamic and functional status, even though there was a relatively high operative mortality rate after stage I Norwood palliation. Therefore, thise staged operation should be strongly recommended as an important surgical strategy for treating hypoplastic left heart syndrome.

• Korean Journal of Radiology
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• v.19 no.6
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• pp.1042-1052
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• 2018

Objective: To determine the utility of computed tomography (CT) ventricular volumes and morphometric parameters for deciding the treatment strategy in children with a hypoplastic left ventricle (LV). Materials and Methods: Ninety-four consecutive children were included in this study and divided into small LV single ventricle repair (SVR) (n = 28), small LV biventricular repair (BVR) (n = 6), disease-matched control (n = 19), and control (n = 41) groups. The CT-based indexed LV volumes, LV-to-right-ventricular (LV/RV) volume ratio, left-to-right atrioventricular valve (AVV) area ratio, left-to-right AVV diameter ratio, and LV/RV long dimension ratio were compared between groups. Proportions of preferred SVR in the small LV SVR group suggested by the parameters were evaluated. Results: Indexed LV end-systolic (ES) and end-diastolic (ED) volumes in the small LV SVR group ($6.3{\pm}4.0mL/m^2$ and $14.4{\pm}10.2mL/m^2$, respectively) were significantly smaller than those in the disease-matched control group ($16.0{\pm}4.7mL/m^2$ and $37.7{\pm}12.0mL/m^2$, respectively; p < 0.001) and the control group ($16.0{\pm}5.5mL/m^2$ and $46.3{\pm}10.8mL/m^2$, respectively; p < 0.001). These volumes were $8.3{\pm}2.4mL/m^2$ and $21.4{\pm}5.3mL/m^2$, respectively, in the small LV BVR group. ES and ED indexed LV volumes of < $7mL/m^2$ and < $17mL/m^2$, LV/RV volume ratios of < 0.22 and < 0.25, AVV area ratios of < 0.33 and < 0.24, and AVV diameter ratios of < 0.52 and < 0.46, respectively, enabled the differentiation of a subset of patients in the small LV SVR group from those in the two control groups. One patient in the small LV biventricular group died after BVR, indicating that this patient might not have been a good candidate based on the suggested cut-off values. Conclusion: CT-based ventricular volumes and morphometric parameters can suggest cut-off values for SVR in children with a hypoplastic LV.

• Archives of Plastic Surgery
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• v.46 no.6
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• pp.599-602
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• 2019

We report a case of autologous breast reconstruction in which a thoracodorsal vessel was used as a recipient vessel after a hypoplastic internal mammary vessel was found on preoperative computed tomography (CT) angiography. A 46-year-old woman with no underlying disease was scheduled to undergo skin-sparing mastectomy and breast reconstruction using a deep inferior epigastric artery perforator flap. Preoperative CT angiography showed segmental occlusion of the right subclavian artery with severe atherosclerosis and calcification near the origin of the internal mammary artery, with distal flow maintained by collateral branches. The thoracodorsal artery was selected to be the recipient vessel because CT showed that it was of adequate size and was not affected by atherosclerosis. The patient experienced no postoperative complications, and the flap survived with no vascular complications. The breasts were symmetrical at a 6-month follow-up. This case highlights that preoperative vascular imaging modalities may help surgeons avoid using diseased vessels as recipient vessels in free flap breast reconstructions.

• Clinics in Shoulder and Elbow
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• v.1 no.1
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• pp.132-137
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• 1998

Neurofibromatosis is characterized by its skeletal involvement and cutaneous manifestation. Some patients develop focal area of hypoplasia and atrophy at limbs. We present the case of a 13-year-old boy with a fracture of the hypoplastic clavicle associated with neurofibromatosis and brownish cutaneous macules. The patient received open reduction and internal fixation with a semitubular plate and biopsy was done in order to clarify the pathology.

• Journal of Chest Surgery
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• v.23 no.6
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• pp.1191-1203
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• 1990

Pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries [abbreviated as PA+VSD+MAPCA in the following] has limited the success of attempts at accurate diagnosis and complete surgical repair. From April 1986 to September 1990, 23 patients with PA+VSD+MAPCA among 96 patients of PA+VSD in Seoul National University Children’s Hospital were encountered. The group comprised 14 male and 9 female patients with ages ranging from 17 days to 177 months [mean 49.6 months]. We operated one stage total repair on good pulmonary artery sized two patients by R.E.V. [Reparation a l’etage ventriculaire] and Rastelli operation respectively. And the 11 patients who had independent MAPCAs and hypoplastic central pulmonary artery were dealt with unifocalization and modified Blalock-Taussig Shunt and followed by second stage repair in 3 patients later. We successfully had managed 7 patients whose MAPCAs could be ligated with modified Blalock-Taussig Shunt and followed by second stage repair in 3 patients with R.E.V or Rastelli operation. Recently, three obstruction after 11 unifocalization procedures made us to try palliative right ventricle-pulmonary artery conduit operation by Gore-Tex vascular graft interposition under cardiopulmonary bypass. And so we managed another 3 patients with these procedures for the purpose of pulmonary artery growth whose central pulmonary artery were severely hypoplastic. We experienced one death after second stage repair whose central pulmonary artery was created by 12mm Gore-Tex vascular graft and was unifocalized.